Other Autoimmune Diseases

Abstract

Autoimmune diseases described herein include systemic lupus erythematosus (SLE), dermatomyositis/polymyositis (DM/PM), Sjögren syndrome (SS), and mixed connective tissue disease (MCTD). SLE predominantly affects women of reproductive age. Although pleural involvement is the most common thoracic manifestation, other manifestations include pneumonia, diffuse alveolar hemorrhage and lupus pneumonitis. Interstitial lung disease in patients with SLE include non-specific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). DM/PM affects the skeletal muscle and may frequently result in hypoventilation and respiratory failure (respiratory muscle involvement) and aspiration (laryngeal involvement). Interstitial lung disease is also frequent, and NSIP and organizing pneumonia are the most common patterns. SS typically affects women in the 4th to 5th decades of life. Classic symptoms include xerophtalmia and xerostomia. Interstitial lung disease is among the most common thoracic manifestations; and although NSIP, UIP, organizing pneumonia and amyloidoisis can occur, lymphocytic interstitial pneumonia (LIP) is a characteristic form of interstitial lung disease in SS. MCTD combines clinical features of RS, SLE, PSS and PM/DM. Thoracic involvement typically manifests with pulmonary hypertension and interstitial lung disease (NSIP, UIP and LIP). Pulmonary hypertension can occur in any autoimmune disease and is often associated with a worse prognosis. Chest radiography and thin-section chest CT (or HRCT) are the imaging modalities of choice to detect and assess thoracic manifestations of autoimmune disease.