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Background: Clinically amyopathic dermatomyositis (CADM) is a rare subtype of idiopathic inflammatory myositis often linked with the presence of autoantibodies targeting melanoma differentiation-associated protein 5 (MDA5). Patients with CADM are at increased risk of developing rapidly progressing interstitial lung disease, which significantly increases both morbidity and mortality compared to other forms of inflammatory myopathies. While there is no standardized treatment regimen, current therapeutic strategies are generally focused on combination immunosuppressive therapies.

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Two case reports of MDA5-type seronegative dermatomyositis.

SAGE Open Med Case Rep

December 2024

Division of Dermatology, Department of Medicine, University of Alberta, Edmonton, AB, Canada.

Dermatomyositis (DM) is an autoimmune idiopathic inflammatory myopathy with characteristic dermatologic manifestations. Myositis-specific autoantibodies (MSAs) delineate DM subtypes and their prognoses. Uncommonly, patients present with distinct clinical features of DM, including photosensitive dermatitis, heliotrope rash, Gottron's papules, and nailfold changes; however, their autoimmune serology is negative for expected MSAs.

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The skin ulcers sometimes appear in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM), and usually associates with disease activity. Here, we report a case of 41-year-old woman with anti-MDA5 antibody-positive DM, who developed refractory skin ulcers during the remission induction therapy, which were proven to be associated with clinically silent Staphylococcus aureus bacteremia with septic thrombi in her lung. The patient was referred to our hospital for the treatment of amyopathic DM with interstitial lung disease.

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Pulmonary manifestations of the idiopathic inflammatory myopathies in a South African population.

Afr J Thorac Crit Care Med

October 2024

Department of Medicine, School of Clinical Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.

Article Synopsis
  • Pulmonary complications are a significant health issue for patients with idiopathic inflammatory myopathies (IIMs) in South Africa, causing high morbidity and mortality rates.
  • A study reviewed clinical records of adult IIM patients with respiratory issues in a tertiary care facility, revealing that most patients suffered from dermatomyositis and exhibited symptoms like dyspnoea and dry cough.
  • Interstitial lung disease (ILD) emerged as the most common complication, with associations found between respiratory symptoms and poorer lung function outcomes over time.
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Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by progressive muscle weakness and distinctive cutaneous findings. The exact incidence and prevalence of DM in the general population is largely unknown, and data on demographic and clinical features in patients in Canada are lacking. This study aimed to comprehensively evaluate the patients with DM in Southwestern Ontario, Canada.

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