Pulmonary disease in polymyositis/dermatomyositis

Gastro-oesophageal reflux and aspiration in patients with advanced lung disease

“…A cohort study of 47 patients with polymyositis or dematomyositis and lung disease found that 18 (47%) had dysphagia, six (14%) had aspiration pneumonia, and oesophageal motility abnormalities were found by oesophagram or oesophageal motility studies in eight of 27 patients (30%). 28 Among a cohort of 125 patients with scleroderma (51 (41%) of whom had pulmonary involvement), 45 (36%) had oesophagitis and 61 of the 78 (78%) who had oesophageal pH measured had pathological GORD. 29 These data were confirmed by an ongoing study at our institution that has found that 18 of 23 patients (83%) referred for lung transplantation with connective tissue diseases (ie, scleroderma, mixed connective tissue disease or dematomyositis) had pathological GORD, and 18 (78%) with manometry data had aperistalsis or abnormal peristalsis (unpublished data).…”

Interstitial lung disease: are we missingformes frustesof connective tissue disease?

“…Although several histopathological patterns have been described in patients with idiopathic inflammatory myopathyassociated ILD, including organising pneumonia, usual interstitial pneumonia (UIP), organising diffuse alveolar damage and lymphocytic interstitial pneumonia [11,[15][16][17][18][19][20][21], nonspecific interstitial pneumonia (NSIP) is the most frequent histopathological pattern [11,20,22]. Idiopathic NSIP has been demonstrated to have a better prognosis than idiopathic pulmonary fibrosis (IPF) [23].…”

Persistent Pneumomediastinum and Dermatomyositis: A Case Report and Review of the Literature

“…Pulmonary manifestations are present in approximately 10 % of cases [1]. Although the association of interstitial lung disease [2] and lung cancer [3] with polymyositis and dermatomyositis is well established, the occurrence of a spontaneous pneumomediastinum in these conditions is relatively rare.…”