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December 11, 1995

Pneumocystis carinii Pneumonia in Patients Without AIDS, 1980 Through 1993: An Analysis of 78 Cases

Author Affiliations

From the Department of Infectious Diseases, University Hospital, Leiden, the Netherlands.

Arch Intern Med. 1995;155(22):2436-2441. doi:10.1001/archinte.1995.00430220094010

Background:  Pneumocystis carinii pneumonia (PCP) occurs in immunocompromised patients without the acquired immunodeficiency syndrome (AIDS). There has been an increasing yearly number of cases of PCP in our patients without AIDS.

Objective:  To determine the nature of the underlying disorder and previous immunosuppressive treatment in patients with PCP without AIDS.

Method:  A study of the charts of 78 such patients admitted to our hospital from 1980 through 1993.

Results:  The number of PCP cases per year increased during the period studied. All patients had an underlying disorder, either hematologic malignancy (49%), solid organ tumor (4%), vasculitis or other immunologic disorder (22%), or they had undergone renal transplantation (17%) or bone marrow transplantation (9%). Previous immunosuppressive medication consisted of prednisone or other corticosteroids in 72 (92%) of 78 patients, cytotoxic drugs in 55 (71%) of 78 patients, both in 50 (64%) of 78 patients, and none in one patient. Quantification of previous corticosteroid treatment showed a large variability among patients. The overall mortality rate for patients was 35% (27/78). Mortality was significantly higher in patients with a concomitant pulmonary infection (P=.01), an underlying disorder other than that which resulted in renal transplantation (P=.03), mechanical ventilation (P<.001), previous chemotherapy (P=.04), as well as previous cyclophosphamide treatment (P=.01). A trend toward a higher mortality in patients with previous corticosteroid use was detected (P=.06).

Conclusion:  Pneumocystis carinii pneumonia may complicate a variety of immunocompromised states, with considerable mortality. Pneumocystis carinii pneumonia occurred at all levels of immunosuppression; no threshold level could be defined.(Arch Intern Med. 1995;155:2436-2441)

