Laurène Mansy, Agnès Caille, Martine Reynaud-Gaubert, Julien Bermudez, Philippe Bonniaud, Raphael Borie, Pierre-Yves Brillet, Jacques Cadranel, Isabelle Court-Fortune, Bruno Crestani, Marie-Pierre Debray, Mathilde Duprez, Anne Guillaumot, Sandrine Hirschi-Santelmo, Dominique Israel-Biet, Stéphane Jouneau, Karine Juvin, Mallorie Kerjouan, Julie Mankikian, Charles-Hugo Marquette, Jean-Marc Naccache, Hilario Nunes, Laurent Plantier, Grégoire Prevot, Sébastien Quetant, Julie Traclet, Victor Valentin, Yurdagul Uzunhan, Lidwine Wémeau-Stervinou, Vincent Cottin, Sylvain Marchand-Adam on behalf of the EVER-ILD investigators and the OrphaLung network
European Respiratory Journal 2024 64: 2401368; DOI: 10.1183/13993003.01368-2024 

Extract

The nonspecific interstitial pneumonia (NSIP) pattern is a usual feature of interstitial lung disease (ILD) associated with connective tissue diseases (CTDs) [1], idiopathic interstitial pneumonia with autoimmune features (IPAF) [2] and several idiopathic ILDs (iILDs) [3]. The EVER-ILD trial randomised 126 patients between rituximab (1000 mg, on day 1 and day 15; n=65) versus placebo (n=61), in addition to mycophenolate mofetil (MMF) (2 g daily) for 6 months, in patients with NSIP pattern associated with CTD-ILD or idiopathic interstitial pneumonia (with or without autoimmune features) [4]. A primary analysis after 6 months of treatment showed a significant benefit of the rituximab+MMF combination on forced vital capacity (FVC) % predicted variation from baseline to 6 months (group*month interaction 3.60, 95% CI 0.41–6.80; p=0.03) and on progression-free survival (PFS) (crude HR 0.47, 95% CI 0.23–0.96; p=0.03).