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Although the disease course is variable, CTD\u2010ILD 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\u2010ILD is diverse and pharmacological management, when indicated, is typically centred around corticosteroids and various immunosuppressive or anti\u2010fibrotic agents. Until recently, treatment decisions have been extrapolated from the evidence base available for SSc\u2010related ILD. Many recent trials and prospective studies have evaluated treatment options in a range of CTD\u2010ILD, thus guiding therapeutic intervention. Amidst an expanding evidence base, this comprehensive review describes new management strategies in CTD\u2010ILD with a focus on evidence from clinical trials. 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