An Unusual Presentation of Amyopathic Dermatomyositis Associated With Fatal Interstitial Lung Disease

Neuhaus, Isaac M.; Connolly, M. Kari

doi:10.1001/archderm.142.1.113

Cited by 9 publications

(1 citation statement)

References 7 publications

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“…Dermatomyositis can occur as an idiopathic process, as an association with malignancy, as a drug reaction or in association with another connective tissue disease [ 3 ]. Additionally, dermatomyositis can be complicated by ulcerated skin lesions [ 4 ] or interstitial lung disease, which is often associated with poor prognosis [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

Intravenous Immune Globulin in Amyopathic Dermatomyositis - Report of Two Cases and Review of the Literature

Cafardi

Sami²

2015

TORJ

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Amyopathic dermatomyositis (ADM) is a rare subtype of dermatomyositis which is often recalcitrant to immune suppressing treatments. Intravenous immunoglobulin (IVIG) has been used in the treatment of refractory dermatomyositis. We present two patients with severe ADM, who were treated with IVIG at 2 g/kg every four weeks. Both patients had a successful response and were able to taper the dosage of prednisone. We present both cases in describing IVIG as a rescue and maintenance steroid-sparing agent in the treatment of severe refractory ADM. We also review the treatment of refractory ADM with IVIg in the English literature.

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Section: Discussionmentioning

confidence: 99%

Intravenous Immune Globulin in Amyopathic Dermatomyositis - Report of Two Cases and Review of the Literature

Cafardi

Sami²

2015

TORJ

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Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study

et al. 2007

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The aim of the study was to investigate the characteristics of adult clinically amyopathic dermatomyositis (CADM) with rapid progressive interstitial lung disease (ILD). Hospitalized patients with dermatomyositis (DM) and polymyositis (PM) between 1998 and 2005 in the Shanghai Renji Hospital were retrospectively studied. One hundred and forty-five patients were classified into CADM, classic DM or PM according to the modified Sontheimer's definition or Bohan-Peter's classification criteria. They were further stratified based on the presence or absence of clinical ILD. The Kaplan-Meier survival analysis and COX regression were performed. The predictive factors for ILD and other clinical properties of CADM-ILD were explored. The presence of clinical ILD was a significant risk factor for the poor outcome of DM/PM (OR = 4.237, CI 95%: 1.239-14.49, p = 0.021). Other risk factors are the presence of rashes and elevated urea nitrogen. Patients with DM/PM complicated by ILD had different clinical courses. Patients with CADM-ILD showed a rapidly progressive pattern with 6-month survival rate of 40.8%. The DM-ILD manifested a progressive pattern with a 5-year survival rate of 54%, while PM-ILD was chronic with 5- and 10-year survival rate of 72.4% and 60.3%, respectively. Better preserved muscle strength, elevated erythrocyte sedimentation rate, and hypoalbuminemia may herald ILD in DM/PM. Patients with CADM-ILD who later died had lower PO(2), higher lactate dehydrogenase, and prominent arthritis/arthralgia compared with those who survived. The presence of antinuclear antibody seems to be protective. Rapid progressive CADM-ILD is refractory to conventional treatment. ILD is a common complication in over 40% of our hospitalized DM/PM cohort and is also a prominent prognostic indicator. CADM is a special phenotype of DM/PM. CADM-ILD, which is usually rapidly progressive and fatal, requires further investigation.

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Amyopathic dermatomyositis developing rapidly progressive interstitial lung disease with elevation of anti-CADM-140/MDA5 autoantibodies

et al. 2011

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Anti-clinically amyopathic dermatomyositis (CADM)-140/MDA5 autoantibodies are specifically detected in patients with dermatomyositis and are known to have a strong association with rapidly progressive interstitial lung disease (RP-ILD). Here we report an amyopathic dermatomyositis (ADM) patient who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 titer. Respiratory symptoms gradually improved, and anti-CADM-140/MDA5 titer decreased in parallel to below the cutoff level. It may be useful to quantify CADM-140-specific autoantibodies for monitoring disease activity in patients with ADM and RP-ILD.

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An Unusual Presentation of Amyopathic Dermatomyositis Associated With Fatal Interstitial Lung Disease

Cited by 9 publications

References 7 publications

Intravenous Immune Globulin in Amyopathic Dermatomyositis - Report of Two Cases and Review of the Literature

Intravenous Immune Globulin in Amyopathic Dermatomyositis - Report of Two Cases and Review of the Literature

Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study

Amyopathic dermatomyositis developing rapidly progressive interstitial lung disease with elevation of anti-CADM-140/MDA5 autoantibodies

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