Fulminant Pneumocystis carinii Pneumonia in 4 Patients With Dermatomyositis

Abstract

Between 1989 and 1996, 4 cases of Pneumocystis carinii pneumonia (PCP) were observed in patients seronegative for the human immunodeficiency virus who were receiving corticosteroid therapy for dermatomyositis in our institution. These cases were considered unusual in light of the short delay of their onset after initiation of immunosuppressive therapy and their fulminant course: 3 of these patients died of PCP occurring during the first month of treatment with prednisone. In all 4 patients lymphopenia was observed before the initiation of corticosteroid treatment and low CD4 and CD8 cell counts were evident at the time of PCP. These observations support the view of an increase in both the severity and incidence of PCP in patients without human immunodeficiency virus infection and question the need for a primary prophylaxis in patients with connective tissue diseases receiving high-dose corticosteroid therapy.

When is it safe to stop Pneumocystis jiroveci pneumonia prophylaxis? Insights from three cases complicating autoimmune diseases

“…There is some evidence in the literature that a patient's underlying disease affects the risk of PCP. For example, some reports suggest that patients with dermatomyositis are at a heightened risk for PCP (20–22). The basis of this increased risk, if true, is not clear.…”

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies

“…Pneumonia by Pneumocystis jiroveci is a complication in immunocompromised patients and is seen in individuals submitted to high doses GC or other immunosuppressive treatments (B) [15,16] (C) [17,18]. Despite the controversies and lack of available evidence, prophylaxis The education of individuals with SAM, as well as their families and/or caregivers, is of great importance, since they are looking for environmental adaptations and implementation of rehabilitation programs aiming to maintain/improve the patient's quality of life.…”

Prophylaxis in dermatologic patients receiving immunosuppressive therapy

“…In patients with connective tissue diseases, high‐dose corticosteroid regimens and treatment with macrolactams, alkylating agents, or antimetabolites are considered major risk factors for the development of PCP (28–30). Based on the available literature, PCP chemoprophylaxis should always be considered in the patients listed in Table 6(1–31).…”