The efficacy and safety of systemic corticosteroids as first line treatment for granulomatous lymphocytic interstitial lung disease

BACKGROUND: Granulomatous and lymphocytic interstitial lung disease (gl-ILD) is a major cause of morbidity and mortality among patients with common variable immunodeficiency. Corticosteroids are recommended as first-line treatment for gl-ILD, but evidence for their efficacy is lacking. OBJECTIVE: We analyzed the effect of high-dose corticosteroids (≥0.3mg/kg prednisone equivalent) on gl-ILD, measured by high-resolution computed tomography (HRCT) scans, pulmonary function test (PFT) results. METHODS: Patients who had received high-dose corticosteroids but no other immunosuppressive therapy at the time (n=56) and who underwent repeated HRCT scanning or PFT during the retrospective and/or prospective phase of the STILPAD study (n=39) were included in the analysis. Patients without any immunosuppressive treatment were selected as controls (n=23). HRCT scans were blinded, randomized and scored using the Hartman score. Differences between the baseline and follow up HRCT scans and PFT were analyzed. RESULTS: Treatment with high-dose corticosteroids significantly improved HRCT scores and forced vital capacity. Carbon monoxide diffusion capacity significantly improved in both groups. 13/18 patients, for whom extended follow-up data was available, achieved a long-term, maintenance therapy independent remission. All patients with relapse were re-treated with corticosteroids, but only 1/5 responded. Two opportunistic infections were found in the corticosteroid treatment group, while overall infection rate was similar between cohorts. CONCLUSION: Induction therapy with high-dose corticosteroids improved HRCT scans and PFT results of patients with gl-ILD and achieved long-term remission in 42% of patients. It was not associated with major side-effects. Low dose maintenance therapy provided no benefit and efficacy was poor in relapsing disease.

• Smits, B.
• Goldacker, S.
• Seneviratne, S.
• Malphettes, M.
• Longhurst, H.
• Mohamed, O. E.
• Witt-Rautenberg, C.
• Leeman, L.
• Schwaneck, E.
• Raymond, I.
• Meghit, K.
• Uhlmann, A.
• Winterhalter, C.
• van Montfrans, J.
• Klima, M.
• Workman, S.
• Fieschi, C.
• Lorenza, L.
• Boyle, S.
• Onyango-Odera, S.
• Price, S.
• Schmalzing, M.
• Aurillac, V.
• Prasse, A.
• Hartmann, I.
• Meerburg, J. J.
• Kemner-van de Corput, M.
• Tiddens, H.
• Grimbacher, B.
• Kelleher, P.
• Patel, S. Y.
• Korganow, A. S.
• Viallard, J. F.
• Tony, H. P.
• Bethune, C.
• Schulze-Koops, H.
• Witte, T.
• Huissoon, A.
• Baxendale, H.
• Grigoriadou, S.
• Oksenhendler, E.
• Burns, S. O.
• Warnatz, K.