Successful treatment of rapidly progressive interstitial lung disease complicated by a refractory pneumomediastinum in a patient with anti‐MDA5 antibody‐positive dermatomyositis

Cytokine profiles in polymyositis and dermatomyositis complicated by rapidly progressive or chronic interstitial lung disease.

OBJECTIVE PM and DM are often complicated by interstitial lung disease (ILD). In this study we aimed to evaluate various serum cytokines in patients with PM/DM with ILD so as to clarify the differences in pathophysiology between anti-melanoma differentiation-associated gene 5 antibody-associated ILD (anti-MDA5-ILD) and anti-aminoacyl tRNA synthetase antibody-associated ILD (anti-ARS-ILD). MET...

Subcutaneous Emphysema, Pneumomediastinum and Pneumothorax in a Patient with Dermatomyositis

Introduction:Spontaneous pneumomediastinum, pneumothorax, and subcutaneous emphysema are rare, but serious complications of inflammatory myopathies and occur more commonly in DM than PM. complications of dermatomyositis (DM) and polymyositis (PM), both of which can be fatal. Case Report:A 20-year-old woman was admitted with neck pain, dyspnea, cough, and fever. She had been diagnosed with derma...

Rapidly progressive interstitial lung fibrosis in a patient with amyopathic dermatomyositis and anti‑MDA5 antibodies.

A Case of Sarcoidosis with Interstitial Lung Disease Mimicking Clinically Amyopathic Dermatomyositis and Rapidly Progressive Interstitial Lung Disease

Here, we report a patient with sarcoidosis who developed edematous erythema and interstitial lung disease. At the initial visit, clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD) was suspected because he had progressive dyspnea but no muscle weakness. The presence of anti-CADM-140/MDA5 autoantibodies was immediately assessed to facilitate a...

Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were pr...