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Pirfenidone + DMARDs is a combination therapy regimen being investigated by Qilu Hospital of Shandong University for the treatment of connective tissue disease-associated interstitial lung disease (CTD-ILD). This regimen combines the antifibrotic and anti-inflammatory properties of pirfenidone with the immunosuppressive effects of disease-modifying antirheumatic drugs (DMARDs). Pirfenidone is a small molecule that acts by inhibiting the synthesis of transforming growth factor-beta (TGF-β), a key mediator of fibrogenesis, and by reducing the production of pro-inflammatory cytokines such as TNF-α and IL-6. DMARDs, which in this clinical context include various immunosuppressive agents like mycophenolate mofetil or azathioprine, target the underlying autoimmune processes of the primary connective tissue disease. The combination is currently being evaluated in a Phase 4 trial to determine its efficacy in preserving lung function (measured by FVC) and improving clinical outcomes in patients with ILD associated with systemic sclerosis, rheumatoid arthritis, and inflammatory myopathies.
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