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Chest Imaging

Mixed connective tissue disease

condition associated with clinical and laboratory findings overlapping those of progressive systemic sclerosis (PSS), systemic lupus erythematosus, polymyositis pulmonary involvement and rheumatoid arthritis. It is characterized by the presence of high titres of a circulating antinuclear, antiribonucleoprotein antibody that is uncommon in other connective tissue diseases. Mixed connective tissue disease is commonly associated with radiological and functional evidence of interstitial lung disease and/or pleural effusion. Pulmonary vasculitis with pulmonary arterial hypertension and pulmonary haemorrhage are also associated with mixed connective tissue disease. More than two thirds of patients with mixed connective tissue disease have abnormal pulmonary function tests, but chest radiographic abnormalities are less frequent, visible in about 20%. The interstitial lung disease of mixed connective tissue disease (Fig.1) appears identical to that of usual interstitial pneumonia (UIP) or interstitial pulmonary fibrosis (IPF) on histological examination, radiographs and high resolution CT.

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Fig.1

HRCT in a patient with mixed connective tissue disease showing bilateral peripheral areas of ground-glass attenuation containing intralobular lines.
Mixed connective tissue disease, Fig.1