The efficacy and safety of systemic corticosteroids as first line treatment for granulomatous lymphocytic interstitial lung disease.
Smits B., Goldacker S., Seneviratne S., Malphettes M., Longhurst H., Mohamed OE., Witt-Rautenberg C., Leeman L., Schwaneck E., Raymond I., Meghit K., Uhlmann A., Winterhalter C., van Montfrans J., Klima M., Workman S., Fieschi C., Lorenzo L., Boyle S., Onyango-Odera S., Price S., Schmalzing M., Aurillac V., Prasse A., Hartmann I., Meerburg JJ., Kemner-van de Corput M., Tiddens H., Grimbacher B., Kelleher P., Patel SY., 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 SO., Warnatz K.
BackgroundGranulomatous 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.ObjectivesThis study analyzed the effect of high-dose corticosteroids (≥0.3 mg/kg prednisone equivalent) on gl-ILD, measured by high-resolution computed tomography (HRCT) scans, and pulmonary function test (PFT) results.MethodsPatients who had received high-dose corticosteroids but no other immunosuppressive therapy at the time (n = 56) and who underwent repeated HRCT scanning or PFT (n = 39) during the retrospective and/or prospective phase of the Study of Interstitial Lung Disease in Primary Antibody Deficiency (STILPAD) 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.ResultsTreatment with high-dose corticosteroids significantly improved HRCT scores and forced vital capacity. Carbon monoxide diffusion capacity significantly improved in both groups. Of 18 patients, for whom extended follow-up data was available, 13 achieved a long-term, maintenance therapy independent remission. All patients with relapse were retreated with corticosteroids, but only one-fifth of them responded. Two opportunistic infections were found in the corticosteroid treatment group, while overall infection rate was similar between cohorts.ConclusionsInduction 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.