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Dermatomyositis pp 79–88Cite as

Pulmonary Complications of Dermatomyositis

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In addition to the primary disturbance of the skeletal muscles, extramuscular manifestations may be prominent in patients with idiopathic inflammatory myopathy [1]. Pulmonary complications of PM/DM are divided into primary and secondary. PM and DM may be associated with a diffuse interstitial lung disease (ILD). Initially described by Mills and Mathews in 1956, ILD has been reported as a PM/DM complication in between 5% and 30% of the cases [2]. The presence of ILD in patients with myositis affects the prognosis, and often has an influence on the choice of immunosuppressive treatment [3]. Recent studies show that lung involvement in PM/DM varies between 9% and 46%, depending on the diagnostic method [3–5], but pulmonary function is abnormal in about 40% of DM patients [6, 7]. Chest computed tomography (CT) scan can identify earlier lung involvement than a routine chest radiography [3, 6]. Interstitial lung disease is a primary process seen in DM and PM, and is more frequent in patients with esophageal involvement [8].

Six different entities have been described in patients with ILDs: bronchiolitis obliterans organizing pneumonia (BOOP); diffuse alveolar damage (DAD); nonspecific interstitial pneumonia (NSIP); usual interstitial pneumonia (UIP); pulmonary capillaritis and alveolar hemorrhage (PCAH); and acute interstitial pneumonia (AIP). Early descriptions of the ILD associated with PM/DM suggested that it shared many features with idiopathic pulmonary fibrosis (IPF) [9, 10], although some patients had histological findings described as DAD, BOOP, UIP, or a nonclassifiable interstitial pneumonia (NCIP) [11, 12]. The frequency of the HLA-DRB1*1302-DQA1*0102-DQB1*0604 haplotype in Japanese DM patients was significantly higher than in healthy controls (42.1% vs 17.7%), and in PM patients (42.1% vs 9.4%). Furthermore, the frequency of the HLA-DRB1*0405-DQA1*03-DQB1*0401 haplotype was higher in PM patients with ILD than in controls (50.0% vs 17.7%), and in PM without ILD (50.0% vs 5.5%) [13].

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(2009). Pulmonary Complications of Dermatomyositis. In: Dermatomyositis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-79313-7_11

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