The epidemiology of sarcoidosis in England is largely uncharted, with no population-level prevalence data and outdated incidence and mortality estimates. Our objective was to investigate contemporary trends in incidence, prevalence, and mortality.

This cohort study used primary care data from the UK Clinical Practice Research Datalink (CPRD), linked to secondary-care and national death registration. Patients aged ≥18 with sarcoidosis were identified using primary care codes. Age-and-sex standardised incidence and prevalence were calculated. Standardised mortality ratios (SMRs) compared mortality with the general population. A matched non-sarcoidosis cohort was constructed within CPRD, and Poisson regression compared all-cause mortality between incident cases and controls.

Between 2003 and 2023, 18,554 incident sarcoidosis patients were identified. The age- and sex-standardised incidence per 100,000 person-years increased from 6.65 in 2003 to 7.73 in 2023, with the most pronounced rise occurring between 2010 and 2016. Incidence rose notably among males and those over 60-year-olds. Sarcoidosis prevalence increased from 167 to 230 per 100,000 individuals. The age-and-sex standardised all-cause mortality rate was 12.2 per 1000 patients in 2023. Elevated mortality was observed in males [SMR: 1.8 (1.7-1.8)] and females [SMR: 2.1(2.0-2.2)], particularly in those aged 30-70 years old. Regression models indicated higher all-cause mortality in the incident sarcoidosis cohort compared to controls [adjusted mortality rate ratio 1.36 (95% CI 1.27-1.44)].

Sarcoidosis incidence has increased during the study period, with shifts in age-and-sex distribution and excess mortality risk. Recognising this burden is key to refining healthcare policies, optimising resources and improving patient outcomes.

None.

Some patients with sarcoidosis achieve spontaneous remission, whereas others repeatedly experience relapse. We examined differences in the clinical course of active sarcoidosis according to peripheral blood immunophenotypes before treatment.

This retrospective study compared peripheral blood immunophenotypes between patients with active sarcoidosis (n = 28) and healthy control subjects (n = 10). Patients with sarcoidosis were divided into the spontaneous remission group without treatment (n = 9), the non-relapsed group after treatment (n = 13), and the relapsed group after treatment (n = 6) and were compared for peripheral blood immunophenotypes and background characteristics at baseline.

Patients with sarcoidosis showed increases in activated T helper (Th) 1 cells, activated Th17 cells, and regulatory T (Treg) cell subsets. The proportion of effector Treg cells was highest in the spontaneous remission group, and the proportion of non-suppressive Treg cells was highest in the relapsed group. No differences were observed in the proportions of other CD4+ T cell subsets. The cut-off values for predicting spontaneous remission and relapse were calculated for the effector Treg/non-suppressive Treg ratio. As a result, A ratio ≥1.469 predicted spontaneous remission (75%), while ≤0.722 predicted relapse (66.7%).

Effector and non-suppressive Treg cell proportions before treatment may predict spontaneous remission and relapse in active sarcoidosis.

The incidence and prevalence of sarcoidosis vary globally, with the highest estimates in Scandinavia. Since there are no recent data from Norway, we aimed to investigate the prevalence, incidence and mortality of sarcoidosis over three decades among participants in the population-based HUNT Study. The study population included participants in the first three surveys of the HUNT Study (HUNT1,1984-86; HUNT2,1995-97 and HUNT3, 2006-08). We identified 365 participants with sarcoidosis. Approximately 10 % of sarcoidosis patients were identified by x-ray screening in HUNT1, the remaining by linkage to hospital records. We randomly selected four age, sex and survey-matched participants without sarcoidosis for each of the 310 participants with incident sarcoidosis and followed these from diagnosis until death or end of follow-up in 2023. We used Cox regression to estimate adjusted hazard ratios (HRs) with 95 % confidence intervals (CIs) of death from all-causes. From 1984 to 2018, annual prevalence increased from 0.54 (95 % CI 0.41, 0.72) to 1.34 (95 % CI 1.08, 1.64) percent, while incidence rate increased from 12.1 (95 % CI 9.5, 14.7) to 18.7 (95 % CI 13.4, 24.0) per 100 000/year. The largest increase was seen in non-Lofgren syndrome (nLS). People with nLS had more than twice the mortality compared to people without sarcoidosis (HR 2.4; 95 % CI, 1.9, 3.1). There was no clear association between Lofgren syndrome and overall mortality. Sarcoidosis prevalence and incidence appeared to increase, particularly for nLS. Mechanisms leading to increased mortality in nLS needs to be further addressed.

Sarcoidosis is a complex multisystem inflammatory disease characterized by noncaseating granulomas and variable clinical manifestations, most commonly affecting the lungs, skin, heart, and nervous system. Imaging is central in its diagnosis, staging, and management, providing essential insights into organ involvement and disease activity. Pulmonary manifestations remain the hallmark, with modalities such as high-resolution chest computed tomography (CT) and chest radiography offering critical diagnostic clues. Imaging techniques, including Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) and cardiac magnetic resonance imaging, are invaluable for identifying cardiac and systemic involvement, including cutaneous and musculoskeletal, while abdominal MRI and ultrasound help delineate hepatic and splenic manifestations. Neurosarcoidosis requires MRI for precise evaluation, supplemented by FDG-PET to guide biopsy and monitor treatment response. This chapter synthesizes the imaging features of sarcoidosis across organ systems, emphasizing practical approaches to diagnosis and management while identifying key areas for future research.

Sarcoidosis is a systemic granulomatous disease that may differ in clinical presentation, organ involvement and severity among different populations. The primary objective was to evaluate demographic characteristics, disease stage, diagnostic work-up and treatment of patients with sarcoidosis. The sarcoidosis registry was founded in 2022 at the Medical University of Vienna and is intended to record patients from all over Austria who were diagnosed with sarcoidosis. All relevant information was collected at the time of the initial presentation and then on an ongoing basis every 6 months thereafter. Data was available for 199 sarcoidosis patients who were treated at the Medical University of Vienna between 2022 and 2023. The mean age of all patients was 52 ± 13 years. At 57.5%, women were significantly more represented than men were. Of these patients, 44.5% were smokers, and significantly more of them were men (63.8%). Chest X-ray revealed sarcoidosis in lung stages 1, 2, 3 and 4 in 34. 5%, 46%, 9.5% and 6% respectively. Overall, 37.5% (male: 33 (38.8%)) of all patients received oral corticosteroid therapy (OCS) during their illness, the mean duration of treatment with OCS (years, median, IQR) was 5 (3; 7.5). Most patients who received OCS for their disease were in pulmonary stage 4 with 81.8%. Patients were on average overweight with a mean BMI of 28.3 kg/m2 (± 6.5). This study is the first characterization of sarcoidosis patients in Austria.