Bartlett MS, Smith JW.  Pneumocystis carinii: an opportunist in immunocompromised patients.  Clin Microbiol Rev . 1991:4:137-149.
Nouza M.  Pneumocystis cariniipneumonia after 40 years.  Infection . 1992:20: 113-117Crossref
Sepkowitz KA.  Pneumocystis carinii pneumonia in patients without AIDS.  Clin Infect Dis . 1993;17( (suppl 2) ):S416-S422.Crossref
Gajdusek DC.  Pneumocystis carinii: etiologic agent of interstitial plasma cell pneumonia of premature and young infants.  Pediatrics . 1957:19:543-565.
Walzer PD, Perl DP, Krogstad DJ, Rawson PG, Schultz MG.  Pneumocystis carinii pneumonia in the United States: epidemiologic, diagnostic, and clinical features.  Ann Intern Med . 1974;80:83-93.Crossref
Hughes WT.  Pneumocystis pneumonia: a plague of the immunosuppressed.  Johns Hopkins Med J ., 1978;143:184-192.
Ruebush TK II, Weinstein RA, Baehner RL, et al.  An outbreak of Pneumocystis pneumonia in children with acute lymphocytic leukemia.  AJDC . 1978;132:143-148.
Rosen P, Armstrong D, Ramos C.  Pneumocystis carinii pneumonia: a clinicopathological study of 20 patients with neoplastic diseases.  Am J Med . 1972; 53:428-436.Crossref
Sepkowitz KA, Brown AE, Telzak EE, Gottlieb S, Armstrong D.  Pneumocystis carinii pneumonia among patients without AIDS at a cancer hospital.  JAMA . 1992;267:832-837.Crossref
Varthalitis I, Aoun M, Daneau D, Meunier F.  Pneumocystis carinii pneumonia in patients with cancer.  Cancer . 1993;71:481-485.Crossref
Fossieck BE, Spagnolo SV.  Pneumocystis carinii pneumonitis in patients with lung cancer.  Chest . 1980;78:721-722.Crossref
Brunvand MW, Collins C, Livingston RB, Raghu G.  Pneumocystis carinii pneumonia associated with profound lymphopenia and abnormal T-lymphocyte subset ratios during treatment for early-stage breast carcinoma.  Cancer . 1991;67: 2407-2409.Crossref
Rifkind D, Starzl TE, Marchioro TL, Waddell WR, Rowlands DT, Hill RB.  Transplantation pneumonia.  JAMA . 1964;189:808-812.
Le Clair RA.  Transplantation pneumonia, associated with Pneumocystis carinii, among recipients of cardiac transplants.  Am Rev Respir Dis . 1969:100: 874-875.
Linder J.  Infection as a complication of heart transplantation.  J Heart Transplant . 1988:7:390-394.
Grossi P, Ippoliti GB, Goggi C, Cremaschi P, Scaglia M, Minoli L.  Pneumocystis carinii pneumonia in heart transplant recipients.  Infection . 1993;21:75-79.Crossref
Wallis PJW, Ryatt KS, Constable TJ.  Pneumocystis carinii pneumonia complicating low dose methotrexate treatment for psoriatic arthropathy.  Ann Rheum Dis . 1989;48:247-249.Crossref
Smith MB, Hanauer SB.  Pneumocystis carinii pneumonia during cyclosporine therapy for ulcerative colitis.  N Engl J Med . 1992;327:497-498.Crossref
Porter DR, Marshall DAS, Madhok R, Capell H, Sturrock RD.  Pneumocystis carinii infection complicating cytotoxic therapy in two patients with lymphopenia, but a normal total white cell count.  Br J Rheumatol . 1992;31:71-72.Crossref
Godeau B, Coutant-Perronne V, Huong DLT, et al.  Pneumocystis carinii pneumonia in the course of connective tissue disease: report of 34 cases.  J Rheumatol . 1994;21:246-251.
Hughes WT, Kuhn S, Chaudhary S, et al.  Successful chemoprophylaxis for Pneumocystis carinii pneumonitis.  N Engl J Med . 1977;297:1419-1426.Crossref
Harris RE, McCallister JA, Allen SA, Barton AS, Baehner RL.  Prevention of Pneumocystis pneumonia: use of continuous sulfamethoxazole-trimethoprim therapy.  AJDC . 1980;134:35-38.
Hughes WT, Rivera GK, Schell MJ, Thornton D, Lott L.  Successful intermittent chemoprophylaxis for Pneumocystis carinii pneumonitis.  N Engl J Med . 1987; 316:1627-1632.Crossref
Elinder CG, Andersson J, Bolinder G, Tydén G.  Effectiveness of low-dose cotrimoxazole prophylaxis against Pneumocystis carinii pneumonia after renal and/or pancreas transplantation.  Transpl Int . 1992;5:81-84.
Kovacs JA, Hiemenz JW, Macher AM, et al.  Pneumocystis carinii pneumonia: a comparison between patients with the acquired immunodeficiency syndrome and patients with other immunodeficiencies.  Ann Intern Med . 1984; 100:663-671.Crossref
Limper AH, Offord KP, Smith TF, Martin WJ II.  Pneumocystis carinii pneumonia: differences in lung parasite number and inflammation in patients with and without AIDS.  Am Rev Respir Dis . 1989;140:1204-1209.Crossref
Wehle K, Schirmer M, Dunnebacke-Hinz J, Kupper T, Pfitzer P.  Quantitative differences in phagocytosis and degradation of Pneumocystis carinii by alveolar macrophages in AIDS and non-HIV patients in vivo.  Cytopathology . 1993; 4:231-236.Crossref
Sattler FR, Remington JS.  Intravenous trimethoprim-sulfamethoxazole therapy for Pneumocystis carinii pneumonia.  Am J Med . 1981;70:1215-1221.Crossref
