Pediatric Nephrology

, Volume 28, Issue 1, pp 145–149

Atypical Pneumocystis jiroveci pneumonia with multiple nodular granulomas after rituximab for refractory nephrotic syndrome

  • Mai Sato
  • Shuichi Ito
  • Masao Ogura
  • Koichi Kamei
  • Isao Miyairi
  • Ippei Miyata
  • Masataka Higuchi
  • Kentaro Matsuoka
Brief Report

DOI: 10.1007/s00467-012-2286-6

Cite this article as:
Sato, M., Ito, S., Ogura, M. et al. Pediatr Nephrol (2013) 28: 145. doi:10.1007/s00467-012-2286-6

Abstract

Background

Rituximab, an anti-CD20 antibody that targets B cells, is a promising agent against steroid-dependent and steroid-resistant nephrotic syndrome in children.

Case-Diagnosis/Treatment

We report a 3-year-old boy who presented with atypical Pneumocystis jiroveci pneumonia (PCP) following administration of rituximab for refractory nephrotic syndrome. He had received cyclosporine and daily prednisolone for over 1 year. Following rituximab therapy, a hazy shadow was observed on his chest X-ray. Chest-computed tomography revealed multiple nodular lesions in bilateral lungs, although his clinical symptoms were subtle. PCR analysis demonstrated the presence of Pneumocystis DNA in his bronchoalveolar lavage. Lung wedge resection of the nodular lesion exhibited granulomas containing a few cysts of P. jiroveci that primarily consisted of T cells and histiocytes and lacked B cells. A deficiency of B cells following rituximab treatment suggests a dramatic effect on the immune response and, therefore, could result in granulomatous PCP. Nodular granulomatous lesions of PCP comprise an emerging concept previously reported in adults with hematological disease, bone marrow transplant, or treatment with rituximab. We report the first pediatric case of nodular PCP. Granulomatous PCP can be life-threatening. Moreover, bronchoalveolar lavage often fails to demonstrate the presence of P. jiroveci DNA. Wedge biopsy is warranted for definitive diagnosis. Our patient fully recovered with sulfamethoxazole/trimethoprim treatment because of early detection.

Conclusions

The indication of rituximab for refractory nephrotic syndrome has increased recently. Therefore, recognition of the risk of atypical PCP is important. Our findings suggest that PCP prophylaxis should be considered following rituximab therapy.

Keywords

Nephrotic syndromeRituximabPneumocystis jiroveciGranulomatousGranulomatous Pneumocystis jiroveci pneumonia

Abbreviations

PCP

Pneumocystis jiroveci pneumonia

RTX

Rituximab

NS

Nephrotic syndrome

FRNS

Frequently relapsing nephrotic syndrome

SDNS

Steroid-dependent nephrotic syndrome

SRNS

Steroid-resistant nephrotic syndrome

Copyright information

© IPNA 2012

Authors and Affiliations

  • Mai Sato
    • 1
  • Shuichi Ito
    • 1
  • Masao Ogura
    • 1
  • Koichi Kamei
    • 1
  • Isao Miyairi
    • 2
  • Ippei Miyata
    • 2
  • Masataka Higuchi
    • 3
  • Kentaro Matsuoka
    • 4
  1. 1.Division of Nephrology and RheumatologyNational Center for Child Health and DevelopmentSetagaya-kuJapan
  2. 2.Division of Infectious DiseasesNational Center for Child Health and DevelopmentSetagaya-kuJapan
  3. 3.Division of PulmonologyNational Center for Child Health and DevelopmentSetagaya-kuJapan
  4. 4.Division of PathologyNational Center for Child Health and DevelopmentSetagaya-kuJapan