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

Abstract

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.

RNA helicase encoded by melanoma differentiation–associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive interstitial lung disease

“…Asia, rapidly progressive interstitial lung disease (ILD) sometimes develops; this condition is resistant to immunosuppressive treatment and is associated with poor outcomes (3,4).…”

Could C-reactive protein and erythrocyte sedimentation rate support monitoring of dermatomyositis and polymyositis activity?

“…In contrast to other studies available, [11][12][13][14][15][16][17][18][19][20][21][22][23][24] in this study ESR and CRP were assessed in newly diagnosed patients, with clinical and laboratory activity. In addition, the study did not include patients with factors that might have interfered with ESR and CRP values, such as infections, neoplasia associated myositis, overlap myositis syndromes, and patients submitted to glucocorticoid and/ or immunosuppressant treatment regimes.…”

“…Likewise, high CRP levels seem to be associated with neuropathy risk in this population. 23,24 However, these studies [11][12][13][14][15][16][17][18][19][20][21][22][23][24] are limited by the fact that ESR and/or CRP were analyzed in patients who had been submitted to chronic use of glucocorticoids and/or immunosuppressants.…”

“…8,9 However, the IMACS does not include erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP), which have been used indiscriminately as possible markers of DM and PM activity. [10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] High ESR levels have been correlated with neoplasia risk [11][12][13][14][15][16] and pulmonary involvement [17][18][19][20][21][22] in inflammatory myopathies. Likewise, high CRP levels seem to be associated with neuropathy risk in this population.…”

Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

“…Patients with CADM have a greater risk of developing ILD, especially prone to rapidly progressive lung disease corresponding to DAD [111,112]. Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies (also referred to as anti-CADM-140 antibodies) were identified in the serum from patients with CADM by firstly immunoprecipitation assays [113].…”