Collagen vascular disease-associated interstitial lung disease

Abstract

Interstitial lung disease (ILD) is an important manifestation of collagen vascular diseases. It is a common feature of scleroderma, and also occurs in dermatomyositis and polymyositis, mixed connective tissue disease, Sjogren’s syndrome, rheumatoid arthritis, systemic lupus erythematosus, and Antineutrophil cytoplasmic antibody-associated vasculitis. When present, it is associated with increased morbidity and mortality, thus making early diagnosis important. In fact, in many patients, ILD may be the first manifestation of a collagen vascular disease. The most common symptoms are cough and dyspnea. The diagnosis is made based on pulmonary function tests showing restrictive lung disease and impaired oxygen diffusion and chest imaging showing ground glass infiltrates, interstitial thickening, and/or fibrosis. The most common histologic finding on lung biopsy is non-specific interstitial pneumonia, though organizing pneumonia and usual interstitial pneumonia may also be seen. Treatment is focused on addressing the underlying collagen vascular disease with immunosuppression, either with corticosteroids or a steroid-sparing agent such as cyclophosphamide, azathioprine, or mycophenolate, although the optimal agent and duration of therapy is not known. There are few clinical trials to guide therapy that focus specifically on the progression of ILD. The exception is in the case of scleroderma-associated ILD, where cyclophosphamide has been shown to be effective.

Keywords: Interstitial lung disease; Collagen vascular disease; Connective tissue disease; Rheumatoid arthritis; Scleroderma; Myositis; Sjogren’s syndrome; Systemic lupus erythematosus; Antineutrophil cytoplasmic antibody-associated vasculitis; Mixed connective tissue disease

Core tip: Interstitial lung disease (ILD) is a significant manifestation of collagen vascular diseases due to its association with increased morbidity and mortality. Thus it is important for clinicians to consider and be able to recognize collagen vascular disease-associated ILD and initiate appropriate treatment. This review will discuss the clinical features, histologic findings, and treatment options of collagen vascular disease-associated ILD.