Pulmonary disease in polymyositis/dermatomyositis: a clinicopathological analysis of 65 autopsy cases.

Abstract

SUMMARY The clinical and autopsy records of 65 patients with either polymositis (24) or dermatomyositis (41) and pulmonary disease were reviewed. Pulmonary symptoms were recorded in 43 of the cases and included dyspnoea in 31, cough in 23, and chest pain in six. Interstitial lung disease was noted at autopsy in 27 patients; almost half of these had arthritis. Bronchopneumonia was found in 35 patients, 31 of these had received prednisone. Dysphagia was present in a similar proportion of patients with and without pneumonia. Pulmonary vasculitis was seen in five patients; pulmonary symptoms, arthritis, and raised erythrocyte sedimentation rate were present in four of these cases and all five had associated interstitial lung disease. Other pulmonary manifestations included pulmonary oedema, primary pulmonary malignancy, diffuse alveolar damage, fibrinous pleuritis, pulmonary emboli, and diaphragmatic atrophy. The mean survival after disease onset was 29 months but was much less for those with interstitial lung disease and pulmonary vasculitis. Data were abstracted from patients' clinical records on data collection forms and analysed. Autopsy data were similarly abstracted on data collection forms. All histopathology slides were reviewed by one of us (JTL). ResultsOf the 65 patients, 24 had PM and 41 had DM. There were 24 men and 41 women with a mean and median age at the time -of death of 57 years (range . Of the 24 men, eight had PM and 16 DM; the 41 women were divided into 16 with PM and 25 with DM. Pulmonary symptoms had been recorded in 43 of the cases and included dyspnoea in 31, cough in 23, and chest pain in six (Table 1).

Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis

“…Cellular interstitial pneumonia was also described [24], and may also well correspond to NSIP. A recent study by DOUGLAS et al [1] of 22 patients with DPM who underwent surgical lung biopsy concluded that the interstitial lung disease associated with DPM was characterised by a pathological NSIP pattern in 82% of cases.…”

“…Early reports of the interstitial lung disease associated with DPM suggested similarities with idiopathic pulmonary fibrosis [3][4][5]24], but it is likely that some of these cases may actually correspond to fibrosing NSIP, according to the current pathological criteria. Cellular interstitial pneumonia was also described [24], and may also well correspond to NSIP.…”

Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement: Table 1—

“…ILD, which develops in 23.1-65.0% of PM/DM patients [116,[118][119][120], is a major cause of death in this disease [116,119,[121][122][123]. ILD leads to life-threatening complications, such as ventilatory failure, secondary pulmonary arterial hypertension and cor pulmonale [116,117,121,122,[124][125][126][127], so the early detection of ILD is a high priority in PM/DM patients. However, in PM/DM patients a careful search for underlying malignancies is strongly advocated.…”

“…Pulmonary involvement in PM/DM may precede muscle and skin manifestations and includes respiratory muscle weakness, aspiration pneumonia, ILD, ventilatory insufficiency, infections and drug-induced pneumonia [114][115][116][117]. ILD, which develops in 23.1-65.0% of PM/DM patients [116,[118][119][120], is a major cause of death in this disease [116,119,[121][122][123]. ILD leads to life-threatening complications, such as ventilatory failure, secondary pulmonary arterial hypertension and cor pulmonale [116,117,121,122,[124][125][126][127], so the early detection of ILD is a high priority in PM/DM patients.…”

Polimiosite associada à arterite linfocítica do sistema nervoso central

“…6,7 Among the extramuscular manifestations of polymyositis, pulmonary involvement is the most common. 3,10,11 On the other hand, complications of the central nervous system (CNS) are rarely reported. 12 …”