Differential clinicopathologic features of EGPA-associated neuropathy with and without ANCA
| Autor: | Shohei Ikeda, Masahiro Iijima, Haruki Koike, Ryoji Nishi, Gen Sobue, Yuichi Kawagashira, Yuki Fukami, Masahisa Katsuno, Ken Ohyama |
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| Rok vydání: | 2020 |
| Předmět: |
Lung Diseases
Male Pathology Neural Conduction Churg-Strauss Syndrome Pathogenesis 0302 clinical medicine immune system diseases Eosinophilic Medicine Muscle Weakness biology Electrodiagnosis Peripheral Nervous System Diseases Middle Aged Magnetic Resonance Imaging Otorhinolaryngologic Diseases Lower Extremity Myeloperoxidase Female Kidney Diseases medicine.symptom Granulomatosis with polyangiitis Vasculitis medicine.medical_specialty Myeloblastin Ischemia Inflammation Skin Diseases Vascular Vascular occlusion Antibodies Antineutrophil Cytoplasmic Upper Extremity 03 medical and health sciences Sural Nerve Humans Peripheral Nerves cardiovascular diseases Aged Peroxidase 030203 arthritis & rheumatology business.industry medicine.disease Asthma respiratory tract diseases Somatosensory Disorders biology.protein Neurology (clinical) Tomography X-Ray Computed business 030217 neurology & neurosurgery |
| Zdroj: | Neurology. 94:e1726-e1737 |
| ISSN: | 1526-632X 0028-3878 |
| Popis: | ObjectiveTo investigate the clinicopathologic features of eosinophilic granulomatosis with polyangiitis (EGPA)–associated neuropathy with a focus on the presence or absence of anti-neutrophil cytoplasmic antibodies (ANCAs).MethodsWe examined the clinical features and pathologic findings of sural nerve biopsy specimens from 82 patients with EGPA-associated neuropathy. Of these patients, 32.9% were myeloperoxidase (MPO)-ANCA positive, and 67.1% were MPO-ANCA negative. PR3-ANCA was negative in all of 78 examined patients.ResultsUpper limb symptoms were more frequently reported as initial neuropathic manifestations in the MPO-ANCA–positive group than in the MPO-ANCA–negative group (44.4% vs 14.6%, p < 0.01). The serum levels of C-reactive protein were significantly higher in the MPO-ANCA–positive group than in the MPO-ANCA–negative group (p < 0.05). Sural nerve biopsy specimens showed findings suggestive of vasculitis (i.e., destruction of vascular structures) in epineurial vessels; these results were seen more frequently in the MPO-ANCA–positive group than in the MPO-ANCA–negative group (p < 0.0001). Conversely, the numbers of eosinophils in the lumen of the epineurial vessels (p < 0.01) and epineurial vessels occluded by intraluminal eosinophils (p < 0.05) were higher in the MPO-ANCA–negative group than in the MPO-ANCA–positive group. Furthermore, the incidence of eosinophil infiltration in the endoneurium was higher in the MPO-ANCA–negative group than in the MPO-ANCA–positive group (p < 0.01).ConclusionsThis study suggests that the pathogenesis of EGPA comprises at least 2 distinct mechanisms: ANCA-associated vasculitis resulting in ischemic effects and inflammation, which is prominent in MPO-ANCA–positive patients, and eosinophil-associated vascular occlusion leading to ischemia and eosinophil-associated tissue damage, which is conspicuous in MPO-ANCA–negative patients. |
| Databáze: | OpenAIRE |
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