Haron E, Bodey GP, Luna MA, Dekmezian R, Elting L.  Has the incidence of Pneumocystis carinii pneumonia in cancer patients increased with the AIDS epidemic?  Lancet . 1988;2:904-905.Crossref
Chave JP, David S, Wauters JP, Van Melle G, Francioli P.  Transmission of Pneumocystis carinii from AIDS patients to other immunosuppressed patients: a cluster of Pneumocystis carinii pneumonia in renal transplant recipients.  AIDS . 1991;5:927-932.Crossref
Flament M, Couderc LJ, Aubert P, Epardeau B, Guédon J, Caubarrère I.  Pneumocystose pulmonaire et transplantation rénale: influence de l'épidémie de SIDA.  Ann Med Interne (Paris) . 1993:144:61.
Stover DE, Zaman MB, Hajdu SI, Lange M, Gold J, Armstrong D.  Bronchoalveolar lavage in the diagnosis of diffuse pulmonary infiltrates in the immunosuppressed host.  Ann Intern Med . 1984;101:1-7.Crossref
Peters SG, Prakash UBS.  Pneumocystis carinii pneumonia: review of 53 cases.  Am J Med . 1987;82:73-78.Crossref
Anthony LB, Greco FA.  Pneumocystis carinii pneumonia: a complication of Cushing's syndrome.  Ann Intern Med . 1981;94:488-489.Crossref
Natale RB, Yagoda A, Brown A, Singer C, Stover D, Bajorunas D.  Combined Pneumocystis carinii and Nocardia asteroides pneumonitis in a patient with an ACTH-producing carcinoid.  Cancer . 1981;47:2933-2935.Crossref
Fulkerson WJ, Newman JH.  Endogenous Cushing's syndrome complicated by Pneumocystis carinii pneumonia.  Am Rev Respir Dis . 1984;129:188-189.
Singer C, Armstrong D, Rosen PP, Schottenfeld D.  Pneumocystis carinii pneumonia: a cluster of 11 cases.  Ann Intern Med . 1975;82:772-777.Crossref
Frenkel JK, Good JT, Shultz JA.  Latent Pneumocystis infection of rats, relapse, and chemotherapy.  Lab Invest . 1966;15:1559-1577.
DeVita VT, Goodell B, Hubbard S, Geelhoed GW, Young RC.  Pneumocystis pneumonia in patients with cancer: clinical setting.  Natl Cancer Inst Monogr . 1976;43:41-46.
Henson JW, Jalaj JK, Walker RW, Stover DE, Fels AOS.  Pneumocystis carinii pneumonia in patients with primary brain tumors.  Arch Neurol . 1991;48:406-409.Crossref
Slivka A, Wen PY, Shea WM, Loeffler JS.  Pneumocystis carinii pneumonia during steroid taper in patients with primary brain tumors.  Am J Med . 1993:94: 216-219.Crossref
Zaman MK, White DA.  Serum lactate dehydrogenase levels and Pneumocystis carinii pneumonia: diagnostic and prognostic significance.  Am Rev Respir Dis . 1988;137:796-800.Crossref
Lipschik GY, Gill VJ, Lundgren JD, et al.  Improved diagnosis of Pneumocystis carinii infection by polymerase chain reaction on induced sputum and blood.  Lancet . 1992:340:203-206.Crossref
Goldenberg DM, Sharkey RM, Udem S.  Immunoscintigraphy of Pneumocystis carinii pneumonia in AIDS patients.  J Nucl Med . 1994;35:1028-1034.
Walzer PD.  Pneumocystis carinii: recent advances in basic biology and their clinical application.  AIDS . 1993;7:1293-1305.Crossref
Pesanti EL.  Effects of bacterial pneumonitis on development of pneumocystosis in rats.  Am Rev Respir Dis . 1982;125:723-726.
The National Institutes of Health—University of California Expert Panel for Corticosteroids as Adjunctive Therapy for Pneumocystis Pneumonia.  Consensus statement on the use of corticosteroids as adjunctive therapy for Pneumocystis pneumonia in the acquired immunodeficiency syndrome.  N Engl J Med . 1990;323:1500-1504.Crossref
Suffredini AF, Tobin MJ, Wajszczuk CP, et al.  Acute respiratory failure due to Pneumocystis carinii pneumonia: clinical, radiographic, and pathologic course.  Crit Care Med . 1985;13:237-243.Crossref
Moore EH, Webb WR, Amend WJC.  Pulmonary infections in renal transplantation patients treated with cyclosporine.  Radiology . 1988;167:97-103.Crossref
Phair J, Muñoz A, Detels R, et al.  The risk of Pneumocystis carinii pneumonia among men infected with human immunodeficiency virus type 1.  N Engl J Med . 1990:322:161-165.Crossref
Browne MJ, Hubbard SM, Longo DL, et al.  Excess prevalence of Pneumocystis carinii pneumonia in patients treated for lymphoma with combination chemotherapy.  Ann Intern Med . 1986:104:338-344.Crossref
Waßerman K, Pothoff G, Heitz W, et al.  Akute Pneumozystosen unter Polychemotherapie nach dem MACOP-B-Protokoll.  Dtsch Med Wochenschr . 1990; 115:1705-1711.Crossref
Kautz-Freimuth S.  Pneumocystis-carinii Pneumonie: Einsatzmöglichkeiten von Pentamidin-Aerosol in Hämatologie, Onkologie und Transplantationsmedizin.  Internist (Berl) . 1991;32:235-236.
Hughes WT.  Prevention and treatment of Pneumocystis carinii  Annu Rev Med . 1991;42:287-295.Crossref
Opravil M, Hirschel B, Lazzarin A, et al.  Once weekly administration of dapsone/ pyrimethamine vs aerosolized pentamidine as combined prophylaxis for Pneumocystis carinii pneumonia and toxoplasmic encephalitis in human immunodeficiency virus—infected patients.  Clin Infect Dis . 1995:20:531-541.Crossref