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.

[1]  M. Carballeira,et al.  Peripheral polyneuropathy and Churg-Strauss syndrome. , 2014 .

[2]  M. Carballeira,et al.  Polineuropatía periférica y síndrome de Churg-Strauss , 2014 .

[3]  A. Grau,et al.  Peripheral neuropathy as initial manifestation of primary systemic vasculitides , 2013, Journal of Neurology.

[4]  A. Mahadevan,et al.  Usefulness of superficial peroneal nerve/peroneus brevis muscle biopsy in the diagnosis of vasculitic neuropathy , 2012, Journal of Clinical Neuroscience.

[5]  N. Arden,et al.  Peripheral neuropathy in ANCA-associated vasculitis: outcomes from the European Vasculitis Study Group trials. , 2011, Rheumatology.

[6]  D A Bloch,et al.  The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). , 2010, Arthritis and rheumatism.

[7]  F. Buggle,et al.  Neurologic complications of Churg–Strauss syndrome – a prospective monocentric study , 2010, European journal of neurology.

[8]  H. Sasaki,et al.  Clinical characterization and successful treatment of 6 patients with Churg–Strauss syndrome-associated neuropathy , 2009, Clinical Neurology and Neurosurgery.

[9]  G. Espinosa,et al.  Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review. , 2007, JAMA.

[10]  L. Cattaneo,et al.  Peripheral neuropathy in Wegener’s granulomatosis, Churg–Strauss syndrome and microscopic polyangiitis , 2007, Journal of Neurology, Neurosurgery, and Psychiatry.

[11]  G. Sobue,et al.  Clinicopathological features of Churg-Strauss syndrome-associated neuropathy. , 1999, Brain : a journal of neurology.

[12]  J. Mcleod,et al.  Vasculitis confined to peripheral nerves. , 1996, Brain : a journal of neurology.

[13]  T. Colby,et al.  Neurologic manifestations of Churg-Strauss syndrome. , 1995, Mayo Clinic proceedings.

[14]  P. Dyck,et al.  Vasculitic Neuropathies , 2015, Current Treatment Options in Neurology.

[15]  K. Chakravarty,et al.  BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. , 2014, Rheumatology.

[16]  E. Kararizou,et al.  Churg-Strauss syndrome complicated by neuropathy: a clinicopathological study of nine cases. , 2011, Clinical neuropathology.