Biological treatments and connective tissue disease associated interstitial lung disease

There is no specific therapy for interstitial lung disease associated with connective tissue diseases (CTDs-ILD), a potentially fatal condition for some of these patients. This article reviews currently available information on the effects on CTDs-ILD of biological treatments that are increasingly used with considerable success in various systemic diseases.

A beneficial effect of antitumor necrosis factor (TNF) agents on CTDs-ILD has been described in sporadic patients with rheumatoid arthritis (RA), systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). However, and despite the fact that there was no clear evidence of pulmonary toxicity of these agents in randomized-controlled trials comprising thousands of patients with RA and spondylarthropathies, new onset or exacerbation of preexisting ILD with high mortality rates has so far been observed in 144 RA patients following anti-TNF treatment in clinical practice. Likewise, administration of the B-cell depleting anti-CD20 antibody rituximab was beneficial for ILD in SSc patients but associated with new-onset ILD in isolated patients with RA and SLE. Pertinent information on other biological treatments is currently lacking.

Data on the therapeutic role of biological agents in CTDs-ILD is preliminary and controversial. Although preexisting ILD is not a contraindication for these agents, until more information is available their administration should be stopped when new pulmonary symptoms occur.