While the skin is a common target organ for sarcoidosis, cutaneous granulomatous vasculitis is rare among patients with sarcoidosis. Due to the lack of detailed studies on cutaneous sarcoid vasculitis, both dermatologists and pathologists remain unfamiliar with this rare but important vasculitic disorder. We clinicopathologically evaluated eight cases with biopsy-proven cutaneous vasculitis and cutaneous sarcoidosis and analyzed morphologic changes in the process of vasculitis for both small vessels and muscular vessels in detail. The various skin lesions ranged from papulonodular erythema, annular erythema, maculopapular erythema, livedo reticularis-like eruptions, erythema nodosum-like lesions, subcutaneous nodules to ulcerative lesions. The extremities were the most frequently affected sites. Bilateral hilar lymphadenopathy with pulmonary sarcoidosis was the most common extracutaneous comorbidity. Skin-limited sarcoidosis was identified in 3 cases. All cases demonstrated a common histopathologic feature with sarcoid granulomas impinging on the target vessels with resultant vessel destruction. Perivascular infiltration of sarcoid granulomas resulted in compression and destruction of small vessels. In muscular arteries and veins, sarcoid granulomas closely attached to the muscular vessel wall, infiltrated the muscular layers and either occupied or penetrated the vessel walls, eventually invading the vascular lumen and replacing the entire muscular layers. The intimal infiltration of sarcoid granulomas resulted in a marked luminal narrowing. The scarcity of reports on cutaneous sarcoid vasculitis may be due to the overlooking or misinterpretation of vascular destruction caused by sarcoid granuloma infiltration as a feature of sarcoid granuloma masses.

To evaluate possible risk factors for uveitis among Finnish sarcoidosis patients.

Patient charts of patients with sarcoidosis, with (n = 97) or without (n = 255) uveitis, and with a comprehensive eye examination from January 2014 to January 2021 at Tays Eye Centre, Tampere University Hospital, Finland were studied.

Sarcoidosis patients with uveitis had higher rate of lymphocytopenia (43% vs. 29%, p = 0.041) and lower serum lysozyme levels (2.0 mg/L vs. 2.3 mg/L, p = 0.049; 95% CI, -0.692 to -0.002). Lysozyme level or lymphocytopenia did not have a statistically significant effect on the probability of uveitis in a binary logistic regression analysis. No other differences in the potential risk factors with p-values ≤0.05 were found, including bilateral hilar lymphadenopathy, serum angiotensin-converting enzyme (ACE) levels, sex, age and history of smoking.

Lymphocytopenia and lower serum lysozyme levels present as possible risk factors for uveitis among patients with sarcoidosis. Systematic measurement of lymphocyte and lysozyme levels in sarcoidosis is needed to further understand their role as potential risk factors.

Sarcoidosis is a multi-system granulomatous disease of unknown etiology. In genetically susceptible individuals, the precipitating factors generate, via immunity mechanisms, a host granulomatous response. The granuloma, for unknown reasons thus far, may resolve or may persist and lead to organ damage and fibrosis. Infectious agents, occupational exposure, obesity, smoking and genetic factors are implicated in the pathogenesis of sarcoidosis. Macrophages are important in granuloma formation, and their M1/M2 phenotype is associated with the prognosis of the disease. CD4+ T helper cells play a central role in the pathogenesis of sarcoidosis. The major contributors appear to be Th1 and Th17.1 cells, whose microenvironmental behavior is dictated by the secretions of macrophages and dendritic cells. Higher levels of Th1 and Th17.1 cells are associated with chronic disease and resistance to corticosteroid treatment. In recent years, advances in the phenotyping of sarcoidosis with the help of HRCT, PET-CT and lung function tests have provided us with a better understanding of the disease. Genetic phenotyping performed by the GenPhenReSa consortium and the SAGA study has led to the recognition of new, distinct phenotypes. The reconstitution of dysregulated autophagy through persistent m-TORC-1 pathways may be a new treatment target in sarcoidosis.

Löfgren syndrome (LS) is a sarcoidosis subtype characterised by an acute disease course, bilateral hilar lymphadenopathy (BHL), erythema nodosum (EN), and ankle arthritis. LS in Caucasians appears to be self-limiting; however, our patients require glucocorticoid (GC) treatment. Here, we present a case of LS and review the literature to identify the characteristics of Japanese patients with LS. A 66-year-old woman was referred to Kobe City Medical Center General Hospital; she initially presented with an acute onset of low-grade fever and ankle arthritis, followed by EN. Skin biopsy revealed a noncaseating granuloma, and a chest computed tomography scan displayed BHL; she was diagnosed with LS. Her arthralgia ameliorated spontaneously, but the erythema persisted, necessitating GC treatment. A literature review revealed that the Japanese LS patients showed more fever, were more frequently treated with GCs, and more patients seemed to relapse, which may be explained by the absence of human leucocyte antigen-DR isotype 3, a good prognostic allele in Caucasians. Japanese LS may cause severe symptoms after development because of the differences in human leucocyte antigens from foreign countries. For early diagnosis, it is important to evaluate EN and BHL in patients with polyarthritis involving ankle arthralgia.

A 63-year-old Japanese housewife was admitted to our hospital because of hematuria and proteinuria lasting for 3 months. At the age of 59 years, she was diagnosed with neurosarcoidosis at another hospital, and she received oral glucocorticoid therapy for 1 year. Her serum angiotensin-converting enzyme (ACE) and 1, 25-dihydroxyvitamin D levels were elevated. Computed tomography showed lymphadenopathy of the tracheal bifurcation and diffuse nodular shadow in the lungs and liver. Renal biopsy findings were compatible with IgA nephropathy without noncaseating granulomas and glomerular galactose-deficient IgA1 (Gd-IgA1) was stained in mesangial area. Because of clinical suspicion of sarcoidosis, liver biopsy was also performed, which showed inflammation with multiple noncaseating granulomas. The patient was diagnosed with IgA nephropathy coincident with sarcoidosis. After oral administration of prednisolone, mild proteinuria persisted; however, serum creatinine level was normalized, hematuria disappeared, and serum ACE and 1, 25-dihydroxyvitamin D levels returned to normal. Although some patients with sarcoidosis occasionally present with glomerulonephritis, there have been few case reports of sarcoidosis with IgA nephropathy. This was the first case report in which glomerular Gd-IgA1 was identified in a patient with IgA nephropathy and sarcoidosis.

Uveitis is a common ocular manifestation in individuals with sarcoidosis, a multisystem inflammatory disorder. This study aimed to explore clinical and genetic factors associated with the presence or absence of uveitis in sarcoidosis patients.

Total 625 Dutch sarcoidosis patients were included. Among these, 170 underwent ophthalmic examination, and 61 were diagnosed with uveitis. Demographic and clinical data, including age, gender, race, biopsy status, chest radiography findings, TNF-α inhibitor treatment, and uveitis classification were collected retrospectively from medical records. Genetic data was available for HLA haplotypes, TNF-α G-308A, and BTNL2 G16071A polymorphisms.

The majority of the patients presented with bilateral uveitis (80.3%). The proportion of women was higher in the uveitis group compared to the non-uveitis group (67.2% and 47.7%; p = 0.014). Pulmonary involvement (chest radiographic stage II-III) was significantly lower in patients with uveitis (36.1% versus 64.2%; p < 0.001). Patients with uveitis were more often treated with TNF-α inhibitors (67.2% versus 29.4%; p < 0.001) and the outcome was better compared with the non-uveitis group, 92% vs 68%, responders (p < 0.012). Uveitis patients treated with TNF-α inhibitors (either adalimumab or infliximab) were more likely to suffer from intermediate or posterior uveitis than anterior uveitis. Genetic analysis identified a significant association between the BTNL2 G16071A GG genotype and uveitis (p = 0.012).

