• Humans
• Lupus Erythematosus, Systemic/diagnosis
• Lupus Erythematosus, Systemic/therapy
• Lupus Erythematosus, Systemic/complications
• Child
• Age of Onset

• HEp-2 indirect immunofluorescence
• antinuclear antibodies
• recommendations

• Humans
• Antibodies, Antinuclear
• Autoimmune Diseases/diagnosis
• Fluorescent Antibody Technique, Indirect/methods
• Reference Standards
• Cell Line, Tumor

• Ro52/TRIM21
• Ro60
• SSA
• antinuclear antibodies
• autoimmunity
• extractable nuclear antigens

• Autoantibodies
• Autoimmunity
• Neonatal lupus
• Skin lesions

• anti-Ro60
• antibodies
• autoantibodies
• diagnostics
• laboratory medicine

• Antibodies, Antinuclear/blood
• Antibodies, Antinuclear/immunology
• Australia
• Autoantigens/blood
• Autoantigens/immunology
• Autoimmune Diseases/blood
• Autoimmune Diseases/immunology
• Enzyme-Linked Immunosorbent Assay
• Humans
• K562 Cells
• RNA, Small Cytoplasmic/blood
• RNA, Small Cytoplasmic/immunology
• Ribonucleoproteins/blood
• Ribonucleoproteins/immunology

• dermatology
• neonatal health

• dermatology
• materno-fetal medicine

• autoantibodies
• autoimmune diseases
• autoimmunity

• Antibodies, Antinuclear
• Autoimmune Diseases
• Consensus
• Fluorescent Antibody Technique, Indirect
• Humans
• Internationality

• RNA degradation
• Ro60
• Rsr
• Y RNA
• autoantigen
• noncoding RNA

To determine the measurement properties of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the paediatric adaptation of the Skindex29 (pSkindex27) when used in childhood-onset SLE (cSLE).

Patients with mucocutaneous involvement of cSLE were evaluated at the study entry and 6 months later. Besides the CLASI and pSkindex27, the Pediatric Quality of Life Inventory Generic Core scale (PedsQL-GC), its Rheumatology Module (PedsQL-RM), the SLE Disease Activity Index (SLEDAI) and the SLE Damage Index (SDI) were completed.

The CLASI and pSkindex27 had high internal consistency (both Cronbach α >0.82). Children were able to complete the pSkindex27, with self-report and caregiver proxy-reports showing excellent agreement (intraclass correlation coefficient=0.97). The CLASI Activity Score (CLASI-A) was strongly correlated with the mucocutaneous domain score of the SLEDAI as was the CLASI Damage Score (CLASI-D) with that of the SDI (both: Spearman correlation coefficients (r_s) >0.68). pSkindex27 summary scores were moderately correlated with those of the PedsQL-GC and PedsQL-RM (all: r_s>|0.51|), the CLASI-A and CLASI-D (both: r_s> 0.64), respectively. Patients who experienced a >50% improvement of the CLASI-A between study visits had significantly higher PedsQL-GC and pSkindex27 scores than those without improvement of mucocutaneous features.

Both CLASI and pSkindex27 are useful assessment tools in cSLE, active and chronic mucocutaneous lesions and their changes over time can be measured using the CLASI and the pSkindex27 can capture the impact of mucocutaneous involvement on patient health-related quality of life.

• clasi
• csle
• pediatrics
• skin
• skindex

• UL1 TR001425 NCATS NIH HHS
• Saudi Department of Health
• National Institutes of Health

Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Oral ulcers are classified as lupus erythematosus (LE) specific, where the lesional biopsy shows a unique pattern of mucosal change in LE, and LE nonspecific, where the ulcers and their histopathological findings can be found in other oral diseases. Here, the clinical manifestations, diagnosis and management of oral ulcers in JSLE patients are reviewed.

