Clinical Spotlight: Collaborative Treatment for ILD Patients with Connective Tissue Disease

By Erin R. Narewski, DO

Erin Narewski, DO

Connective tissue diseases (scleroderma, rheumatoid arthritis, Sjogren’s syndrome, systemic lupus erythematosus, polymyositis, dermatomyositis and mixed connective tissue disease) in about 11% of patients can be associated with interstitial lung disease (i.e., CT-ILD). Patients may first present with rheumatologic or lung complaints; however, patients usually initially present with manifestations of the connective tissue disease. The CT-ILD may cause inflammation of the lungs or fibrosis, or in some cases, both. Additionally, patients require assessment and treatment of their underlying rheumatologic disorder. Recently, antifibrotic agents have been reported to help attenuate the rate of lung function decline in patients with rheumatologic disease (scleroderma)1 and in other forms of non-UIP fibrotic lung disease.2

The Temple Lung Center, in collaboration with the Temple Department of Rheumatology, has formed a joint clinic specifically for the care of ILD patients with possible connective tissue disease. This clinical partnership allows us to bring collaborative data to multidisciplinary committee discussions and to improve diagnostic accuracy and evidence-based treatment for patients, a particularly important feature as steroid-sparing therapies become more available for use in this population. In addition, this partnership allows for close collaboration on research to fill the medical evidence gap that has long hampered treatment efforts for CT-ILD patients.

All ILD patients should receive careful screening and examination for serologic, symptomatic and physical examination findings suggestive of CTD. If screening suggests CTD as a potential cause of ILD, please consider early referral of your patient to a CT-ILD center of excellence where these patients may receive a thorough evaluation and evidence-based care.

To make a referral to Temple, please call 800-Temple-MED.

1 Distler, O., et al. 2019. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. N Engl J Med. 380:2518–2528. DOI: 10.1056/NEJMoa1903076
2 Flaherty, K., et al. 2019. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med., September 29 (online ahead of print). DOI: 10.1056/NEJMoa1908681