Abstract

Interstitial lung disease is a serious complication of connective tissue disorders causing significant morbidity and mortality. While interstitial lung disease is a less commonly noted respiratory manifestation in systemic lupus erythematosus where other pulmonary processes predominate, it is increasingly recognized in patients with Sjogren’s syndrome and can be severe and progressive in patients with mixed connective tissue disease as well. Interstitial lung disease may be the initial or predominant presentation of these connective tissue diseases, thus a systematic evaluation for autoimmune symptoms and serologies is required for accurate diagnosis.

The objective of this review is to present our current knowledge of interstitial lung disease in systemic lupus erythematosus, Sjogren’s syndrome and mixed connective tissue disease. We discuss the clinical presentation and highresolution CT and histopathologic patterns typical of interstitial lung disease in each of these three connective tissue diseases. We review management recommendations, and when available, present the evidence regarding specific medications used for treatment. We begin with general comments about the diagnostic evaluation of patients with connective tissue disease associated interstitial lung disease and conclude with a brief discussion of our clinical practice for follow-up of these patients. While our knowledge of the manifestations of interstitial lung disease in systemic lupus erythematosus, Sjogren’s syndrome and mixed connective tissue disease has increased, data regarding management and evidence for benefit of therapy remains limited.

Keywords: Connective tissue disease, interstitial lung disease, management, mixed connective tissue disease, pulmonary fibrosis, pulmonary manifestations, Sjogren’s syndrome, systemic lupus erythematosus, therapy.