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.

Download full-text PDF

Source
http://dx.doi.org/10.1002/mus.880070210DOI Listing

Publication Analysis

Top Keywords

inflammatory myopathy
8
patient skin
8
focal inflammatory
4
myopathy report
4
report three
4
patients
4
three patients
4
patients inflammatory
4
myopathy presented
4
presented clinically
4

Similar Publications

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.

View Article and Find Full Text PDF

Myosin Heavy Chain Myopathy and Immune-Mediated Muscular Disorders.

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 PDF

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 PDF

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 PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!