This study highlights distinctive demographic, clinical and genetic features associated with uveitis in sarcoidosis patients. Ocular sarcoidosis was more prevalent in women. Further research is warranted to explore the implications of these findings for treatment strategies and prognostic assessments.

Sarcoidosis is a sterile non-necrotizing granulomatous disease without known causes that can involve multiple organs with a predilection for the lung and thoracic lymph nodes. Worldwide it is estimated to affect 2-160/100,000 people and has a mortality rate over 5 years of approximately 7%. For sarcoidosis patients, the cause of death is due to sarcoid in 60% of the cases, of which up to 80% are from advanced cardiopulmonary failure (pulmonary hypertension and respiratory microbial infections) in all races except in Japan were greater than 70% of the sarcoidosis deaths are due to cardiac sarcoidosis. Scadding stages for pulmonary sarcoidosis associates with clinical outcomes. Stages I and II have radiographic remission in approximately 30%-80% of cases. Stage III only has a 10%-40% chance of resolution, while stage IV has no change of resolution. Up to 40% of pulmonary sarcoidosis patients progress to stage IV disease with lung parenchyma fibroplasia, bronchiectasis with hilar retraction and fibrocystic disease. These patients are at highest risk for the development of precapillary pulmonary hypertension, which may occur in up to 70% of these patients. Sarcoid patients with pre-capillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications. Stage IV fibrocytic sarcoidosis with significant pulmonary physiologic impairment, >20% fibrosis on HRCT or pre-capillary pulmonary hypertension have the highest risk of mortality, which can be >40% at 5-years. First line treatment for patients who are symptomatic (cough and dyspnea) with parenchymal infiltrates and abnormal pulmonary function testing (PFT) is oral glucocorticoids, such as prednisone with a typical starting dose of 20-40 mg daily for 2 weeks to 2 months. Prednisone can be tapered over 6-18 months if symptoms, spirometry, PFTs, and radiographs improve. Prolonged prednisone may be required to stabilize disease. Patients requiring prolonged prednisone ≥10 mg/day or those with adverse effects due to glucocorticoids may be prescribed second and third line treatements. Second and third line treatments include immunosuppressive agents (e.g., methotrexate and azathioprine) and anti-tumor necrosis factor (TNF) medication; respectively. Effective treatments for advanced fibrocystic pulmonary disease are being explored. Despite different treatments, relapse rates range from 13% to 75% depending on the stage of sarcoid, number of organs involved, socioeconomic status, and geography. CONCLUSION: The mortality rate for sarcoidosis over a 5 year follow up is approximately 7%. Unfortunately, 10%-40% of patients with sarcoidosis develop progressive pulmonary disease, and >60% of deaths resulting from sarcoidosis are due to advance cardiopulmonary disease. Oral glucocorticoids are the first line treatment, while methotrexate and azathioprine are considered second and anti-TNF agents are third line treatments that are used solely or as glucocorticoid sparing agents for symptomatic extrapulmonary or pulmonary sarcoidosis with infiltrates on chest radiographs and abnormal PFT. Relapse rates have ranged from 13% to 75% depending on the population studied.

Sarcoidosis is a multisystem granulomatous syndrome that arises from a persistent immune response to a triggering antigen(s). There is no "gold standard" test or algorithm for the diagnosis of sarcoidosis, making the diagnosis one of exclusion. The presentation of the disease varies substantially between individuals, in both the number of organs involved, and the manifestations seen in individual organs. These qualities dictate that health care providers diagnosing sarcoidosis must consider a wide range of possible alternative diagnoses, from across a range of presentations and medical specialties (infectious, inflammatory, cardiac, neurologic). Current guideline-based diagnosis of sarcoidosis recommends fulfillment of three criteria: 1) compatible clinical presentation and/or imaging 2) demonstration of granulomatous inflammation by biopsy (when possible) and, 3) exclusion of alternative causes, but do not provide guidance on standardized strategies for exclusion of alternative diagnoses. In this review, we provide a summary of the most common differential diagnoses for sarcoidosis involvement of lung, eye, skin, central nervous system, heart, liver, and kidney. We then propose a framework for testing to exclude alternative diagnoses based on pretest probability of sarcoidosis, defined as high (typical findings with sarcoidosis involvement confirmed in another organ), moderate (typical findings in a single organ), or low (atypical/findings suggesting of an alternative diagnosis). This work highlights the need for informed and careful exclusion of alternative diagnoses in sarcoidosis.

Sarcoidosis is a systemic granulomatous inflammatory disease with varying clinical presentations, including sarcoid uveitis, which is the most prevalent ocular manifestation of sarcoidosis and differs by region and ethnicity. This study aims to determine the incidence and prevalence of sarcoidosis-related uveitis in South Korea, providing essential epidemiological data.

This nationwide, population-based retrospective study used the Korean Health Insurance Review and Assessment database from 2010 to 2019. It included all individuals diagnosed with sarcoidosis (ICD-10 code D86) and focused on those with newly developed uveitis. We assessed annual incidence, prevalence, gender-specific trends, age at diagnosis, treatment patterns, and recurrence. Cases were categorized as single episode or recurrent/chronic based on treatment duration and interval before recurrence.

Among 9,485 patients with sarcoidosis, 2,730 (28.8%) had sarcoidosis-related uveitis, with 2,413 (88.4%) being recurrent/chronic. Anterior uveitis was the most common presentation (78.0%), with posterior segment involvement in 22.0% of cases. The peak age of onset was 50-59 years for both genders. The incidence rate was 0.33 and 0.64 per 100,000 for men and women, respectively. The prevalence was 0.18 and 0.35 per 100,000 for men and women, respectively. Among sarcoidosis patients, 32.9% with uveitis used three or more combinations of systemic steroids and immunosuppressants, compared to 11.1% without uveitis.

The high prevalence of sarcoid uveitis in sarcoidosis patients necessitates regular ophthalmic examinations. Our findings highlight the need for comprehensive eye care in managing sarcoidosis and suggest that uveitis may increase disease severity, warranting tailored treatment and enhanced monitoring protocols in Korea.

This study aims to explore the potential subgroups of sarcoidosis-associated uveitis (SAU) within a multicenter cohort of uveitis participants.

Cross-sectional study.

A cohort of 826 uveitis patients from a uveitis registry from 19 clinical centers in 12 countries between January 2011 and April 2015.

We employed a latent class analysis (LCA) incorporating recommended tests and clinical signs from the revised International Workshop on Ocular Sarcoidosis (IWOS) to identify potential SAU subgroups within the multicenter uveitis cohort. Additionally, we assessed the performance of the individual tests and clinical signs in classifying the potential subclasses.

Latent subtypes of SAU.

Among 826 participants included in this analysis, the 2-class LCA model provided a best fit, with the lowest Bayesian information criteria of 7218.7 and an entropy of 0.715. One class, consisting of 548 participants, represented the non-SAU, whereas the second class, comprised of 278 participants, was most representative of SAU. Snowballs/string of pearls vitreous opacities had the best test performance for classification, followed by bilaterality and bilateral hilar lymphadenopathy (BHL). The combination of 4 tests with the highest classification importance, including snowballs/string of pearls vitreous opacities, periphlebitis and/or macroaneurysm, bilaterality, and BHL, demonstrated a sensitivity of 84.8% and a specificity of 95.4% in classifying the SAU subtypes. In the exploratory analysis of the 3-class LCA model, which had comparable fit indices as the 2-class model, we identified a candidate non-SAU subtype, candidate SAU subtype with pulmonary involvement, and a candidate SAU with less pulmonary involvement.

