Guidelines for ILD in Systemic Autoimmune Rheumatic Diseases

Recommendations for the Screening, Monitoring & Treatment of Interstitial Lung Disease Under Final Development

Dr. Sindhu Johnson

Image Credit: Gajus/shutterstock.com

SAN DIEGO—Many patients with interstitial lung disease (ILD) have concurrent systemic autoimmune rheumatic diseases (SARDs), such as systemic sclerosis, rheumatoid arthritis, Sjögren’s syndrome, mixed connective tissue disease or idiopathic inflammatory myopathies. During a Sunday, Nov.12, scientific session at ACR Convergence, Sindhu Johnson, MD, PhD, professor of medicine at the University of Toronto, Canada, highlighted some key considerations and clinical pearls related to ACR’s new guideline on SARDs and ILD.^1,2

Dinesh Khanna, MD, MSc, professor of medicine and director of the University of Michigan Scleroderma Program, Ann Arbor, illustrated some of the recommendations via case studies from his practice.

Example Case Presentation: Early Diffuse Systemic Sclerosis

Dr. Dinesh Khanna

Dr. Khanna described a 51-year-old woman with a new referral for early diffuse systemic sclerosis. Her first non-Raynauds phenomenon symptoms were joint swelling, followed by puffy fingers and other symptoms such as pruritus and gastroesophageal reflux disease; she now had significant disability.

The patient’s modified Rodnan skin score was 23/51, suggesting significant skin fibrosis. Her platelet count was elevated at 474,000 platelets/mcL (normal, less than 200,000) and her inflammatory markers were also elevated (e.g., a C-reactive protein of 2 mg/dL; normal, less than 0.6 mg/dL). She was also positive for antibodies against topoisomerase (anti-SCL-70 antibodies).

However, she did not report cough or dyspnea on exertion, and her chest was clear to auscultation.

Clinical Questions: Early ILD is often asymptomatic. Does this patient need screening for ILD? If so, which tests? What if she had a different SARD, clinical features or risk factors? 

In her talk, Dr. Johnson pointed out that although all the SARDs impart an increased risk of ILD compared with the general population, some SARDs carry a higher risk than others. Additionally, patients with the same disease may have a greater likelihood of developing an ILD based on known risk factors.

For example, systemic sclerosis imparts the highest risk of ILD among the SARDs, and Dr. Johnson said the vast majority of such patients should be screened. In contrast, not all patients with rheumatoid arthritis or other SARDs need screening. However, if one or more risk factors are present, the patient and physician should at least discuss the need for screening.