Clinical conditions associated with PCP in children

Abstract

Pneumocystis jirovecii is a leading cause of opportunistic infections among the immune compromised. During the 1980s, attention focused on patients with HIV, however, with the advent of anti-retroviral therapy, we wished to revisit the question of underlying diseases associated with Pneumocystis pneumonia in children. We identified 80 cases from 1986 to 2006 and performed a retrospective chart review to identify clinical characteristics for each of the cases. HIV was the single most common associated underlying condition seen in this cohort, accounting for 39% of the cases overall, however, it was seen in just 15% of the cases since 1998. Transplant recipients and oncology patients together comprised another 39% of the cases, with 9% of cases attributed to primary immune deficiency and another 9% of cases associated with less well-recognized causes of susceptibility. This study documents the ongoing need for vigilance to diagnose Pneumocystis pneumonia in less well-recognized clinical scenarios.

Pneumocystis jirovecii pneumonia as an initial manifestation of hyper-IgM syndrome in an infant

“…[1,2] HIV infection has been regarded as an important risk factor for PCP; however, immunocompromised patients who are HIV-negative have recently occupied a greater proportion of patients with PCP than patients with HIV infection. [3] Although most immunocompromised children who are HIV-negative are those with hematologic malignancies or solid tumors and hematopoietic cell or solid-organ transplant recipients, children with PID are also at risk for PCP. [3] CD4+ T cells play an essential role in the host immune defense to P. jirovecii infection, [7] and therefore, T cell impairment rather than B cell or phagocytic impairment, is thought to be a risk factor for PCP among children with PID.…”

“…[3] Although most immunocompromised children who are HIV-negative are those with hematologic malignancies or solid tumors and hematopoietic cell or solid-organ transplant recipients, children with PID are also at risk for PCP. [3] CD4+ T cells play an essential role in the host immune defense to P. jirovecii infection, [7] and therefore, T cell impairment rather than B cell or phagocytic impairment, is thought to be a risk factor for PCP among children with PID. [2] Severe combined immune deficiency, Wiskott-Aldrich syndrome, and HIGM syndrome are the most common PIDs among children with PCP.…”

“…[2] Severe combined immune deficiency, Wiskott-Aldrich syndrome, and HIGM syndrome are the most common PIDs among children with PCP. [3]…”

“…[2] Accordingly, the suspicion and diagnosis of PCP in children without any evidence of an underlying immunocompromised state are not easy. Primary immune deficiency (PID), as well as HIV infection, malignancy, and transplantation, are known as risk factors for PCP, [3] and PCP can be an initial clinical manifestation of PID. [4–6]…”

Pneumocystis jirovecii Pneumonia in Pediatric Inflammatory Bowel Disease: A Case Report and Literature Review

“…PCP has been recognized as a disease in children with primary cell-mediated immunodeficiency, patients receiving chemotherapy for hematological malignancies, solid organ, and bone marrow transplant recipients and in patients requiring immunosuppressants (5). Notably, the incidence in the immunosuppressed non-HIV population is increasing with the escalating use of immunosuppressive agents (4, 6, 7). Inflammatory autoimmune conditions such as IBD account for up to 20% of PCP in HIV-negative patients (8).…”