Latent class modeling, incorporating tests and clinical signs from the revised IWOS criteria, effectively identified a subset of participants with clinical features indicative of SAU. Though the sensitivity of individual ocular signs or tests was not perfect, using a combination of tests provided a satisfactory performance in classifying the SAU subclasses identified by the 2-class LCA model. Notably, the classes identified by the 3-class LCA model, including a non-SAU subtype, an SAU subtype with pulmonary involvement, and an SAU subtype with less pulmonary involvement, may have potential implication for clinical practice, and hence should be validated in further research.

Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Despite the scarcity of data, most guidelines have advocated for the treatment of cardiac sarcoidosis with corticosteroids. However, there is heterogeneity in disease presentation and response to treatment, which can make treatment challenging. The ability to identify disease phenotypes to allow for tailored therapy is therefore highly desirable. This review will seek to outline the disease phenotypes of cardiac sarcoidosis and the role that FDG-PET imaging can play in identifying these phenotypes to optimize disease diagnosis and treatment management.

FDG PET can identify cardiac sarcoidosis and is being increasingly used to monitor therapeutic response to immunosuppressive therapy, to follow treatment response after discontinuation of corticosteroid therapy, and to evaluate for disease relapse. Modern quantitative techniques using FDG PET imaging may allow for even better phenotypic disease characterization and the ability to track the response to immunosuppression more accurately. FDG PET currently plays an important role in cardiac sarcoidosis diagnosis. However, it also affords us the opportunity to offer insights into cardiac sarcoidosis disease phenotypes to better understand the underlying disease process and in the future may allows us to tailor therapies accordingly.

In this review, we aimed to summarize the different aspects of the field of cardio-rheumatology, the role of the cardio-rheumatologist, and future research in the field.

Cardio-rheumatology is an emerging subspecialty within cardiology that focuses on addressing the intricate relationship between systemic inflammation and cardiovascular diseases. It involves understanding the cardiovascular impact of immune-mediated inflammatory diseases on the heart and vascular system. A cardio-rheumatologist's role is multifaceted. First, they should understand the cardiac manifestations of rheumatological diseases. They should also be knowledgeable about the different immunotherapies available and side effects. Additionally, they should know how to utilize imaging modalities, either for diagnosis, prognosis, or treatment monitoring. This field is constantly evolving with new research on both treatment and imaging of the effects of inflammation on the cardiovascular system.

Sarcoidosis is an inflammatory condition that can affect multiple organ systems and is characterized by the formation of non-caseating granulomas in various organs, including the heart. Due to suboptimal diagnostic rates, the true prevalence and incidence of cardiac sarcoidosis (CS) remain to be determined. In patients with suspected CS, an initial examination should include 12-lead ECG or ambulatory ECG monitoring, and echocardiography with the estimation of LV, RV function, and strain rate. In patients with confirmed extracardiac sarcoidosis and with high clinical suspicion for CS, sophisticated imaging modalities, including cardiac MRI and PET, are indicated. Typical inflammation patterns and myocardial scarring should pose a high suspicion for CS. In patients without diagnosed extracardiac sarcoidosis and high clinical suspicion, although with low diagnostic probability, an endomyocardial biopsy should be considered to establish the diagnosis of definite isolated cardiac sarcoidosis. Timely diagnosis enables the initiation of therapy and close monitoring of adverse cardiac events that can be life-threatening, including sudden cardiac death, ventricular tachycardia, high-degree AV block, and heart failure. Implementing biomarkers in correlation to cardiac imaging can determine the disease's severity and progression but can also be helpful in following the treatment response. The formation of larger global registries can be helpful in the identification of independent predictors of adverse clinical events and the development of specific diagnostic algorithms to reduce the overall risk of this serious condition.

The prevalence of sarcoidosis is known to be high in the Nordic countries. There are no recent research data on the incidence or prevalence of sarcoidosis in Finland. Our aim was to investigate the epidemiology of sarcoidosis in Finland through a retrospective registry-based study.

We made an information request to the Hilmo database on patients who had been treated in Finnish specialised care with a main diagnosis related to sarcoidosis. Data were requested for the period 1 January-31 December for the years 2002, 2012 and 2022. In addition, we examined the age and gender distribution and regional differences in these variables between the five university hospital districts covering the whole of Finland.

The incidence of sarcoidosis was 17‒19/100 000/year throughout the follow-up period. The prevalence of sarcoidosis in the ≥18-year-old population had risen from 85/100 000 in 2002-106/100 000 in 2022. There were considerable differences between university hospital districts: The highest prevalence rate was 170/100 000 in the Tampere University Hospital district in 2022, which was twice as high as in the Helsinki University Hospital district (84/100 000). The proportion of pulmonary sarcoidosis in all sarcoidosis cases decreased from 62% to 45% while the proportion of multiorgan sarcoidosis (D86.8) increased from 11% to 34%. The incidence of sarcoidosis was 15/100 000 and the prevalence was 82/100 000 in the age groups of ≥60 years in 2002. In 2022, the incidence in this same age group had risen to 20/100 000 and the prevalence to 109/100 000. In the ≥60-year-old population, the proportion of D86.8 increased from 11% to 35%.

Sarcoidosis was a more common disease in Finland than in previous studies. Multiorgan sarcoidosis among the elderly has increased over the past 20 years. This might be explained by changes in environmental factors associated with sarcoidosis. Significant regional differences in prevalence might be partly explained by familial clustering.

A 70-year-old female presented with bilateral numbness in her upper limbs. She was diagnosed with cervical spondylotic myelopathy and underwent cervical laminoplasty. However, there was no significant improvement in sensory disturbance, and at 6 months after surgery, she developed subacute motor and gait disturbance in four extremities. Spinal MRI revealed a long lesion of the spinal cord with edema, and a part of the lesion showed gadolinium contrast enhancement. Bronchoscopy revealed an elevated CD4/8 ratio, and gallium scintigraphy demonstrated an accumulation in the hilar lymph nodes, leading to a diagnosis of neurosarcoidosis. In case of rapid deterioration during the course of cervical spondylotic myelopathy, neurosarcoidosis should be considered as a differential diagnosis, which can be assessed by contrast-enhanced MRI.

Sarcoidosis is a rare disease that severely affects the lungs and superficial lymph nodes. In addition, this disease can also affect the skin, eyes and kidneys to varying degrees. The present report described a 32-year-old male patient who was admitted to Renmin Hospital of Wuhan University (Wuhan, China) due to joint pain in the extremities. He was diagnosed with uncorrectable hypercalcemia. A lymph node biopsy revealed the hypercalcemia to be associated with sarcoidosis, with the patient also demonstrating renal failure and lymph node enlargement. Administration of glucocorticoids provided benefits in terms of both primary and recurrent sarcoidosis, which also improved and preserved renal function. After being prescribed with oral prednisone treatment, blood calcium levels returned to normal, which indicated markedly improving renal function. However, the discontinuation of glucocorticoids for 2 months resulted in increased serum calcium and creatinine levels, both of which returned to abnormal levels. Overall, the present case report suggests that clinicians should actively perform sarcoidosis treatment in clinical practice to overcome any unexpected results associated with organ damage.

