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ANCA-associated vasculitis with protein-losing enteropathy is characterized by hypocomplementemia

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Abstract

Protein-losing enteropathy (PLE) has been reported to be associated with various systemic autoimmune diseases. However, reports regarding PLE in ANCA-associated vasculitis (AAV) patients are limited. We herein aimed to describe the clinical characteristics of AAV with PLE. We conducted a retrospective chart review of patients who were diagnosed with AAV and who began treatment at the University of Tokyo Hospital between June 2003 and June 2020. Among 68 AAV patients, there were four patients (5.9%) with PLE, consisting of two patients with MPA, one patient with GPA, and one patient with EGPA. Clinical courses were described, and their data were compared with AAV patients without PLE. Demographic characteristics, disease activity, and the pattern of organ involvement were similar between patients with PLE and without PLE. Patients with PLE had hypocomplementemia more frequently than the patients without PLE (CH50 75.0% vs 1.8%, p < 0.001, C3 50.0% vs 1.8%, p = 0.01, C4 75.0% vs 3.5%, p = 0.001). Although hypoalbuminemia improved with immunosuppressive therapy for AAV, the improvement in hypoalbuminemia was slow in most cases. We also performed a systematic review on PLE associated with vasculitis. Thirteen reports were included, and Henoch–Schonlein Purpura patients with PLE also tended to have hypocomplementemia. In conclusion, PLE is a rare complication of AAV and complement system may associate with the mechanism of PLE.

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Acknowledgements

We would like to express our gratitude to the patients at our hospital for their cooperation with this study.

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Authors and Affiliations

  1. Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan

    Manaka Goto, Yumi Tsuchida, Keigo Terada, Hideyuki Takahashi, Yusuke Sugimori, Toshihiko Komai, Yukiko Iwasaki, Hirofumi Shoda & Keishi Fujio

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  1. Manaka Goto
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  2. Yumi Tsuchida
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  3. Keigo Terada
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  4. Hideyuki Takahashi
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  6. Toshihiko Komai
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  7. Yukiko Iwasaki
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Contributions

Study design: MG, YT, HS, KF. Data acquisition: MG, YT, KT, HT, YS, TK, YI. Data analysis: MG. Manuscript preparation: MG, YT.

Corresponding author

Correspondence to Yumi Tsuchida.

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Conflicts of interest

MG has received speaking fees from Bristol-Myers Squibb and Ono Pharmaceutical. YT has received grants from GlaxoSmithKline and speaking fees from GlaxoSmithKline and Chugai. TK has received speaking fees and/or honoraria from Tanabe Mitsubishi, Kissei, Pfizer, Amgen and received grants and speaking fees from GlaxoSmithKline. HS has received fees from Pfizer, Bristol-Myers Squibb, Eli Lilly, Sanofi, AbbVie, GlaxoSmithKline, Gilead, Boehringer Ingelheim, Jansen, Novartis, Chugai, Takeda, Astellas, Eisai, Asahi Kasei and Daiichi-Sankyo, and has received research grants from Novartis. KF has received speaking fees and/or honoraria from Tanabe Mitsubishi, Bristol-Myers Squibb, Eli Lilly, Chugai, Jansen, Pfizer, Ono, Abbie, Ayumi, Astellas, Sanofi, Novartis, Daiichi Sankyo, Eisai, Asahi Kasei, Japan Blood Products Organization, and Kowa, and has received research grants from Tanabe Mitsubishi, Bristol-Myers Squibb, Eli Lilly, Chugai, Abbie, Ayumi, Astellas, Sanofi, Eisai, and Asahi Kasei.

Ethics approval

This study was approved by the ethics committee of the University of Tokyo Hospital (2431) on February 20th in 2009.

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The ethics committee at our institution does not require patient consent for retrospective chart studies. Patients were provided chances to opt out of the study.

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Goto, M., Tsuchida, Y., Terada, K. et al. ANCA-associated vasculitis with protein-losing enteropathy is characterized by hypocomplementemia. Rheumatol Int 42, 1863–1872 (2022). https://doi.org/10.1007/s00296-021-04923-2

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