Interstitial Lung Disease in Systemic Lupus Erythematosus, Sjogren’s Syndrome, and Mixed Connective Tissue Disease
Mary E. Strek.
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.
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