Clinical presentation and prevalence of organ involvement is highly variable in sarcoidosis and depends on ethnic, genetic and geographical factors. These data are not extensively studied in a Dutch population.

To determine the prevalence of organ involvement and the indication for systemic immunosuppressive therapy in newly diagnosed sarcoidosis patients in the Netherlands.

Two large Dutch teaching hospitals participated in this prospective cohort study. All adult patients with newly diagnosed sarcoidosis were prospectively included and a standardized work-up was performed. Organ involvement was defined using the WASOG instrument.

Between 2015 and 2020, a total of 330 patients were included, 55% were male, mean age was 46 (SD 14) years. Most of them were white (76%). Pulmonary involvement including thoracic lymph node enlargement was present in 316 patients (96%). Pulmonary parenchymal disease was present in 156 patients (47%). Ten patients (3%) had radiological signs of pulmonary fibrosis. Cutaneous sarcoidosis was present in 74 patients (23%). Routine ophthalmological screening revealed uveitis in 29 patients (12%, n = 256)). Cardiac and neurosarcoidosis were diagnosed in respectively five (2%) and six patients (2%). Renal involvement was observed in 11 (3%) patients. Hypercalcaemia and hypercalciuria were observed in 29 (10%) and 48 (26%, n = 182) patients, respectively. Hepatic involvement was found in 6 patients (2%). In 30% of the patients, systemic immunosuppressive treatment was started at diagnosis.

High-risk organ involvement in sarcoidosis is uncommon at diagnosis. Indication for systemic immunosuppressive therapy was present in a minority of patients.

We report the case of a 48-year-old male with a history of pulmonary and ocular sarcoidosis. Non-caseating granulomas, identified histologically, are the most characteristic manifestation of sarcoidosis. Hepatic sarcoidosis is difficult to diagnose using radiological imaging. In the patient reported in this study, ultrasound and contrast-enhanced computed tomography scans identified multiple intra-abdominal lymphadenopathies, with evidence of liver and splenic infiltrations. The first liver biopsy revealed non-caseating granulomatous hepatitis consistent with hepatic sarcoidosis. The patient was treated with ursodeoxycholic acid (UDCA), but his laboratory parameters did not improve. Prednisone was initiated at a dose of 30 mg daily and slowly tapered. At a dose of 12.5 mg daily, marked improvements in the fibrotic and sarcoid-like lesions were noted at the second biopsy. A third biopsy was performed, with the patient on a prednisone taper of 5 mg/day showed mild fibrous expansion in the portal tracts and mild parenchymal necro-inflammatory lesions. However, overall, fibrosis marker levels remained stable over the course of treatment. A fourth biopsy was performed after a 5-year course of 5 mg/day prednisone. This revealed minimal lobular inflammation without fibrosis. Thus, treatment of this patient with corticosteroids and UDCA resulted in marked improvements in his biochemical and histological parameters.

Imaging with late gadolinium enhancement (LGE) magnetic resonance (MR) and 18F-fluorodeoxyglucose (18F-FDG) PET allows complementary assessment of myocardial injury and disease activity and has shown promise for improved characterization of active cardiac sarcoidosis (CS) based on the combined positive imaging outcome, MR(+)PET(+).

This study aims to evaluate qualitative and quantitative assessments of hybrid MR/PET imaging in CS and to evaluate its association with cardiac-related outcomes.

A total of 148 patients with suspected CS underwent hybrid MR/PET imaging. Patients were classified based on the presence/absence of LGE (MR+/MR-), presence/absence of 18F-FDG (PET+/PET-), and pattern of 18F-FDG uptake (focal/diffuse) into the following categories: MR(+)PET(+)FOCAL, MR(+)PET(+)DIFFUSE, MR(+)PET(-), MR(-)PET(+)FOCAL, MR(-)PET(+)DIFFUSE, MR(-)PET(-). Further analysis classified MR positivity based on %LGE exceeding 5.7% as MR(+/-)5.7%. Quantitative values of standard uptake value, target-to-background ratio, target-to-normal-myocardium ratio (TNMRmax), and T2 were measured. The primary clinical endpoint was met by the occurrence of cardiac arrest, ventricular tachycardia, or secondary prevention implantable cardioverter-defibrillator (ICD) before the end of the study. The secondary endpoint was met by any of the primary endpoint criteria plus heart failure or heart block. MR/PET imaging results were compared between those meeting or not meeting the clinical endpoints.

Patients designated MR(+)5.7%PET(+)FOCAL had increased odds of meeting the primary clinical endpoint compared to those with all other imaging classifications (unadjusted OR: 9.2 [95% CI: 3.0-28.7]; P = 0.0001), which was higher than the odds based on MR or PET alone. TNMRmax achieved an area under the receiver-operating characteristic curve of 0.90 for separating MR(+)PET(+)FOCAL from non-MR(+)PET(+)FOCAL, and 0.77 for separating those reaching the clinical endpoint from those not reaching the clinical endpoint.

Hybrid MR/PET image-based classification of CS was statistically associated with clinical outcomes in CS. TNMRmax had modest sensitivity and specificity for quantifying the imaging-based classification MR(+)PET(+)FOCAL and was associated with outcomes. Use of combined MR and PET image-based classification may have use in prognostication and treatment management in CS.

Bronchial and lung parenchymal structural remodeling may occur due to disease progression in patients with pulmonary sarcoidosis; however, its mechanisms remain unclear. Central bronchial deformity (CBD) associated with shrinkage in the upper lobe (SUL) is often observed in such patients. This study aimed to examine the association between CBD and structural remodeling to identify features indicating disease severity on chest images.

This retrospective cohort study included 72 patients with pulmonary sarcoidosis, excluding patients with only bilateral hilar lymphadenopathy. The participants were divided into with and without CBD groups to examine the association between CBD and other structural remodeling, including SUL, cyst and/or low attenuation area-like emphysema (Cyst/LAA), pleural/sub-pleural thickening (PT), and traction bronchiectasis (TrBE), in the upper lobe on chest images. The association of CBD phenotype with respiratory dysfunction was also examined.

CBD was highly associated with SUL (81.4% vs. 8.9%), Cyst/LAA (44.4% vs. 6.7%), and PT (59.2% vs. 3.7%). The respective odds ratios in the univariable and multivariable analyses were as follows: SUL, 45.1 and 39.9; Cyst/LAA, 11.2 and 14.2; and PT, 64.0 and 68.7. TrBE was frequently associated with CBD (22.25% vs. 4.4%); the odds ratio was 6.14 in the univariable analysis. Furthermore, participants with CBD exhibited lower %FVC and %DLCO.

CBD is significantly associated with lung remodeling (SUL, Cyst/LAA, TrBE, and PT) and respiratory dysfunction. CBD may be a crucial clinical phenotype to identify upper lobe fibrotic changes.

