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Systemic AA-amyloidosis related to MPO-ANCA microscopic polyangiitis: a case report
Yoshifumi Ubara1, Tetsuo Tagami, Tatsuya Suwabe
1Nephrology Center, Toranomon Hospital, Tokyo, Japan. ubara@toranomon.gr.jp
Summary
This autopsy case study reveals how myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis can lead to systemic AA amyloidosis, causing severe gastrointestinal issues and organ damage.
Area of Science:
- Pathology
- Immunology
- Nephrology
Background:
- Microscopic polyangiitis is a systemic vasculitis often associated with anti-neutrophil cytoplasmic antibodies (ANCA).
- AA amyloidosis is a condition where amyloid protein A deposits in organs, often secondary to chronic inflammation.
Observation:
- The patient presented with MPO-ANCA-positive microscopic polyangiitis and systemic AA amyloidosis.
- Autopsy revealed inactive vasculitis in multiple organs, including the kidney, lung, and intestinal tract.
- Significant thickening of gastrointestinal viscera was noted, with AA-amyloid deposits in arterioles and surrounding tissues.
Findings:
- AA amyloidosis was widespread, affecting the heart, kidney, gallbladder, pancreas, salivary gland, and subcutis.
- The patient died due to intractable enteritis, likely a complication of the vasculitis and amyloidosis.
- Inactive MPO-ANCA-positive microscopic polyangiitis was identified as the probable cause of the patient's AA amyloidosis.
Implications:
- This case highlights a potential link between ANCA-associated vasculitis and the development of secondary AA amyloidosis.
- Understanding this association is crucial for diagnosing and managing complex inflammatory and deposition diseases.
- Further research may elucidate the mechanisms driving amyloid formation in ANCA vasculitis.
