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…
For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics. Pneumomediastinum & Ulcerative Cutaneous Dermatomyositis These images depict a 22-year-old woman with a recent diagnosis of anti-melanoma differentiation-associated gene 5 (MDA-5) antibody dermatomyositis who sought evaluation of worsening skin rashes and pleuritic…
For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics. Unilateral Heliotrope Rash in Anti-MDA-5 Antibody Dermatomyositis These images depict a 36-year-old patient with arthralgias, dyspnea and a unilateral heliotrope rash (image A). A unilateral heliotrope rash is a distinctive, but rare,…
For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics. Chronic Facial Ulcers in Anti-Melanoma-Differentiation-Associated Gene 5 (MDA-5) Antibody Amyopathic Dermatomyositis These images depict a 27-year-old patient who developed erythematous violaceous lesions over his upper chest, face and scalp over six months….
Since the New England Journal of Medicine publication on intravenous immune globulin (IVIG) for dermatomyositis late last year, the data have proved a major lift for patient care, offering crucial new insights into how best to manage patients and clues about how to minimize risks.1 The ProDERM trial results also seem to have opened up…
Localized Scleroderma in Anti-NXP2-Antibody Positive Dermatomyositis A 67-year-old woman presented with erythematous, indurated skin on her left flanks. She had been diagnosed with dermatomyositis one year earlier when proximal muscle weakness, dysphagia and skin rash developed (see Figure A). Tests at the time showed the presence of anti-NXP2 and anti-Ro52 antibodies, as well as pathological…
In medicine, as in advertising, pictures can be worth a thousand words. From arthritis to vasculitis, imaging studies have been variably employed to aid in the diagnosis, treatment, risk stratification and prognostication of patients with rheumatic and musculoskeletal disorders. The same holds true with the idiopathic inflammatory myopathies (IIM), in which the clinical utility is…
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.