Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders

Dear Colleagues,

Interstitial lung disease (ILD) is one of the most common and serious pulmonary complications of connective tissue diseases (CTD) and rheumatoid arthritis (RA), with variable clinical impact and severity, often resulting in significant morbidity and mortality. The prevalence reported for ILD is variable according to different classification modalities among case series and specific diagnoses, with a higher frequency in patients with systemic sclerosis and idiopathic inflammatory myopathies. The real incidence of RA-ILD is unknown, but prevalence rates of 7.7% for symptomatic RA-ILD, 67% for radiologic RA-ILD, and 80% for biopsy-identified RA-ILD have been reported. About 10% of RA patients develop a clinically significant ILD that is responsible for 10%–20% of mortality, with a mean survival of 5–8 years. Due to the lack of randomized controlled trials and recommendations, the therapeutic choice for CTD-ILD and RA-ILD is currently very challenging. To further complicate this scenario, both conventional and biologic disease-modifying anti-rheumatic drugs (DMARDs) have been implicated in the development of drug-related pulmonary toxicity, with conflicting data. Due to the absence of evidence for RA-ILD treatment efficacy and potential adverse effects, the decision to treat should be based on the balance between treatment benefits and the burden of the disease in each single patient.

Dr. Marco Sebastiani
Dr. Andreina Manfredi
Guest Editors

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