Abstract

Abstract Pneumocystis jirovecii is commonly associated with a potentially fatal pneumonia in acquired immune deficiency syndrome patients, but its spread outside lungs is uncommon and poses a diagnostic challenge. Clinical awareness is therefore needed to start promptly adequate therapy. In this review, we report the case of an acquired immune deficiency syndrome patient with rapidly progressive disseminated pneumocystosis and subsequently analyze 189 other cases of extrapulmonary pneumocystosis described in literature. The most common extrapulmonary sites of Pneumocystosis jirovecii infection were spleen, lymph nodes, liver, kidneys, and eyes; clinical manifestations, which may occur either with or without lung involvement, ranged from asymptomatic incidental findings to a multifaceted disease with multisystem involvement. Human immunodeficiency virus (HIV) infection was documented in the majority of cases (91.6%), and concurrent or subsequent immunodeficiency-related conditions other than Pneumocystosis jirovecii were reported in 63.4% of patients, with cytomegalovirus (CMV) infection as the most common. In conclusion, disseminated pneumocystosis is a rare, little-known, but life-threatening opportunistic infection that must be taken into account, together with other immunodeficiency-related conditions such as Cytomegalovirus infection, to draw up an adequate diagnostic algorithm, establish a timely diagnosis, and ensure proper treatment.

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