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Proteinase 3-antineutrophil cytoplasmic antibody-positive ulcerative colitis presenting with abducens neuropathy
Yuki Kirito1, Daisuke Yamamoto1, Tsuyoshi Uchiyama1
1Department of Neurology, Seirei Hamamatu General Hospital, Hamamatsu, Shizuoka, Japan.
Abstract:
A 72-year-old man with ulcerative colitis (UC) presented with complete left abducens nerve palsy. Although MRI showed no significant changes, cerebrospinal fluid analysis revealed pleocytosis and elevated protein and interleukin (IL)-6 levels. His serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) level was also elevated to 31.1 U/mL, but granulomatosis with polyangiitis was not observed. On the basis of the diagnosis of autoimmune cranial neuropathy, he was treated with steroid therapy. While tapering steroid therapy, his serum PR3-ANCA levels; cerebrospinal fluid findings, including IL-6 levels; and symptoms improved. Serum PR3-ANCA could be a useful parameter of neurological disorders associated with ANCA-positive UC.
Insights
A patient with ulcerative colitis experienced abducens nerve palsy. Elevated proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) levels and cerebrospinal fluid changes improved with steroid therapy, suggesting PR3-ANCA
Area of Science:
- Neurology
- Immunology
- Gastroenterology
Background:
- Ulcerative colitis (UC) is a chronic inflammatory bowel disease.
- Neurological complications can occur in patients with UC.
- Cranial neuropathies are rare but serious manifestations.
Observation:
- A 72-year-old male with UC presented with complete left abducens nerve palsy.
- Cerebrospinal fluid analysis revealed pleocytosis and elevated protein and interleukin-6 (IL-6) levels.
- Elevated serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) was detected, without evidence of granulomatosis with polyangiitis.
Findings:
- Diagnosis of autoimmune cranial neuropathy was established.
- Treatment with steroid therapy led to improvement in symptoms.
- Tapering steroids correlated with improvements in serum PR3-ANCA, cerebrospinal fluid IL-6 levels, and neurological function.
Implications:
- Serum PR3-ANCA may serve as a biomarker for neurological disorders in ANCA-positive UC patients.
- This case highlights the potential autoimmune link between UC and cranial nerve palsies.
- Further research is warranted to explore the role of ANCA in UC-associated neurological complications.
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