Clinicopathological studies of peripheral neuropathy in Churg–Strauss syndrome

Abstract

Clinicopathological studies were performed on the visceral organs and the sural nerve of a male patient with Churg-Strauss syndrome (CSS) in order to understand the mechanisms of peripheral nervous system damage. A 67-year-old man, with a 2-year history of bronchial asthma, developed acutely painful paraplegia and dyspnea. Laboratory data showed a leukocytosis, an elevated serum creatinine kinase (CK) and marked eosionophilia. Autoantibodies including p- and c-ANCA were negative. Electrophysiological studies revealed a severe sensory-motor neuropathy of multiple mononeuritis type. Steroid pulse therapy performed a day after biopsy of skin, muscle and sural nerve was effective in resolving his respiratory and neurological dysfunction but a perforation of an intestinal ulcer occurred which required surgical intervention. In the biopsied sural nerve and the surgically resected intestine and mesentery there was vasculitis with fibrinoid necrosis accompanied by numerous eosinophils and macrophages containing eosinophil cationic protein (ECP). These findings suggest that in addition to ischemic changes due to vasculitis some neurotoxic substances generated by the eosinophils may be involved in the development of neuropathy in CSS.

Two subtypes of Churg–Strauss syndrome with neuropathy: the roles of eosinophils and ANCA

“…Unfortunately, ANCA results were not given, but six cases showed necrotizing vasculitis and 8 cases showed extravascular eosinophilic infiltrates, but only one patient presented an overlap of necrotizing vasculitis and extravascular eosinophilic infiltrates. Nagashima et al [24] reported an ANCA-negative patient with CSS in whom fibrinoid necrosis was shown in a biopsy from intestinal samples, but not in a biopsy from the sural nerve.…”

Clinical characterization and successful treatment of 6 patients with Churg–Strauss syndrome-associated neuropathy

“…In our 3 cases, pathological findings were essential for the diagnosis, and crucial in selecting the proper treatment in the 2 cases primarily expressing AIDP. The pathological findings of the sural nerve biopsies in cases 1 and 5, and muscle biopsy of rectus femoris in case 3, were compatible with eosinophilic necrotizing vasculitis of CSS [1,2,7,9].…”

“…There are not many articles describing CSS-associated neuropathy. These reports describe CSS-associated neuropathy in the relation to systemic small-vessel vasculitis, Wegener's granulomatosis and microscopic polyangiitis [4,5], or with central nervous system involvement [6], or present a single case [7,8];…”

“…Electrophysiological examinations show axonopathy at the involved extremities and this is based on pathological findings of necrotizing vasculitis. This involves small to medium sized arteries, and is characterized by destruction and hyaline degeneration of the wall, inner elastic lamina destruction, thrombotic occlusion and eosinophilic perivascular invasion [1,2,7,9]. The first-line of therapy for CSS, with or without neuropathy, is high-dose corticosteroid therapy often combined with cyclophosphamide.…”

Síndrome de Churg Strauss: uma vasculite rara

“…Uma de nossas pacientes apresentou acidente vascular cerebral isquêmico, sem seqüela. Um estudo com 47 pacientes mostrou que 53,2% apresentavam neuropatia sendo 68% mononeuropatia múltipla e 24% polineuropatia distal simétrica (11) . Guillevin, entretanto, não encontrou polineuropatia progressiva bilateral em sua série (9) ; em nossa casuís-tica todos os pacientes apresentaram mononeuropatia múltipla, grave, sendo necessário a introdução de ciclofosfamida em dois casos para controlar os sintomas.…”