We report three patients with inflammatory myopathy who presented clinically with weakness and wasting of only one limb. The myopathy progressed over 6 months and 5 years, respectively, in two patients and was stable after 8 years in the third patient. One patient had a skin rash. Serum CK was elevated in the two patients with progressive disease. Electromyography showed brief duration, small amplitude motor unit potentials and fibrillations in the affected limbs. Muscle biopsy revealed variable fiber size, degenerating and regenerating fibers, and inflammatory foci. Vasculitis was seen in the patient with skin lesions and marked fibroblastic proliferation in the patient with the most chronic course. Immunosuppressive therapy has arrested the progression in the two patients treated; both have regained strength.
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Int Ophthalmol
January 2025
Department of Ophtalmology, Dokuz Eylul University School of Medicine, Izmir, Turkey.
Purpose: This retrospective study aimed to characterize the clinical features, histopathological findings, and treatment outcomes of patients diagnosed with orbital inflammatory disease (OID) co-managed by the rheumatology and ophthalmology departments in a tertiary hospital.
Methods: Medical records of 14 patients with OID were analyzed. Data on demographics, clinical presentation, laboratory investigations, radiological imaging, histopathological results, treatment regimens, and disease outcomes were collected and reviewed.
Vet Clin North Am Equine Pract
January 2025
Large Animal Clinical Sciences, College of Veterinary Medicine, Michigan State University, East Lansing, MI, USA.
Several inflammatory myopathies have an infectious or immune-mediated basis in the horse. Myosin heavy chain myopathy is caused by a codominant missense variant in MYH1 and has 3 clinical presentations: immune-mediated myositis, calciphylaxis, and nonexertional rhabdomyolysis in Quarter Horse-related breeds. An infarctive form of purpura hemorrhagica affects numerous breeds, presenting with focal firm, painful muscle swelling, and subsequent infarction of multiple tissues.
View Article and Find Full Text PDFJ Transl Autoimmun
June 2025
Medical University of Vienna, Borschkegasse 8a, 1090, Vienna, Austria.
Autoimmune rheumatic diseases (ARDs) are a heterogeneous group of conditions characterized by excessive and misdirected immune responses against the body's own musculoskeletal tissues. Their exact aetiology remains unclear, with genetic, demographic, behavioural and environmental factors implicated in disease onset. One prominent hypothesis for the initial breach of immune tolerance (leading to autoimmunity) is molecular mimicry, which describes structural or sequence similarities between human and microbial proteins (mimotopes).
View Article and Find Full Text PDFMuscle Nerve
January 2025
Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Introduction: Mixed connective tissue disease (MCTD) patients often have myositis, however, myopathological and clinical data for MCTD are limited. Recent reports have shown that the pathology of MCTD myositis resembles that of immune-mediated necrotizing myopathy (IMNM), whereas earlier reports described perifascicular atrophy or inflammatory infiltrates predominantly in the perivascular region in MCTD myositis. We aim to describe the clinical and myopathological features of MCTD myositis.
View Article and Find Full Text PDFMil Med Res
January 2025
Department of Medicine, Medicine Service Line-Rheumatology, VA Pittsburgh Health Care System, University Drive Campus, Pittsburgh, PA, 15240, USA.
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