Adult-onset Still’s disease (AOSD) is a systemic autoinflammatory disorder characterized by persistent fever at regular intervals, arthralgias or arthritis, rash, sore throat and neutrophilic leukocytosis.1,2 Significant elevation in ferritin levels is characteristic and tends to correlate with disease activity. Additional clinical features may include myalgias, lymphadenopathy, hepatosplenomegaly, serositis, myocarditis, abnormal liver function tests and development…
Understanding muscle pathology reports is important to best treat rheumatology patients. Peter Pytel, MD, shared pearls of wisdom specific to the autoimmune inflammatory myopathies and gave a detailed review of what rheumatologists need to know, during the 2022 ACR Education Exchange.
Albayda et al. describes a North American cohort of patients with dermatomyositis, reporting that small ubiquitin-like modifier activating enzyme (SAE) autoantibodies are clearly associated with this clinical disease. Patients with this clinical phenotype most commonly present with a rash first, followed by muscle involvement.
The FDA has granted a seven-year marketing exclusivity for Octagam 10%, a 10% solution of a human intravenous immunoglobulin (IVIG) that treats adults with dermatomyositis.
In July, the FDA approved the use of Octagam 10%, an intravenous immunoglobulin solution, to treat dermatomyositis in adults after an international study demonstrated the treatment’s safety and efficacy.
Dermatomyositis is an uncommon autoimmune condition involving skeletal muscle characterized by subacute onset of progressive weakness, intramuscular inflammatory infiltrates and the presence of myositis-specific autoantibodies.1 Immune-mediated myopathies may exert some pathogenic effects on the muscle tissue by targeting the microvasculature.1 Capillary inflammation, fragility and loss may contribute to heightened bleeding events in these patients. Here,…
Dermatomyositis (DM) is an idiopathic inflammatory myopathy involving proximal muscle weakness and skin rash. An associated increased risk of malignancy is well established.1 The most frequent malignancies are related to the ovary, endometrium, lung, gastrointestinal tract, prostate, breast and lymphatics.2 On rare occasions, DM has been reported with certain types of non-Hodgkin’s lymphoma, specifically cutaneous…
Interstitial lung disease accompanied by anti-melanoma differentiation-associated gene 5 positive dermatomyositis is often rapidly progressive and associated with poor prognosis. In this study, a combined immunosuppressive regimen of high-dose glucocorticoids, tacrolimus and intravenous cyclophosphamide proved more effective than treatment with high-dose glucocorticoids and stepwise addition of an immunosuppressant in a historical control group.
The ACR and EULAR have approved and released response criteria for adult and juvenile myositis, the result of a collaborative initiative that involved the International Myositis Assessment and Clinical Studies Group (IMACS) and the Paediatric Rheumatology International Trials Organisation (PRINTO). The decade-long collaboration was consensus driven, examined multiple clinical data sets and natural history studies,…