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Am. J. Respir. Crit. Care Med., Volume 162, Number 2, August 2000, 571-577

Cryptogenic Organizing Pneumonia
Characteristics of Relapses in a Series of 48 Patients

ROMAIN LAZOR, ANDRÉ VANDEVENNE, ANTOINE PELLETIER, PASCAL LECLERC, ISABELLE COURT-FORTUNE, JEAN-FRANÇOIS CORDIER, and the Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P)

Centre d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires, Hôpital Cardiovasculaire et Pneumologique Louis Pradel, Université Claude Bernard, Hospices Civils de Lyon, Lyon; Service de Pneumologie, Centre Hospitalier Universitaire, Hôpital de Hautepierre, Strasbourg; Service de Pneumologie, Clinique St-Laurent, Rennes; Service de Pneumologie, Centre Hospitalier Privé des Yvelines, Sartrouville; Service de Pneumologie, Centre Hospitalier Universitaire de Saint-Etienne, Hôpital Nord, Saint-Etienne; and Service de Pneumologie, Hôpital Cardiovasculaire et Pneumologique Louis Pradel, Hospices Civils de Lyon, Lyon, France

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 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. 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.




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