Differential clinicopathologic features of EGPA-associated neuropathy with and without ANCA

被引:62
作者
Nishi, Ryoji [1 ]
Koike, Haruki [1 ]
Ohyama, Ken [1 ,3 ]
Fukami, Yuki [1 ]
Ikeda, Shohei [1 ]
Kawagashira, Yuichi [1 ]
Iijima, Masahiro [1 ]
Katsuno, Masahisa [1 ]
Sobue, Gen [2 ]
机构
[1] Nagoya Univ, Grad Sch Med, Dept Neurol, Nagoya, Aichi, Japan
[2] Nagoya Univ, Grad Sch Med, Res Div Dementia & Neurodegenerat Dis, Nagoya, Aichi, Japan
[3] Okazaki City Hosp, Dept Neurol, Okazaki, Aichi, Japan
关键词
CHURG-STRAUSS-SYNDROME; ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES; NONSYSTEMIC VASCULITIC NEUROPATHY; EOSINOPHILIC GRANULOMATOSIS; MICROSCOPIC POLYANGIITIS; CLASSIFICATION;
D O I
10.1212/WNL.0000000000009309
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objective To 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). Methods We 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. Results Upper 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). Conclusions This 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.
引用
收藏
页码:E1726 / E1737
页数:12
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