Proteinase 3-antineutrophil cytoplasmic antibody-positive ulcerative colitis presenting with abducens neuropathy

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.

The Impact of Serum Anti-neutrophil Cytoplasmic Antibody on Clinical Characteristics and Outcomes in Pediatric-Onset Systemic Lupus Erythematosus Patients

“…Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies directed against cytoplasmic antigens within human neutrophils and monocytes and have been detected in patients with small vessel vasculitis and inflammatory disease (2)(3)(4). In fact, vasculitis is a common manifestation in lupus patients, and the presence of ANCAs related to clinical presentation, including interstitial lung disease, chronicity index and histopathology of lupus nephritis, has been flourishingly reported in adult lupus patients in this 5-year study (5)(6)(7)(8)(9).…”

Proteinase-3-(cANCA)-assoziierte Colitis ulcerosa mit möglicher entzündlicher leukenzephalopathischer ZNS‑Beteiligung

“…Demyelinisierungsvorgänge im ZNS wurden auch nach Einnahme von TNF-α-Blockern bei entzündlichen Darmerkrankungen berichtet [6], diese hatte die Patientin aber bisher nicht eingenommen. Der Fall einer Proteinase-3-Antikörper-assoziierten Colitis ulcerosa mit einer Abduzensparese wurde bisher allerdings erst einmal bei einem 72-jährigen japanischen Patienten von Kirito et al veröffentlicht [7]. Auch in diesem Fall war die Erstdiagnose der Kolitis der neurologischen Symptomatik um einige Jahre vorausgegangen, und es fanden sich ebenfalls keine klinischen Hinweise für eine Granulomatose mit Polyangiitis.…”