• anti-tnf
• inflammatory bowel disease
• infliximab
• sarcoidosis
• upadacitinib

• Crohn Disease
• DIAGNOSIS
• Pathology, Surgical

• Humans
• Crohn Disease/pathology
• Female
• Male
• Myenteric Plexus/pathology
• Adult
• Middle Aged
• Young Adult
• Aged
• Adolescent
• Intestinal Mucosa/pathology
• Giant Cells/pathology

• Anti-inflammatory therapy
• Clinical trials
• Efzofitimod
• Health-related quality of life (HRQoL)
• Multi-organ
• Multi-system
• Novel therapies
• Sarcoidosis
• Therapeutic pathways

• Humans
• Sarcoidosis/drug therapy
• Sarcoidosis/diagnosis
• Drug Development
• Immunosuppressive Agents/therapeutic use
• Quality of Life
• Treatment Outcome

• Humans
• Anthracosis/pathology
• Anthracosis/epidemiology
• Coal Mining
• Retrospective Studies
• Male
• Middle Aged
• United States/epidemiology
• Occupational Exposure/adverse effects
• Silicon Dioxide/adverse effects
• Dust/analysis
• Aged
• Lung/pathology
• Adult

• chest imaging
• differential diagnosis
• granulomatous lung diseases (GLDs)
• high-resolution computed tomography (HRCT)
• radiology

Sarcoidosis is a systemic granulomatous inflammatory disease of unknown etiology. It affects the lungs in over 90% of patients yet extra-pulmonary and multi-organ involvement is common. Spontaneous remission of disease occurs commonly, nonetheless, over 50% of patients will require treatment and up to 30% of patients will develop a chronic progressive non-remitting disease with marked pulmonary fibrosis leading to significant morbidity and death. Guidelines outlining an immunosuppressive treatment approach to sarcoidosis were recently published, however, the strength of evidence behind many of the guideline recommended drugs is weak. None of the drugs currently used for the treatment of sarcoidosis have been rigorously studied and prescription of these drugs is often based on off-label” indications informed by experience with other diseases. Indeed, only two medications [prednisone and repository corticotropin (RCI) injection] currently used in the treatment of sarcoidosis are approved by the United States Food and Drug Administration. This situation results in significant reimbursement challenges especially for the more advanced (and often more effective) drugs that are favored for severe and refractory forms of disease causing an over-reliance on corticosteroids known to be associated with significant dose and duration dependent toxicities. This past decade has seen a renewed interest in developing new drugs and exploring novel therapeutic pathways for the treatment of sarcoidosis. Several of these trials are active randomized controlled trials (RCTs) designed to recruit relatively large numbers of patients with a goal to determine the safety, efficacy, and tolerability of these new molecules and therapeutic approaches. While it is an exciting time, it is also necessary to exercise caution. Resources including research dollars and most importantly, patient populations available for trials are limited and thus necessitate that several of the challenges facing drug trials and drug development in sarcoidosis are addressed. This will ensure that currently available resources are judiciously utilized. Our paper reviews the ongoing and anticipated drug trials in sarcoidosis and addresses the challenges facing these and future trials. We also review several recently completed trials and draw lessons that should be applied in future.

• clinical trials and clinical trial design
• fibrotic pulmonary sarcoidosis
• interstitial lung disease
• novel therapies
• patient centered and patient partners in research
• progressive pulmonary fibrosis
• pulmonary sarcoidosis
• therapeutic pathways

• McIntyre Powder
• clinical phenotype
• granulomatous disease
• hard-rock miner
• respirable crystalline silica
• sarcoidosis

• multiple pulmonary nodule
• pulmonary neoplasm
• sarcoidosis
• vasculitis

• Aged
• Female
• Granuloma/diagnosis
• Humans
• Lung/diagnostic imaging
• Lung Neoplasms
• Multiple Pulmonary Nodules
• Necrosis
• Positron Emission Tomography Computed Tomography
• Rare Diseases
• Sarcoidosis, Pulmonary/diagnosis
• Sarcoidosis, Pulmonary/surgery
• Thoracic Surgery, Video-Assisted
• Vasculitis, Central Nervous System/diagnosis
• Vasculitis, Central Nervous System/surgery

• Granuloma
• Humans
• Nurses
• Sarcoidosis/diagnosis
• Sarcoidosis, Pulmonary/diagnosis
• Skin Diseases

Sarcoidosis is an important member of the family of granulomatous lung diseases. Since its recognition in the late 19th century, sarcoidosis has been thought of as a disease of unknown cause. Over the past 20 years, this paradigm has been shifting, more rapidly in the past 10 years. Epidemiologic studies, bolstered by case reports, have provided evidence of causal associations between occupational exposure to specific agents and sarcoidosis. Pathogenesis has been more clearly defined, including the role of gene-exposure interactions. The use of in vitro lymphocyte proliferation testing to detect sensitization to inorganic antigens is being examined in patients with sarcoidosis. These antigens include silica and certain metals. Results of studies to date show differences in immunoreactivity of occupationally exposed sarcoidosis cases compared with control cases, suggesting that lymphocyte proliferation testing may prove useful in diagnosing work-related disease. This review discusses recently published findings regarding associations between occupational exposure to silica and silicates, World Trade Center dust, and metals and risk for sarcoidosis, as well as advances in the development of diagnostic tools. Not all cases of sarcoidosis have an identified cause, but some do. Where the cause is occupational, its recognition is critical to enable effective treatment through removal of the affected worker from exposure and to inform intervention aimed at primary prevention.

