Pulmonary Manifestations in the Rheumatic Diseases

Lung disease in the rheumatic diseases presents unique challenges for diagnosis and management and is a source of significant morbidity and mortality for patients. Unlike the idiopathic interstitial pneumonias, patients with rheumatic diseases experience lung disease in the context of a systemic disease that may make it more difficult to recognize and that may present greater risks with treatment. Despite recent advances in our awareness of these diseases, there is still a significant lack of understanding of natural history to elucidate which patients will develop disease that is progressive and thus warrants treatment. What we do know is that a subset of patient with rheumatic disease develop parenchymal lung disease that can prognostically resemble idiopathic pulmonary fibrosis, such as in rheumatoid arthritis, and that others can have aggressive inflammatory lung disease in the context of idiopathic inflammatory myopathies, systemic sclerosis, or an undifferentiated autoimmune process. As we enter an era of therapy directed against fibrosis in addition to novel anti- inflammatory therapies, and as we improve our ability to identify those at the highest risk for disease progression, we may be entering a paradigm shift in our ability to treat these complex patients and potentially offer better outcomes.

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