Pulmonary pathology in association with connective tissue disorders

Abstract

Patients with connective tissue disorders (CTD) often have associated pleuropulmonary lesions, and these are major causes of morbidity and mortality. CTD may involve all compartments of the respiratory system, including respiratory muscles, pleura and lung parenchyma, with diffuse interstitial pneumonia and fibrosis being common within the parenchyma. During the last decade, non-specific interstitial pneumonia (NSIP) has been recognized as a specific histological pattern, and the definition of usual interstitial pneumonia (UIP) has been narrowed and refined, this having significant impact on the diagnosis of interstitial pneumonias in CTD. Recent data suggest that a pattern of NSIP is the most common pattern of interstitial pneumonia in CTD such as scleroderma, dermatomyositis/polymyositis, rheumatoid arthritis, and Sjoegren's syndrome. This is consistent with the more favourable prognosis seen with interstitial pneumonias associated with CTD, in comparison to patients with idiopathic pulmonary fibrosis who, with rare exceptions, have a histological pattern of UIP.