Sarcoidosis is a systemic, granulomatous disease with variable presentation earning it the term "the great mimicker." The current epidemiology confirms that the disease occurs worldwide, affecting both sexes, and all races, ethnicities, and ages. To date, no causal exposure or agent has been identified. The organ systems most frequently affected by sarcoidosis are also those with greatest exposure to the natural world suggesting environmental and lifestyle contributions to the disease. These include particulate matter, microorganisms, nicotine, and obesity. In this article, we review the epidemiology of sarcoidosis and discuss these non-genetic risk factors in the hope of providing important insight into sarcoidosis and stimulating future research.

Cardiac involvement is a major cause of morbidity and mortality in patients with sarcoidosis. It is important to distinguish between clinical manifest diseases from clinically silent diseases. Advanced cardiac imaging studies are crucial in the diagnostic pathway. In suspected isolated cardiac sarcoidosis, it's key to rule out alternative diagnoses. Therapeutic options can be divided into immunosuppressive agents, guideline-directed medical therapy, antiarrhythmic medications, device/ablation therapy, and heart transplantation.

Ocular sarcoidosis most commonly presents with symptoms and is first diagnosed before systemic sarcoidosis in at least half of the patients with sarcoidosis. Prevalence of ocular involvement in sarcoidosis varies between 2-80% depending on the study setting, included ocular diseases, and studied population. In many studies, ocular involvement in sarcoidosis has been overestimated mainly because study populations have been collected from eye clinics and because the study criteria have included ocular findings or symptoms that do not require treatment or monitoring. In a screening setting, asymptomatic ocular sarcoidosis has been detected in only 2-5%. 0-1% of the screened sarcoidosis patients have required treatment. For these reasons, ocular screening in sarcoidosis seems generally of little value. Patients with sarcoidosis who present with ocular symptoms should be screened for ocular sarcoidosis in a timely manner because they are at high risk of ocular disease.

A 70-year-old man had developed a high fever and arthralgia in his right elbow 6 months prior. Loxoprofen improved the symptoms temporarily, but arthropathy developed in other joints. Long-term recurrent arthropathy and the fever caused activity reduction and progressive debilitation. We performed fluorine-18 fluorodeoxyglucose-positron emission tomography and detected a positive accumulation in multiple joints and lymph nodes. A lymph node biopsy revealed epithelioid cell granulomas, which, along with elevated angiotensin-converting enzyme levels, led to the diagnosis of sarcoid arthropathy. After prednisolone administration, the fever and arthralgia resolved, and his activities of daily living improved. Clinicians should be aware of this type of sarcoid arthropathy.

Neurosarcoidosis is a rare manifestation of sarcoidosis with heterogeneous presentations. Patient management is challenging due to the current lack of knowledge about the long-term disease course.

To identify specific disease courses of neurosarcoidosis according to the clinical and paraclinical presentations at onset.

We conducted an observational multicenter cohort study by retrospectively collecting data from the medical records of 84 patients diagnosed with definite, probable, or possible neurosarcoidosis in three tertiary referral centers in France (Nancy, Strasbourg, and Bordeaux). We collected demographic characteristics, clinical and paraclinical data at the beginning of patient management, and during follow-up under the different treatment lines. Two expert neurologists determined disease course profiles.

The mean follow-up was 6.6 years. Almost every patient (96.4%) received steroids at some point of their follow-up. Tumor Necrosis Factor-alpha blockers were given in 10.7% as first-line treatment and in 33.3% during follow-up. Every patient presented with a relapsing disease, often monophasic (75%) and sometimes polyphasic with the recurrence of identical manifestations (11.9%). Patients developing new neurological symptoms during follow-up were a minority (13.1%). No patients exhibited a progressive course. Patients with isolated cranial nerves injury or aseptic meningitis always exhibited a monophasic course, and 62.5-75% of them had a full recovery after first-line treatments. This proportion was 15.6% in other forms of the disease. Those with peripheral presentations were more likely to present a polyphasic course than patients with other forms of neurosarcoidosis. Spinal cord presentations were monophasic, but resulted in sequelae and exhibited poor response to first-line treatments despite frequent use of TNF-alpha blockers.

Identification of these disease course profiles, based on the initial clinical and paraclinical presentation, could guide the clinician to select the optimal therapeutic approach and follow-up modalities for their patients with neurosarcoidosis.

Systematic ocular screening is recommended in sarcoidosis, because of a high rate of ocular involvement. The purpose of this study was to determine whether ocular screening is useful in sarcoidosis in a Finnish university hospital population with 0.5 M inhabitants.

Patient charts of patients with sarcoidosis, without a history of ocular sarcoidosis, without ocular inflammatory symptoms, and with a comprehensive eye exam from January 2014 to January 2021 at Tays Eye Centre, Tampere, Finland, were studied.

Five of 262 patients (2%) were diagnosed with asymptomatic uveitis. No other types of ocular sarcoidosis were found. Anterior uveitis without complications was present in three patients, unilaterally in two and bilaterally in one patient. Posterior uveitis was present in two patients, a unilateral choroidal granuloma requiring treatment in one and bilateral punched-out chorioretinal lesions in the other patient.

With this low rate of ocular involvement requiring treatment in sarcoidosis, systematic screening for asymptomatic ocular sarcoidosis does not seem useful in a Finnish population. In Tays Eye Centre, systematic screening of ocular sarcoidosis was discontinued in 2021.

Sarcoidosis is a heterogenous, multisystemic inflammatory disease that primarily affects lungs. In this study, we multiplex genotyped 18 single-nucleotide polymorphisms (SNPs) to replicate the findings from previous genome-wide association studies (GWAS) and candidate gene studies, and extended analyses to different clinical manifestations (Lofgren syndrome and chest X-ray [CXR] stages) including treatment response among West-Slavonic subjects (564 sarcoidosis patients and 301 healthy controls). We confirm the replication (with Bonferroni correction) of ANXA11 rs1049550 as protective variant for sarcoidosis (odds ratio [OR]=0.71, p=1.33×10^-3), non-LS (OR=0.66, p=2.71×10^-4) and CXR stages 2-4 (OR=0.62, p=7.48×10^-5) compared to controls in West-Slavonic population. We also validate the association of risk variants C6orf10 rs3129927 (OR=2.61, p=2.60×10^-8), TNFA rs1800629 (OR=1.56, p=6.65×10^-4), ATF6B rs3130288 (OR=2.75, p=1.06×10^-9) and HLA-DQA1 rs2187668 (OR=1.74, p=8.83×10^-4) with sarcoidosis compared to controls. For sub-phenotypes compared to controls, risk variants C6orf10 rs3129927 (OR=5.35, p=1.07×10^-12), TNFA rs1800629 (OR=2.66, p=5.94×10^-7), ATF6B rs3130288 (OR=5.24, p=5.21×10^-13), LRRC16A rs9295661 (OR=2.97, p=4.29×10^-4), HLA-DQA1 rs2187668 (OR=3.14, p=1.09×10^-6) and HLA-DRA rs3135394 (OR=5.23, p=8.25×10^-13) were associated with LS while C6orf10 rs3129927 (OR=1.96, p=4.27×10^-4) and ATF6B rs3130288 (OR=2.15, p=3.36×10^-5) were associated with non-LS. For CXR stages compared to controls, C6orf10 rs3129927 (OR=3.67, p=3.63×10^-11), TNFA rs1800629 (OR=1.84, p=1.32×10^-4), ATF6B rs3129927 (OR=3.63, p=1.82×10^-11), HLA-DQA1 rs2187668 (OR=2.13, p=9.59×10^-5) and HLA-DRA rs3135394 (OR=3.42, p=3.45×10^-10) were risk variants for early CXR stages 0-1 while C6orf10 rs3129927 (OR=1.99, p=5.51×10^-4), ATF6B rs3129927 (OR=2.23, p=3.52×10^-5) and HLA-DRA rs3135394 (OR=1.85, p=2.00×10^-3) were risk variants for advanced CXR stages 2-4. The present findings nominate gene variants as plausible prognostic markers for clinical phenotypes, treatment response and disease resolution/progression and may form the basis for establishing genotype-phenotype relationships in patients with sarcoidosis among West-Slavonic population.

