Symposium
Pneumocystis carinii Pneumonia in Patients without HIV Infection

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ABSTRACT

Pneumocystis carinii is an important, but sporadic, opportunistic pulmonary pathogen in immu- nosuppressed HIV seronegative persons. Historically, patients at highest risk for P carinii pneumonia are included infants with severe malnutrition, children with primary immunodeficiencies, patients with hematological malignancies, and recipients of solid organ or bone marrow transplants. Recently, solid tumor patients, in particular those receiving high-dose corticosteroids for brain neoplasms, and patients with inflammatory or collagen-vascular disorders, especially patients with Wegener granulomatosis receiving immunosuppressive therapy, have been identified as subgroups at increased risk for P carinii pneumonia. Other factors associated with P carinii pneumonia include the intensity of the immunosuppressive regimen and tapering doses of corticosteroids. Because P carinii pneumonia is associated with significant morbidity and mortality, it is important to identify high-risk patient populations to administer effective chemoprophylactic agents, such as trimethoprimsulfamethoxazole

Section snippets

Historical Perspective of P carinii Pneumonia

Pneumocystis carinii was initially described by Carlos Chagas in 1909, who noted cystic organisms in the lungs of guinea pigs infected with Trypanosoma cruzi, but incorrectly concluded that these forms represented a sexual stage of the trypanosome life cycle.4., 5., 6. Although Antonio Carini similarly identified cysts within rat lungs infected with Trypanosoma lewisi in 1910, he, too, incorrectly surmised that they represented variants in the trypanosome life cycle. The organism was

Overview of P carinii Pneumonia in Patients without AIDS

Two recent studies have reviewed the clinical experience of individual medical centers with P carinii pneumonia in patients without AIDS. In a retrospective report from the Department of Infectious Disease, University Hospital, Leiden, the Netherlands, 58 cases of definite and 20 cases of highly probable P carinii pneumonia were identified during a 13-year period (1980–1993).11 During this study, the number of patients per year with P carinii pneumonia increased. All patients had an underlying

Comparison of P carinii Pneumonia in HIV Seronegative and HIV Seropositive Persons

Several studies have compared the clinical manifestations of P carinii pneumonia in HIV seronegative and seropositive persons. The typical presentation of P carinii pneumonia in HIV seropositive persons is characterized by nonspecific symptoms that are initially mild in severity and insidious in onset, sometimes being present for weeks before diagnosis.14 In contrast, HIV seronegative persons often present in a more acute fashion. For example, a retrospective study of 39 patients without AIDS

P carinii Pneumonia in Infants and Children without AIDS

As outlined in the historical perspective, epidemic cases of P carinii pneumonia were first described in severely malnourished infants and children during World War II.4., 8. Consequently, risk factors for development of P carinii pneumonia in infants were thought to include immunosuppression on the basis of either severe malnutrition, marasmus, or prematurity, especially if the person was residing in an institutional setting in which the epidemic form of disease was known to exist.7

Children,

P carinii Pneumonia Complicating Cancer

Cancer patients are at increased risk to develop P carinii pneumonia, either as a consequence of immunosuppression related to their malignancy or to the administration of immunosuppressive agents. For example, in the series from the Memorial Sloan-Kettering Cancer Center, 87% of cancer patients with P carinii pneumonia either were receiving corticosteroid therapy or had excessive endogenous production.3 Furthermore, 70% of episodes of P carinii pneumonia occurred in the setting of a steroid

Bone Marrow Transplant Recipients

As a consequence of the severe immunosuppression associated with bone marrow transplantation, which often extends well into the post-transplant period, this patient population is known to be at increased risk for infectious complications caused by a variety of pathogens, including P carinii. The diagnosis of P carinii pneumonia in these patients can be challenging because toxicity related to both the conditioning regimens and graft versus host disease can produce clinical manifestations similar

P carinii Pneumonia Complicating Immunosuppressive Therapy for Inflammatory Disease

Patients receiving immunosuppressive therapy with corticosteroids and cytotoxic agents for inflammatory or collagen-vascular disorders, in particular Wegener granulomatosis, are at increased risk for the development of P carinii pneumonia. Furthermore, significant immunosuppression as evidenced by lymphocytopenia, may also represent a risk factor. For example, a recent retrospective study from the Hôpital Henri Mondor, Créteil, France, has reported on 34 cases of P carinii pneumonia

Conclusion

P carinii pneumonia is an uncommon opportunistic pulmonary pathogen in HIV-seronegative persons who are immunosuppressed on the basis of malignancy, immunosuppressive therapy, or a primary immunodeficiency. Historically, patients at highest risk for P carinii pneumonia have included infants with severe malnutrition, children with primary immunodeficiencies, patients with hematological malignancies (ie, acute lymphoblastic leukemia), and recipients of solid organ or bone marrow transplants.

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