Abstract

Objective To investigate clinical features, independent associated factors, treatment, and outcome of patients with peripheral neuropathy (PN) in eosinophilic granulomatosis with polyangiitis (EGPA). Methods We retrospectively analyzed clinical data of 110 EGPA patients from 2007 to 2019 in Peking Union Medical College Hospital. The independent factors associated with PN in EGPA were analyzed with univariate and multivariate logistic regressions. Results In EGPA with PN, paresthesia and muscle weakness were observed in 82% and 33% of patients, respectively. Both the upper and lower limbs were involved in 51% of patients. 30% of EGPA patients had symmetrical multiple peripheral neuropathy, whereas only 16.4% presented with mononeuritis multiplex. Compared to patients without PN, patients with PN had a higher erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, Birmingham vasculitis activity score (BVAS), and positivity of myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA). Regarding manifestations, patients with PN tended to develop weight loss and arthritis or joint pain. Notably, ANCA positivity, arthritis or joint pain, and higher BVAS were found to be independent associated factors for PN in EGPA. Patients with PN more frequently need glucocorticoid pulses and intravenous infusion of cyclophosphamide. With the longest follow-up of 11.0 years, we found that age and cardiac involvement were risk factors for survival, and female was the protective factor. Conclusion PN in EGPA frequently displays with symmetrical multiple peripheral neuropathy in China. Positive ANCA, arthritis or joint pain, and higher BVAS are the independent associated factors of PN in EGPA. Glucocorticoids with immunosuppressants are vital therapeutic strategy.

Highlights

  • Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss Syndrome, is an antineutrophil cytoplasmic antibody- (ANCA-) associated systemic necrotizing small-vessel vasculitis (AAV) [1]

  • EGPA is a form of AAV, ANCA only exists in approximately one-third of EGPA patients, which always displays with a perinuclear labeling on immunofluorescence analysis with specificity against myeloperoxidase (MPO) [1, 6, 7].The disease course of classical EGPA is roughly divided into three phases

  • peripheral neuropathy (PN) was observed as an initial manifestation in 5 EGPA patients

Read more

Summary

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss Syndrome, is an antineutrophil cytoplasmic antibody- (ANCA-) associated systemic necrotizing small-vessel vasculitis (AAV) [1]. EGPA is a form of AAV, ANCA only exists in approximately one-third of EGPA patients, which always displays with a perinuclear labeling on immunofluorescence analysis with specificity against myeloperoxidase (MPO) [1, 6, 7].The disease course of classical EGPA is roughly divided into three phases. The first phase is prodromal phase, which is manifested as allergic symptoms such as asthma, sinusitis, nasal polyps, or allergic rhinitis. This phase can last for several decades.

Methods
Results
Discussion
Conclusion
Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call