What Rheumatologists Need to Know about Diagnosing and Managing Interstitial Lung Disease (ILD)

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Figure 4: Simple staging system for SSc-ILD.

Management Dilemmas

Although there are few prospective data to guide therapeutic decision making, it is clear that not all patients with CTD-ILD require treatment. We believe whether to treat a patient with CTD-ILD depends on the severity and pace of the ILD and which comorbid conditions or contraindications to treatment exist. Therapy for CTD-ILD is generally reserved for patients with clinically significant, progressive disease. When considering immunomodulatory therapy options for CTD-ILD, both intra- and extrathoracic disease manifestations need to be taken into consideration. Because multiple systems may be affected by CTD, coordinated care among rheumatologists and other subspecialists can be helpful. Although extrathoracic manifestations may determine the initial immunomodulatory regimen, quite often, it is the ILD that dictates the intensity (i.e., specific agent and duration) of the immunosuppressive therapy.

We are in desperate need of better therapies for CTD-ILD. Determining which drugs are effective for patients with CTD-ILD will require the completion of thoughtfully constructed and carefully conducted large-scale therapeutic trials. Until then, we have to rely on data from small case series or retrospective studies, scientific rationale, and inferences drawn from the few controlled clinical trials conducted for SSc-ILD.

ILD is a serious, potentially life-threatening manifestation of CTD. Patients with all forms of CTD are at risk for developing ILD, and ILD may be the first or only manifestation of a CTD. There are numerous challenges and opportunities related to the clinical care and research of CTD-ILD. We believe patients with CTD-ILD require a multidisciplinary approach to management, and the challenges in diagnosing, classifying, and treating CTD-ILD create opportunities ripe for carefully planned research.

Disclosures

Drs. Fischer and Swigris are investigators for the NIH-funded Scleroderma Lung Study II. Dr. Fischer is a speaker, consultant, and advisory board member for Actelion pharmaceuticals and a speaker and advisory board member for Gilead pharmaceuticals.

Dr. Fischer is associate professor of medicine, acting chief of the division of rheumatology, and in the autoimmune and interstitial lung disease program at National Jewish Health in Denver. Dr. Swigris is associate professor of medicine and in the autoimmune and interstitial lung disease program at National Jewish Health.

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