• World Trade Center dust
• genetics
• lymphocyte proliferation test
• sarcoidosis
• silica

• Granulomatous lung disease
• High-resolution computed tomography
• Lung parenchyma
• Non-infectious granulomatosis
• Radiography

• Sarcoidosis
• adalimumab
• infliximab
• repository corticotropin injection
• rituximab
• tumor necrosis factor

To evaluate the Right Upper Lobe Bronchus Angle (RUL-BA) on chest CT in patients with Stage 4 sarcoidosis and compare to others with non-fibrotic sarcoidosis.

IRB approval was obtained for review of all chest CT scans performed from January 2015 through December 2017 that contained the word sarcoidosis using the computer program Montage. The most recent CT scans of 633 people were reviewed. The patients’ age and sex at the time of their most recent CT scan were recorded. The radiographic diagnosis and the Right Upper Lobe Bronchus Angle (RUL-BA) were determined by a chest radiologist with 20 years of experience.

The RUL-BA increased with Stage 4 sarcoidosis, measuring on average 104 degrees, compared to the average angle of 88 degrees for those without fibrotic sarcoid. More often men’s CT scans exhibited the earlier stages of sarcoidosis, and a higher number of women’s scans showed fibrotic sarcoidosis. As would be expected, scans with advanced disease were typically from older patients; however, there was no correlation between age and degree of fibrosis as measured by increasing RUL-BA.

The RUL-BA assists radiologists in differentiating fibrotic sarcoidosis from non-fibrotic sarcoidosis. Further research will determine if the RUL-BA measurement can help differentiate fibrotic sarcoid from other fibrotic lung diseases and if the angle can be used to follow disease progression. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (2): 99-103)

• chest CT scan
• pulmonary fibrosis
• sarcoid

Sarcoidosis is an idiopathic, chronic, multisystem, granulomatous, inflammatory disease involving almost all organs. Sarcoidosis can occur with an atypical presentation of hepatosplenic involvement, like in the case of our patient. In this case report, we present a rare case of extrapulmonary sarcoidosis with isolated involvement of the liver and spleen in a 39-year-old Caucasian female. There is a possibility of this isolated involvement of an organ in the complete absence of pulmonary disease, which makes the diagnosis of sarcoidosis very difficult as it is usually not suspected. Ultrasound and CT are important in ruling out other differential diagnoses, but a definitive diagnosis is possible only on histological examination, differentiating sarcoid lesions from tuberculosis, primary biliary cirrhosis, metastasis, malignancy, and other granulomatous infections or diseases. Hence, the most credible criterion for diagnosis remains histology. After diagnosis, regular follow-up for systemic manifestations is recommended. Asymptomatic patients with hepatosplenic sarcoidosis have a good prognosis without any medical intervention, while patients with abnormal labs or symptoms must commence treatment.

Small pulmonary nodules are tissue shadows and thoracoscopic segmentectomy in China is still at the exploratory stage with limited application.

To evaluate the efficacy of thoracoscopic anatomical segmentectomy for small pulmonary nodules.

Medical records of 86 patients with small pulmonary nodules treated at our hospital between August 2016 and October 2019 were retrospectively analyzed; 40 cases who underwent thoracoscopic lobectomy were set as a reference group, and 46 cases who underwent thoracoscopic anatomical segmentectomy were set as an observation group. Preoperative and postoperative parameters were measured in both groups, including the percentage of forced expiratory volume in the first second (FEV₁%), the percentage of forced vital capacity (FVC%), and the FEV₁/FVC ratio (FEV₁/FVC). Patients with positive pathological diagnosis received tests for neuron-specific enolase, carbohydrate antigen 125 (CA125), CA19-9, and squamous cell carcinoma antigen. Intraoperative bleeding volume, drainage volume, the number of dissected lymph nodes, drainage time, hospital stay, treatment cost, postoperative complications, and postoperative pain condition were compared between the two groups.

No significant difference was observed in the results of four serum tumor marker (CA125, CA19-9, squamous cell carcinoma antigen, and neuron-specific enolase), the number of dissected lymph nodes, treatment cost, or preoperative pulmonary ventilation index between the two groups. Intraoperative bleeding volume, drainage volume, drainage time, hospital stay, and visual analogue scale score were significantly lower in the observation group (P < 0.05). The results of FEV₁%, FVC%, and FEV₁/FVC were significantly higher in the observation group (P < 0.05).

The efficacy of thoracoscopic anatomical segmentectomy and lobectomy for small pulmonary nodules shows no significant difference in terms of lesion removal, but anatomical segmentectomy is less invasive with fewer postoperative complications and less influence on lung function.

• Thoracoscopy
• Small pulmonary nodules
• Anatomical segmentectomy
• Clinical efficacy
• Lobectomy