Clinicopathological features of Churg–Strauss syndrome-associated neuropathy

Abstract

We assessed the clinicopathological features of 28 patients with peripheral neuropathy associated with Churg-Strauss syndrome. Initial symptoms attributable to neuropathy were acute painful dysaesthesiae and oedema in the dysaesthetic portion of the distal limbs. Sensory and motor involvement mostly showed a pattern of mononeuritis multiplex in the initial phase, progressing into asymmetrical polyneuropathy, restricted to the limbs. Parallel loss of myelinated and unmyelinated fibres due to axonal degeneration was evident as decreased or absent amplitudes of sensory nerve action potentials and compound muscle action potentials, indicating acute massive axonal loss. Epineurial necrotizing vasculitis was seen in 54% of cases; infiltrates consisted mainly of CD8-positive suppressor/cytotoxic and CD4-positive helper T lymphocytes. Eosinophils were present in infiltrates, but in smaller numbers than lymphocytes. CD20-positive B lymphocytes were seen only occasionally. Deposits of IgG, C3d, IgE and major basic protein were scarce. The mean follow-up period was 4.2 years, with a range of 8 months to 10 years. Fatal outcome was seen only in a single patient, indicating a good survival rate. The patients who responded well to the initial corticosteroid therapy within 4 weeks regained self-controlled functional status in longterm follow-up (modified Rankin score was < or = 2), while those not responding well to the initial corticosteroid therapy led a dependent existence (P < 0.01). In addition the patients with poor functional outcomes had significantly more systemic organ damage caused by vasculitis (P < 0.05). Necrotizing vasculitis mediated by cytotoxic T cells, leading to ischaemic changes, appears to be a major cause of Churg-Strauss syndrome-associated neuropathy. The initial clinical course and the extent of systemic vasculitic lesions may influence the long-term functional prognosis.

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

“…there is only one previous report solely describing CSS-associated neuropathy patients [9]. The mean onset age of neuropathy was about 50 years old, but the individual age of onset was wide-ranged, 15-78 years old [4,6,9].…”

“…The mean onset age of neuropathy was about 50 years old, but the individual age of onset was wide-ranged, 15-78 years old [4,6,9]. There is controversy over the presence of a gender bias as the female ratio of CSS-associated neuropathy patients was reported to be 26.7 % [4], 55.3 % [6], and 78.6 % [9].…”

“…In these reports, the neuropathy initially presents as sensory disturbances of the extremities. These disturbances predominantly present as mononeuritis multiplex, and rapidly progress to polyneuropathy [9]. Electrophysiological examinations show axonopathy at the involved extremities and this is based on pathological findings of necrotizing vasculitis.…”

“…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.…”

“…intravenous immunoglobulin, interferon-α) [2,10]. Although corticosteroids were reported to be effective for neuropathy [9], the clinical improvements and long-term prognosis of these patients have not been fully characterized.…”