Cryptogenic organizing pneumonia - Characteristics of relapses in a seriesof 48 patients

Cryptogenic organizing pneumonia (COP) is a clinicopathologic syndrome char acterized by rapid resolution with corticosteroids, but frequent relapses w hen 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) occ urred 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 (greater than or equal to 3) relapses had longer delays betwe en first symptoms and treatment onset (22 +/- 17 versus 11 +/- 8 wk, p = 0. 02), and elevated gamma-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. Corticostero id treatment side effects occurred in 25% of patients. Standardized treatme nt in 14 patients allowed a reduction of prednisone cumulated doses (p < 0. 05) without affecting outcome or relapse rate. We conclude that: (1) delaye d 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.