• neonatal lupus erythematosus
• antinuclear antibodies
• skin lesions

• Diagnosis, Differential
• Female
• Head
• Humans
• Infant
• Infant, Newborn
• Lupus Erythematosus, Cutaneous/congenital
• Lupus Erythematosus, Cutaneous/therapy
• Male

• Diagnosis
• Juvenile-onset systemic lupus erythematosus
• Lupus erythematosus nonspecific lesions
• Lupus erythematosus specific lesions
• Mucocutaneous lupus lesions
• Treatment

• Age of Onset
• Child
• Delayed Diagnosis/prevention & control
• Diagnosis, Differential
• Humans
• Lupus Erythematosus, Systemic/diagnosis
• Lupus Erythematosus, Systemic/epidemiology
• Lupus Erythematosus, Systemic/pathology
• Mouth Diseases/diagnosis
• Mouth Diseases/pathology
• Mouth Mucosa/pathology
• Skin/pathology
• Skin Diseases/diagnosis
• Skin Diseases/pathology

Systemic lupus erythematosus (SLE) typically affects women in their childbearing age, who have the same fertility rates as the healthy population. The effect of pregnancy on the disease and the effect of SLE on pregnancy and the fetus are highly important issues for the attending physician. Whether lupus flares are more frequent during pregnancy remains controversial. Among the possible effects of SLE on pregnancy are a greater number of abortions, fetal loss, pre-term deliveries and perinatal mortality. The newborn may be affected by the onset of neonatal lupus erythematosus (neonatal LE), either as a skin or blood disease, or by the presence of congenital heart block. The frequent association between SLE and antiphospholipid syndrome represents another risk situation for the mother and the product of conception. Multiples drugs used in SLE patients should be evaluated. Those with teratogenic potential should be withdrawn before pregnancy, and when necessary, appropriate medications should be indicated to treat the mother without compromising the safety of the baby. In conclusion, pregnancies in lupus patients represent a challenge for the physician and must be closely followed up and treated if necessary, during all trimesters and in the puerperium period, to improve outcome.

• Systemic lupus erythematosus
• Pregnancy
• Pre-eclampsia
• Lupus nephritis
• Neonatal lupus erythematosus
• Congenital heart block

Of the various collagen vascular diseases seen in pediatric age group, discoid lupus erythematosus, systemic lupus erythematosus, neonatal lupus erythematosus, juvenile dermatomyositis and childhood scleroderma are common and of practical importance to clinicians. Various treatment modalities of these conditions have been discussed at length. Of these, some are conventional and routine,while others are used in challenging situations of these diseases. Autologous stem cell transplant, biological therapies, intravenous immunoglobulin and narrow band ultraviolet B are among the latest therapeutic options for these difficult-to-treat conditions in children.

• Children
• collagen vascular diseases
• lupus erythematosus

Neonatal lupus erythematosus (NLE) refers to a clinical spectrum of cutaneous, cardiac, and systemic abnormalities observed in newborn infants whose mothers have autoantibodies against Ro/SSA and La/SSB. The condition is rare and usually benign and self-limited but sometimes may be associated with serious sequelae. We review the pathophysiology, clinical features, and management of infants with this condition. Neonates with NLE should be managed at a tertiary care center. Multidisciplinary team involvement may also be indicated. In mothers with anti-Ro/SSA and/or anti-La/SSB antibodies and infants with congenital heart block, the risk of recurrence in subsequent offspring is 17–25%. Therefore, careful monitoring of subsequent pregnancies with serial ultrasonography and echocardiography is essential.

• rna quality control
• environmental stress
• y rnas
• ribonucleoprotein complex

• Animals
• Autoantigens/chemistry
• Autoantigens/genetics
• Autoantigens/metabolism
• Deinococcus/genetics
• Deinococcus/metabolism
• Embryonic Stem Cells/metabolism
• Female
• Humans
• Mice
• Models, Molecular
• Nucleic Acid Conformation
• Oocytes/metabolism
• Phylogeny
• RNA Stability
• RNA, Bacterial/genetics
• RNA, Bacterial/metabolism
• RNA, Ribosomal, 5S/metabolism
• RNA, Small Cytoplasmic/chemistry
• RNA, Small Cytoplasmic/genetics
• RNA, Small Cytoplasmic/metabolism
• RNA, Small Nuclear/metabolism
• RNA, Untranslated/genetics
• RNA, Untranslated/metabolism
• Ribonucleoproteins/chemistry
• Ribonucleoproteins/genetics
• Ribonucleoproteins/metabolism
• Stress, Physiological
• Xenopus laevis

• 5R01GM048410 NIGMS NIH HHS
• R01 GM048410-17 NIGMS NIH HHS
• R01 GM073863 NIGMS NIH HHS
• R01GM073863 NIGMS NIH HHS
• R01 GM048410 NIGMS NIH HHS
• R01 GM073863-04S1 NIGMS NIH HHS
• R01 GM073863-04 NIGMS NIH HHS