Why was this study done? Juvenile dermatomyositis (JDM) is the most common type of idiopathic inflammatory myopathy in childhood, and most patients have a chronic disease course requiring prolonged administration of systemic glucocorticoids and immunosuppressive agents. The initial management for patients with moderately severe JDM is relatively standardized, typically including methotrexate and systemic glucocorticoids with…
Type I interferons (IFNs) play an important role in the immunopathogenesis of cutaneous lupus erythematosus and dermatomyositis skin inflammation, which could potentially be treated with IFN-targeted therapies.
Inclusion body myositis (IBM) is a slowly progressing muscle disease of unknown cause that currently has no effective treatment. IBM is the most common inflammatory myopathy in older individuals, with a rising prevalence of 18.2 per 100,000 in adults older than 50.1,2 The disease characteristically affects the quadriceps and finger flexors, and in later stages…
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….
For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics. These images won the Europe and Central Asia region. Glycogen Storage Disease Type V Diagnosed Using Imaging, Biopsy & Genetic Testing These images depict a 55-year-old patient with suspected polymyositis who presented…
For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics. The images selected as Best Overall by the judges this year come from North America. Systemic Light Chain (AL) Amyloidosis Masquerading as Inflammatory Myositis These images depict an 81-year-old man who presented…
SAN DIEGO—The inflammatory myopathies are a heterogeneous group of conditions that, although discovered decades ago, continue to challenge rheumatologists in terms of their myriad clinical presentations. In Flexing Strong Science on Weak Muscles: Genetics, Genomics and Autoantibodies in Myositis, two speakers provided exceptionally helpful insights into these conditions, using novel tools for analysis. 6 Types…