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ANCA-associated vasculitis with protein-losing enteropathy is characterized by hypocomplementemia
Manaka Goto1, Yumi Tsuchida2, Keigo Terada1
1Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
Protein-losing enteropathy (PLE) is a rare complication in ANCA-associated vasculitis (AAV). Patients with AAV and PLE showed higher rates of hypocomplementemia, suggesting a link with the complement system.
Area of Science:
- Rheumatology
- Gastroenterology
- Immunology
Background:
- Protein-losing enteropathy (PLE) is linked to systemic autoimmune diseases.
- Reports of PLE in ANCA-associated vasculitis (AAV) are scarce, necessitating further investigation.
Purpose of the Study:
- To characterize the clinical features of AAV patients presenting with PLE.
- To compare the clinical course of AAV patients with and without PLE.
- To explore the potential association between PLE and hypocomplementemia in AAV.
Main Methods:
- Retrospective chart review of 68 AAV patients treated at the University of Tokyo Hospital (June 2003-June 2020).
- Analysis of demographic data, disease activity, organ involvement, and complement levels (CH50, C3, C4).
- Systematic literature review of PLE associated with vasculitis.
Main Results:
- Four AAV patients (5.9%) were diagnosed with PLE (MPA, GPA, EGPA).
- Patients with PLE exhibited significantly higher rates of hypocomplementemia compared to those without PLE.
- Hypoalbuminemia in PLE patients showed slow improvement despite immunosuppressive therapy for AAV.
Conclusions:
- PLE is an infrequent complication of AAV.
- The complement system may play a role in the pathogenesis of PLE in AAV.
- Further research is warranted to elucidate the mechanisms linking AAV, PLE, and complement abnormalities.
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