DEFINITION AND DESCRIPTION
Pneumocystis is an opportunistic pathogen that is an important cause of pneumonia in immunocompromised hosts, particularly those with HIV infection (Chap. 97), and in individuals with organ transplants, those with hematologic malignancies, and those receiving immunosuppressive therapy. The organism was discovered in rodents in 1906 and was initially believed to be a protozoan. Because Pneumocystis cannot be cultured, our understanding of its biology has been limited, but molecular techniques have demonstrated that the organism is actually a fungus. Formerly known as Pneumocystis carinii, the species infecting humans has been renamed Pneumocystis jirovecii.
P. jirovecii pneumonia (PCP) came to medical attention when cases were reported in malnourished orphans in Europe during World War II. The disease was later recognized in other immunosuppressed populations but was rare in the era before HIV/AIDS and before intensive immunosuppressive therapy for organ transplantation and autoimmune disorders. In 1981, PCP was first reported in men who had sex with men and in IV drug users who had no obvious cause of immunosuppression. These cases were subsequently recognized as the first cases of what came to be known as the acquired immunodeficiency syndrome (AIDS) (Chap. 97). The incidence of PCP increased dramatically as the AIDS epidemic grew: without chemoprophylaxis or antiretroviral therapy (ART), 80–90% of patients with HIV/AIDS in North America and Western Europe ultimately develop one or more episodes of PCP. While its incidence declined with the introduction of anti-Pneumocystis prophylaxis and combination ART, PCP has continued to be a leading cause of AIDS-associated morbidity in the United States and Western Europe, particularly in individuals who do not know they are infected with HIV until they are profoundly immunosuppressed and in HIV-infected patients who are not receiving ART or PCP prophylaxis.
PCP also develops in HIV-uninfected patients who are immunocompromised secondary to hematologic or malignant neoplasms, stem cell or solid organ transplantation, and immunosuppressive medications. The incidence of PCP depends on the degree of immunosuppression. PCP is increasingly reported among individuals receiving tumor necrosis factor α inhibitors and antilymphocyte monoclonal antibodies for rheumatologic or other diseases. While clinical PCP in immunocompetent hosts has not been clearly documented, studies have shown that Pneumocystis organisms can colonize the airways of children and adults who are not overtly immunocompromised. The relevance of these organisms to acute or chronic syndromes, such as chronic obstructive pulmonary disease (COPD), in immunocompetent patients is being investigated.
In some developing countries, the incidence of PCP among HIV-infected individuals has been found to be lower than that in industrialized countries. This lower incidence may be due to competing mortality from infectious diseases such as tuberculosis and bacterial pneumonia, which typically occur before patients become immunosuppressed enough to develop PCP. Geographic variations in Pneumocystis exposure and underdiagnosis also may explain the apparent lower frequency of PCP in some countries.