Over 50% of patients with systemic sclerosis (SSc) develop interstitial lung disease (ILD), and rheumatologists have long recognized this major cause of morbidity and mortality.1 Sindhu R. Johnson, MD, PhD, professor of medicine at the University of Toronto, Canada, and director of the Toronto Scleroderma Program, notes that more recent studies have increased our awareness of the risks for ILD in other systemic autoimmune rheumatic diseases (SARDs), such as rheumatoid arthritis and Sjögren’s disease. Although comparatively lower than in SSc, the incidence of ILD in these diseases is still significant.
Clinicians now have guidelines to refer to regarding the screening, monitoring and treatment of patients with ILD secondary to SARDs. The guidelines will help clinicians quickly diagnose and optimally manage these diseases.1,2
Dr. Johnson
In January, The Rheumatologist, the ACR and Wiley presented a webinar featuring the following speakers: Dr. Johnson, principal investigator for the guidelines, and Elana J. Bernstein, MD, MSc, Florence Irving associate professor of medicine in the Division of Rheumatology at Columbia University, New York City, and co-first author of the guidelines.
Background
When it occurs in the context of a SARD, progression of ILD varies widely. It can present with subclinical features, such as mild cough, following a slow, progressive course, or with more acute manifestations and rapid disease progression with severe deterioration of pulmonary function. Alveolitis from activated T cells can result in the release of cytokines and the accumulation of alveolar macrophages, T lymphocytes and neutrophils in the lungs. Ongoing inflammation can lead to tissue injury and, sometimes, eventually fibrosis, which may be progressive.3
Immunosuppressants are the cornerstone of therapy for SARD-ILD, according to Dr. Bernstein, because they address ongoing inflammation. In the webinar, she discussed how the guidelines were developed. Like other recent ACR guidelines, these rely on the GRADE methodology (Grading of Recommendations, Assessment, Development and Evaluation) to rate the certainty of evidence on the basis of a literature review targeted to specific, clinically relevant PICO (i.e., population, intervention, comparator and outcome) questions. A panel that included both clinicians and patients voted on the final recommendations.
These recommendations are meant to guide shared decision making between clinicians and patients, and should not be used by third parties to constrain diagnostic or treatment options, says Dr. Bernstein.
The guidelines and recommendations apply specifically to adult patients with systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, rheumatoid arthritis or Sjögren’s disease.
