Cryptogenic organizing pneumonia (COP) is a clinicopathologic syndrome characterized by rapid resolution with corticosteroids, but frequent relapses when treatment is tapered or stopped. We retrospectively studied relapses in 48 cases of biopsy-proven COP. One or more relapses (mean 2.4 ± 2.2) occurred in 58%. At first relapse, 68% of patients were still under treatment for the initial episode. Compared with the no-relapse group, nine patients with multiple (≥ 3) relapses had longer delays between first symptoms and treatment onset (22 ± 17 versus 11 ± 8 wk, p = 0.02), and elevated γ-glutamyltransferase (124 ± 98 versus 29 ± 13 IU/L, p = 0.001) and alkaline phosphatase (190 ± 124 versus 110 ± 68 IU/L, p = 0.04) levels. Relapses did not adversely affect outcome. Corticosteroid treatment side effects occurred in 25% of patients. Standardized treatment in 14 patients allowed a reduction of prednisone cumulated doses (p < 0.05) without affecting outcome or relapse rate. We conclude that: (1) delayed treatment increases the risk of relapses; (2) mild cholestasis identifies a subgroup of patients with multiple relapses; (3) relapses do not affect outcome, and prolonged therapy to suppress relapses appears unnecessary; (4) a standardized treatment allows a reduction in steroid doses.
Lazor, R., Vandevenne, A., Pelletier, A., Lecerc, P., Court-Fortune, I., Cordier, J.-F., Bernard, J. P., Boyer, J., Braud, M. L., Brechot, J. M., Breton, J. L., Carre, P., Couval, F., Dalphin, J. C., Marchand, E., & et al. (2000). Cryptogenic organizing pneumonia: Characteristics of relapses in a series of 48 patients. American Journal of Respiratory and Critical Care Medicine, 162(2), 571-577. https://doi.org/10.1164/ajrccm.162.2.9909015 (Original work published 2000)