PNEUMOCYSTIS-CARINII PNEUMONIA - A MAJOR COMPLICATION OF IMMUNOSUPPRESSIVE THERAPY IN PATIENTS WITH WEGENERS GRANULOMATOSIS

The risk factors and clinical and laboratory parameters in Pneumocysti s carinii pneumonia in patients with Wegener's granulomatosis have not been well characterized. We undertook a retrospective chart review of all patients with a diagnosis of Wegener's granulomatosis and P. cari nii pneumonia who were followed at the National Institute of Allergy a nd Infectious Diseases of the National Institutes of Health. The chart review focused on clinical, laboratory, and roentgenologic evidence o f P. carinii pneumonia. Eleven cases of P. carinii pneumonia were diag nosed in some 180 patients with Wegener's granulomatosis, for an overa ll incidence of approximately 6%. All patients developed P. carinii pn eumonia either during the initial course of treatment or during therap y for recurrent Wegener's granulomatosis. All patients were receiving daily glucocorticoids and a second immunosuppressive therapy. Lymphocy topenia was noted in all patients, with a mean +/- SEM total lymphocyt e count of 303 +/- 69 cells/mu L. All patients tested (10 of 11) were seronegative for human immunodeficiency virus (HIV) infection. Eight p resented with worsening chest roentgenograms compared with baseline, w hereas three presented with normal chest roentgenograms. We conclude t hat P. carinii is a common opportunistic pathogen in patients with Weg ener's granulomatosis receiving immunosuppressive therapy. Therapeutic immunosuppression (daily glucocorticoids and immunosuppressive agents ) and the resultant lymphocytopenia, as well as the lymphocyte and mon ocyte functional abnormalities caused by glucocorticoids, may be the m ost likely factors predisposing to P. carinii pneumonia in patients wi th Wegener's granulomatosis. Based on our data, all patients with Wege ner's granulomatosis should be given chemoprophylaxis against P. carin ii while they are receiving daily glucocorticoids. Clinicians should e valuate these patients for P. carinii if there is even a minor clinica l or roentgenographic change in their status.