Sarcoidosis is an inflammatory disease that involves the eyes in 10-55% of cases, sometimes without systemic involvement. All eye structures can be affected, but uveitis is the most common ocular manifestation and causes vision loss. The typical ophthalmological appearance of these uveitis is granulomatous (in cases with anterior involvement), which are usually bilateral and with synechiae. Posterior involvement includes vitritis, vasculitis and choroidal lesions. Tuberculosis is a classic differential diagnosis to be wary of, especially in people who have spent time in endemic areas. The diagnosis is based on histology with the presence of non-caseating epithelioid granulomas. However, due to the technical difficulty and yield of biopsies, the diagnosis of ocular sarcoidosis is often based on clinico-radiological features. The international criteria for the diagnosis of ocular sarcoidosis have recently been revised. Corticosteroids remain the first-line treatment for sarcoidosis, but up to 30% of patients require high doses, justifying the use of corticosteroid-sparing treatments. In these cases, immunosuppressive treatments such as methotrexate may be introduced. More recent biotherapies such as anti-TNF are also very effective (as they are in other non-infectious uveitis etiologies).

Idiopathic pulmonary fibrosis (IPF) is a severe and progressive chronic fibrosing interstitial lung disease with causes that have remained unclear to date. Development of effective treatments will require elucidation of the detailed pathogenetic mechanisms of IPF at both the molecular and cellular levels. With a biomedical corpus that includes IPF-related entities and events, text-mining systems can efficiently extract such mechanism-related information from huge amounts of literature on the disease. A novel corpus consisting of 150 abstracts with 9297 entities intended for training a text-mining system was constructed to clarify IPF-related pathogenetic mechanisms. For this corpus, entity information was annotated, as were relation and event information. To construct IPF-related networks, we also conducted entity normalization with IDs assigned to entities. Thereby, we extracted the same entities, which are expressed differently. Moreover, IPF-related events have been defined in this corpus, in contrast to existing corpora. This corpus will be useful to extract IPF-related information from scientific texts. Because many entities and events are related to lung diseases, this freely available corpus can also be used to extract information related to other lung diseases such as lung cancer and interstitial pneumonia caused by COVID-19.

Cardiac sarcoidosis is associated with a high mortality rate. Given multiple barriers to obtaining cardiac PET imaging, we suspect individuals with access to this imaging modality are not representative of the Sarcoid patient population, which in the United States are predominantly Black females.

To evaluate the demographics of patients with cardiac PET access and the cost-effectiveness of cardiac PET/MR imaging relative to standard of care.

This is a retrospective, observational study. The demographic information of patients with suspected cardiac sarcoidosis and cardiac PET/CT imaging within a national registry of sarcoidosis were reviewed (n = 4561). An individual-level, continuous, time-state transition model was used for the evaluation of long-term cost-effectiveness for the combined cardiac PET/MR compared to standard of care cardiac MR followed by cardiac PET/CT.

Patients who underwent cardiac PET in the national registry had 88.35% higher odds of being male (p < 0.001) and 43.82% higher odds of being White (p = 0.003) than their counterparts who did not have cardiac PET imaging. Combined cardiac PET/MR had overall lower total lifetime costs ($8761 vs $10,777) and overall improved expected quality of life-years compared to the standard of care (0.77 vs 0.69).

The findings suggest that patients with access to cardiac PET/CT are not representative of the patient population most likely to have cardiac sarcoidosis in this limited study evaluation. Universal insurance coverage should be considered for Cardiac PET imaging as same day cardiac PET and MR imaging has potential long-term cost and quality of life benefit.

Sarcoidosis is a rare systemic inflammatory granulomatous disease of unknown cause. It can manifest in any organ. The incidence of sarcoidosis varies across countries, and by ethnicity and gender. Delays in the diagnosis of sarcoidosis can lead to extension of the disease and organ impairment. Diagnosis delay is attributed in part to the lack of a single diagnostic test or unified commonly used diagnostic criteria, and to the diversity of disease manifestations and symptom load. There is a paucity of evidence examining the determinants of diagnostic delay in sarcoidosis and the experiences of people with sarcoidosis related to delayed diagnosis. We aim to systematically review available evidence about diagnostic delay in sarcoidosis to elucidate the factors associated with diagnostic delay for this disease in different contexts and settings, and the consequences for people with sarcoidosis.

A systematic search of the literature will be conducted using PubMed/Medline, Scopus, and ProQuest databases, and sources of grey literature, up to 25th of May 2022, with no limitations on publication date. We will include all study types (qualitative, quantitative, and mixed methods) except review articles, examining diagnostic delay, incorrect diagnosis, missed diagnosis or slow diagnosis of all types of sarcoidosis across all age groups. We will also examine evidence of patients' experiences associated with diagnostic delay. Only studies in English, German and Indonesian will be included. The outcomes we examine will be diagnostic delay time, patients' experiences, and factors associated with diagnostic delay in sarcoidosis. Two people will independently screen the titles and abstracts of search results, and then the remaining full-text documents against the inclusion criteria. Disagreements will be resolved with a third reviewer until consensus is reached. Selected studies will be appraised using the Mixed Methods Appraisal Tool (MMAT). A meta-analysis and subgroup analyses of quantitative data will be conducted. Meta-aggregation methods will be used to analyse qualitative data. If there is insufficient data for these analyses, a narrative synthesis will be conducted.

This review will provide systematic and integrated evidence on the diagnostic delay, associated factors, and experiences of diagnosis delay among people with all types of sarcoidosis. This knowledge may shed light on ways to improve diagnosis delays in diagnosis across different subpopulations, and with different disease presentations.

Ethical approval will not be required as no human recruitment or participation will be involved. Findings of the study will be disseminated through publications in peer-reviewed journals, conferences, and symposia.

PROSPERO Registration number: CRD42022307236. URL of the PROSPERO registration: https://www.crd.york.ac.uk/PROSPEROFILES/307236_PROTOCOL_20220127.pdf.

