Connective tissue disease-associated interstitial lung disease: an approach to treatment amidst an expanding evidence base.

Connective tissue disease-associated interstitial lung disease (CTD-ILD) comprises a heterogenous group of conditions characterised by immune-mediated fibro-inflammatory pulmonary injury. Although the disease course is variable, CTD-ILD can progress to respiratory failure and thus has a profound impact on morbidity and mortality. Systemic sclerosis (SSc), rheumatoid arthritis, idiopathic inflammatory myositis, Sjogren disease systemic lupus erythematosus and mixed connective tissue disease can all manifest or present with ILD. Histological injury in CTD-ILD is diverse and pharmacological management, when indicated, is typically centred around corticosteroids and various immunosuppressive or anti-fibrotic agents. Until recently, treatment decisions have been extrapolated from the evidence base available for SSc-related ILD. Many recent trials and prospective studies have evaluated treatment options in a range of CTD-ILD, thus guiding therapeutic intervention. Amidst an expanding evidence base, this comprehensive review describes new management strategies in CTD-ILD with a focus on evidence from clinical trials. Supportive care and minimising treatment-associated adverse effects remains paramount in this population with complex respiratory pathology and frequent co-morbidities.