Interstitial lung disease associated with amyopathic dermatomyositis: Review of 18 cases

Abstract

Interstitial lung disease (ILD) associated with amyopathic dermatomyositis (ADM) is a rare and sometimes fatal condition whose clinical features are not well understood. The goal of this study was to clarify the characteristics of ILD based on its development. Eighteen patients diagnosed with ILD associated with ADM were assigned to 1 of 2 groups: (1) a rapidly progressing group, which included patients who developed abnormal lung findings within 1 month of being diagnosed with ADM (n=9); or (2) a slowly progressing group, which including patients who developed lung findings greater than 1 month after diagnosis of ADM (n=9). Serum creatine phosphokinase and C-reactive protein levels were higher in the rapidly progressing group than in the slowly progressing group. Further, arterial pH was higher and PaO(2)/F(I)O(2) was lower in the rapidly progressing group than in the slowly progressing group. On thoracic high-resolution CT, traction bronchiectasis was present in 4 of the 9 rapidly progressing patients but not in any patients of the slowly progressing group. All 9 slowly progressing patients survived with proper treatment, but only 4 of the 9 rapidly progressing patients survived. In ADM, appropriate investigations are likely required for the early diagnosis of ILD. Our data suggest that ILD associated with ADM can be classified into 2 clinical subtypes based on the time course of pulmonary involvement. Patients with rapid progression in respiratory symptoms should undergo intensive treatment as soon as possible to promote favorable outcomes.

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.…”

“…Ideura et al 24 suggested a prognostic value for elevated CRP level correlating with interstitial lung disease in amyopathic DM.…”

“…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.…”

“…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.…”

Rapidly Progressive Interstitial Lung Disease Associated with Dermatomyositis Treated with Combination of Immunosuppressive Therapy, Direct Hemoperfusion with a Polymyxin B Immobilized Fiber Column and Intravenous Immunoglobulin

“…Secondly, the candidates of IVIG and PMX-DHP need to be validated. Cases with predicting factors for poor ILD prognosis in PM/DM may be good candidates; factors such as acute onset of ILD, reduced forced vital capacity (FVC) and DLCO values, older age, a pattern of usual interstitial pneumonia on highresolution computer tomography (HRCT), steroid-refractory ILD and increased ferritin (13)(14)(15)(16). Thirdly, the timing to administer PMX-DHP and IVIG remains controversial.…”