Pneumocystis jirovecii pneumonia prophylaxis in immunocompromised patients with systemic autoimmune diseases

Pneumocystis jirovecii (P. jirovecii) causes a potentially fatal pneumonia in immunocompromised individuals (Pneumocystis pneumonia or PcP), particularly in HIV-infected patients and those treated with immunosuppressive drugs, such as transplant patients and those with systemic autoimmune diseases. P. jirovecii colonization can be found in almost a third of patients with systemic autoimmune diseases. Although the incidence of PcP in such patients is usually low, mortality is quite high, ranging between 30% and 50% in the majority of autoimmune diseases. PcP development is almost always observed in patients not receiving prophylaxis for the infection. Despite the above, there are no clinical guidelines established for PcP prophylaxis in patients with autoimmune diseases treated with glucocorticoids, cytotoxic drugs, or more recently, biological agents. The objective of this review is to analyze the available data on the incidence of PcP and the effect of PcP prophylaxis in patients with autoimmune diseases that may be useful in clinical practice.