Background/Purpose: Pneumocystis jiroveciipneumonia (PCP) is a fatal complication in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). However, the current situation of prophylactic treatment of PCP and its effects on patients with AAV remain unknown. Therefore, we aimed to elucidate the current situation of prophylactic treatment and incidence of PCP in Japanese patients with AAV.

Methods: This study was conducted using the database of the RemIT-JAV-RPGN study. The current study included 321 patients who were registered from 53 tertiary care institutions in Japan and were newly diagnosed with AAV between April 2011 and March 2014. AAV was diagnosed based on the European Medicines Agency (EMA) algorithm. The exclusion criteria were as follows: age < 20 years, recurrent AAV, serological evidence of hepatitis B or C virus infection, and a history of malignancy. During the 2-year observation period, we examined the incidence of PCP, frequency of the usage and side effects of prophylactic drugs for PCP, and backgrounds of patients developing PCP.

Results: Of the 321 patients, 2 were excluded due to the lack of follow-up data. The mean age of the 319 patients was 69 ± 13 years, and 140 patients (44%) were males. Using the EMA algorithm, we identified 27 patients (8%) with eosinophilic granulomatosis with polyangiitis, 53 (17%) with granulomatosis with polyangiitis, 198 (62%) with microscopic polyangiitis/renal limited vasculitis, and 42 (13%) with unclassifiable disorders. The mean initial daily dose of prednisolone (PSL) was 40 ± 13 mg, and methylprednisolone (mPSL) pulse therapy was administered to 145 (45%) patients. Concomitant cyclophosphamide (CY) was administered during the initial 3 weeks of remission induction therapy in 105 (33%) patients. Most patients received prophylactic treatment of PCP along with immunosuppressive agents. Trimethoprim–sulfamethoxazole (TMP–SMX) and pentamidine were administered to 81% and 5% patients, respectively, during the initial 3 months. The percentage of patients treated with TMP–SMX and pentamidine gradually decreased. The following side effects of TMP–SMX were observed: cytopenia, 7 patients; drug eruption, hyponatremia, peripheral neuropathy, and liver function disorder, 2 patients each; and renal dysfunction, 1 patient. No side effect of pentamidine was recognized. During the 2-year observation period, 176 severe infections occurred in 111 (35%) of the 319 patients. Bacterial pneumonia was most frequently observed (34 times), followed by cytomegalovirus infection (28 times). However, PCP occurred only 9 times in 9 patients, and its incidence occupied only 5% of the total severe infections. However, of 26 patients who died, 13 patients died from infectious diseases, of which 4 were due to PCP. The mean age of 9 patients with PCP was 73 ± 10 years and 6 were males. The median duration (min–max) to PCP onset was 96 (15–626) days. The mean initial daily dose of PSL was higher in patients in whom PCP occurred than in those in whom it did not occur (54 ± 24 mg/day vs. 41 ± 12 mg/day). The rates of mPSL pulse and concomitant CY therapies were equal in both groups of patients.

Conclusion: Although the incidence of PCP onset is low in Japanese patients with AAV, the mortality rate is high.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prophylactic-treatment-and-incidence-of-pneumocystis-jirovecci-pneumonia-in-japanese-patients-with-antineutrophil-cytoplasmic-antibody-associated-vasculitis/