Pulmonary Complications of Dermatomyositis
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
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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]. Pulmonary disease in DM patients has been recognized for many years, but was not considered as a common manifestation of the disease [14]. In a retrospective study of PM/ DM, only ten of 213 patients (4.7%) had radiologic evidence of parenchymal lung disease [15]. In the study by Bohan et al. of 153 computer-analyzed PM/DM patients, pulmonary disease was not reported [16]. In another study of 42 patients with PM/DM, the radiologic evidence of ILD [UIP, AIP] was found in 10% of the cases [10]. Reviewing the 65 published cases of ILD, including their own data, they concluded that ILD were associated L.A. Dourmishev and A.L. Dourmishev, Dermatomyositis: Advances in Recognition, Understanding and Management, © Springer-Verlag Berlin Heidelberg 2009
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11 Pulmonary Complications of Dermatomyositis
with IIMs in 55% of the cases with PM and 45% with DM [10], which was confirmed later by others [7]. Reliable epidemiological data [7, 10] for association of ILD and DM can be summarized as follow: 1. ILD is associated in 45% of
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