Cryptogenic organizing pneumonia. Characteristics of relapses in a series of 48 patients. The Groupe d'Etudes et de Recherche sur les Maladles "Orphelines" Pulmonaires (GERM"O"P)

Am J Respir Crit Care Med. 2000 Aug;162(2 Pt 1):571-7. doi: 10.1164/ajrccm.162.2.9909015.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Alkaline Phosphatase / blood
  • Cholestasis / complications
  • Cryptogenic Organizing Pneumonia / drug therapy
  • Cryptogenic Organizing Pneumonia / mortality
  • Cryptogenic Organizing Pneumonia / physiopathology*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Prednisone / adverse effects
  • Prednisone / therapeutic use
  • Probability
  • Recurrence
  • Retrospective Studies
  • Treatment Outcome
  • gamma-Glutamyltransferase / blood

Substances

  • gamma-Glutamyltransferase
  • Alkaline Phosphatase
  • Prednisone