Myocarditis is defined as inflammation of the myocardium according to clinical, histological, biochemical, immunohistochemical, or imaging findings. Inflammation can be categorized histologically by cell type or pattern, and many causes have been implicated, including infectious, most commonly viral, systemic autoimmune diseases, vaccine-associated processes, environmental factors, toxins, and hypersensitivity to drugs. Sarcoid myocarditis is increasingly recognized as an important cause of cardiomyopathy and has important diagnostic, prognostic, and therapeutic implications in patients with systemic sarcoidosis. The clinical presentation of myocarditis may include an asymptomatic, subacute, acute, fulminant, or chronic course and may have focal or diffuse involvement of the myocardium depending on the cause and time point of the disease. For most causes of myocarditis except sarcoidosis, myocardial biopsy is the gold standard but is limited due to risk, cost, availability, and variable sensitivity. Diagnostic criteria have been established for both myocarditis and cardiac sarcoidosis and include clinical and imaging findings particularly the use of cardiac magnetic resonance and positron emission tomography. Beyond diagnosis, imaging findings may also provide prognostic value. This case-based review focuses on the current state of multimodality imaging for the diagnosis and management of myocarditis and cardiac sarcoidosis, highlighting multimodality imaging approaches with practical clinical vignettes, with a discussion of knowledge gaps and future directions.

Sarcoidosis is a multisystem disease with an unknown etiology and is characterized by the formation of noncaseating granulomas in the affected organs. We present the case of a 69-year-old male Japanese patient with bilateral hilar lymphadenopathy on chest radiographs for more than 10 years, left without further investigation. The patient reported no clinical symptoms. Chest computed tomography revealed ground-glass opacities and reticular shadows in both lungs, along with bilateral hilar and mediastinal lymphadenopathy. Lymphocytosis was observed in bronchoalveolar lavage fluid. Pathological examination of transbronchial lung biopsy revealed noncaseating, epithelioid granulomas congruous with sarcoidosis, together with other findings. There were no abnormalities on electrocardiogram, echocardiogram, and ophthalmic examination.For progressive dyspnea on exertion, systemic corticosteroid therapy with oral prednisolone (25 mg/day) was initiated in 2017 and gradually tapered. Despite this intervention, the decline in forced vital capacity (FVC) was accelerated. Three years later, the patient noticed swelling in his right wrist. Further investigation revealed elevated anti-cyclic citrullinated peptide antibodies and absence of noncaseating epithelioid granuloma on surgical biopsy, leading to the diagnosis of rheumatoid arthritis (RA). Thereafter, the anti-fibrotic agent nintedanib was initiated, because interstitial lung disease (ILD) was considered to have converted into a progressive fibrosing phenotype (PF-ILD) with overlapping RA-associated lung involvement. With treatment, the progression of decline in FVC was slowed, although home oxygen therapy was introduced.

The manifestations of sarcoidosis differ by ethnicity and region. However, the few studies that have focused on elderly sarcoidosis are only from Western countries. Therefore, we investigated elderly sarcoidosis in Japan.

We retrospectively reviewed the records of adult patients (≥18 years old) who were diagnosed with sarcoidosis from 1 April 2006 to 31 March 2020. The diagnosis was pathologically confirmed in all patients. We compared the clinical features of elderly (diagnosed at ≥65 years old) and non-elderly (diagnosed at <65 years old) patients.

Thirty-five (33%) of 106 patients were elderly. The elderly group had significantly more comorbidities than the non-elderly group (median [range], 1 [0-4] vs. 0 [0-5]). The biopsy site at diagnosis included significantly more extrathoracic sites in the elderly than non-elderly group (57.1% vs. 33.8%). The elderly group had significantly more muscle lesions than the non-elderly group at the time of diagnosis (11.4% vs. 1.4%) and at any time during follow-up (17.1% vs. 1.4%).

In Japan, elderly patients with sarcoidosis might have more muscle involvement and comorbidities than younger patients. Because comorbidities might affect the prognosis of elderly sarcoidosis, further study is needed to clarify the effect of comorbidities on elderly sarcoidosis.

To estimate the epidemiology of sarcoidosis in Puerto Rico.

Patients with a diagnosis of sarcoidosis between January 2015 and December 2018 were selected from the Puerto Rico Health Insurance Administration Administración de Seguros de Salud de Puerto Rico claims database. The International Classification of Diseases-Tenth Revision coding for sarcoidosis was used for case detection and utilization of health care services. Cases by sex and age were evaluated.

A total of 488 individuals with sarcoidosis were identified over the 4-year study. The median age at diagnosis was 59 years old. The average annual incidence rate was 9.9/100,000. The baseline prevalence was 14.9 per 100,000. Females represented 68.4% of the cases. Patients with lung and unspecified sarcoidosis predominate with 45.6% and 28.2% of cases, respectively. Overall, the yearly cost of health care utilization and medications per patient was $234 and $13,638, respectively.

Annual incidence rate and the median age of sarcoidosis in Puerto Ricans are among the highest and oldest reported globally. The demographic profile of sarcoidosis in Puerto Rico presents a patient in the mid-to advanced age, mostly female. While costs for medications were relatively high for a patient with sarcoidosis, the economic burden of the disease to the national health care system is not substantial.

Cardiac sarcoidosis, often manifested as sudden death, can be the first manifestation of sarcoidosis. Since 12-lead electrocardiogram (ECG) is recommended as an initial screening tool for cardiac sarcoidosis, the recognition of subtle abnormalities assumes utmost significance. The objective of this study was to identify the electrocardiographic abnormalities in patients with pulmonary sarcoidosis.

A detailed analysis of 12-lead ECGs obtained from sixty patients with histopathologically proven pulmonary sarcoidosis and no overt cardiac involvement was done. The findings were compared with those of an age-matched control group. Varying degrees of intraventricular conduction defects were common in the study group [67%], as well as the control group [57%] [P = 0.23]. There was a higher prevalence of biphasic P wave [P = 0.003] and bifid P wave [P = 0.029] in lead III and rsr' in lead aVF [P = 0.03] in the study group as compared to the control group.

Our study demonstrates a greater prevalence of subtle ECG abnormalities in patients with pulmonary sarcoidosis as compared to patients with other forms of pulmonary disease. Atrial depolarization abnormalities were commoner in patients with pulmonary sarcoidosis.

(1) Background: We aimed to analyze temporal trends in hospitalization and in-hospital mortality (IHM) in patients with sarcoidosis in Spain from 2001−2020. (2) Methods: Using the Spanish National Hospital Discharge Database, we included patients (aged ≥ 20 years) hospitalized with a sarcoidosis code in any diagnostic field. (3) Results: We included 44,195 hospitalizations with sarcoidosis (56.34% women). The proportion of women decreased over time, from 58.76% in 2001 and 2002 to 52.85% in 2019 and 2020 (p < 0.001). The crude rates per 100,000 inhabitants increased by 4.02% per year among women and 5.88% among men. These increments were confirmed using Poisson regression analysis, which yielded an IRR of 1.03; 95% CI 1.01−1.04 for women and 1.04; 95% CI 1.02−1.06 for men. During the study period, no significant sex differences in IHM were recorded. Older age, COVID-19, respiratory failure, and the need for mechanical ventilation were independent predictors of IHM in men and women hospitalized with sarcoidosis, with IHM remaining stable over time. (4) Conclusions: The number of hospital admissions among patients with sarcoidosis in Spain increased threefold from 2001 to 2020. Although the incidence rates were higher in women, the trend followed that the incidence rates between sexes became closer. IHM was similar among men and women, with no significant change over time in either sex after multivariable analysis.