Current and emerging treatment options for interstitial lung disease in patients with rheumatic disease

ABSTRACT

The management of connective tissue disease-associated interstitial lung disease (CTD-ILD) is complex and this arena offers many challenges to the practicing clinician. Unfortunately, treatment strategies and recommendations are often based on experience rather than evidence, and there are few effective therapeutic options. Pharmacologic intervention with immunosuppression is usually the mainstay of therapy and is reserved for those with clinically significant and/or progressive ILD. There is a desperate need for controlled trials across the spectrum of CTD-ILD and a number of potentially promising novel therapies warrant further study. It is important to address co-morbid conditions or aggravating factors (e.g., gastroesophageal reflux, aspiration, bone health, pulmonary hypertension, Pneumocystis jiroveci prophylaxis) and to institute non-pharmacologic management strategies (e.g., supplemental oxygen and cardiopulmonary rehabilitation) as part of a comprehensive treatment plan in CTD-ILD.

KEYWORDS:

• Connective tissue disease
• interstitial lung disease
• interstitial pneumonia
• pulmonary fibrosis

Financial and competing interests disclosure

A Fischer is a paid consultant for Actelion Pharmaceuticals, Bayer, Boehringer-Ingelheim, Bristol Meyer Squibb, Genentech, Gilead Sciences, Glaxo-Smith-Kline, Seattle Genetics. J Lee is a paid consultant for Parexel International. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.