Differential clinicopathologic features of EGPA-associated neuropathy with and without ANCA.
Neurology
; 94(16): e1726-e1737, 2020 04 21.
Article
de En
| MEDLINE
| ID: mdl-32217776
ABSTRACT
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.
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Nerfs périphériques
/
Syndrome de Churg-Strauss
/
Neuropathies périphériques
/
Faiblesse musculaire
/
Anticorps anti-cytoplasme des polynucléaires neutrophiles
/
Troubles somatosensoriels
Type d'étude:
Etiology_studies
/
Risk_factors_studies
Limites:
Aged
/
Female
/
Humans
/
Male
/
Middle aged
Langue:
En
Journal:
Neurology
Année:
2020
Type de document:
Article
Pays d'affiliation:
Japon