Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) presenting with polyneuropathy--a case series.

OBJECTIVES

We aim to characterize a group of patients with eosinophilic granulomatosis with polyangiitis (EGPA) with an initial presentation of peripheral neuropathy.

METHODS

A retrospective analysis of 11 patients with EGPA.

RESULTS

The most common chief complaint was neuropathic limb pain and numbness (100%), followed by extremity weakness (82%). Nine (82%) patients had acute to subacute onset. All patients had a history of asthma and serum eosinophilia of more than 11%. Combining clinical and electrophysiological data, 10 (91%) patients demonstrated notable asymmetric involvement, whereas 1 patient presented with a length-dependent symmetrical axonal polyneuropathy. All patients improved significantly after immunotherapy, with average time to improvement of 9 weeks. Ten (91%) patients improved steadily and experienced no relapse, whereas 1 relapsed within the first year.

CONCLUSIONS

EGPA should be suspected in patients with asymmetric axonal peripheral neuropathy associated with asthma and eosinophilia. Early diagnosis and aggressive treatment help to achieve favorable outcome.