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All strategies to prevent Pneumocystis jiroveci pneumonia (PCP) during rituximab treatment have their rationale in patients with granulomatosis with polyangiitis (GPA) and to some extent in patients with other autoimmune diseases (AID). Risk factors of PCP and severe infections are very similar in GPA patients. The decision of PCP prophylaxis should not be limited at RTX initiation and during RTX treatment, but should be reassessed continuously in all GPA patients. Since PCP increases the mortality risk in GPA (and AID) patients, the treating physician should always consider PCP as a possible diagnosis in patients treated with RTX - receiving or not PCP prophylaxis.
PCP in GPA patients has always challenged rheumatologists and internists. While PCP is rare, it is a serious complication of therapy in GPA.
PCP prophylaxis has worked well with HIV positive patients compared with non-HIV positive patients and particularly GPA patients. To this date, EULAR only encourages the use of PCP prophylaxis when AAV patients are treated with cyclophosphamide. Many experts also advocated PCP prophylaxis in GPA patients in other circumstances, such as low lymphocyte and CD4 cell counts, prolonged daily oral prednisolone dose > 15-20 mg and lately during RTX therapy.
The purpose of this commentary is to analyse the benefits and caveats of PCP prophylaxis in GPA patients. Although the commentary focus on GPA, I firmly believe that it also apply to other autoimmune diseases.