Efficacy of high-dose intravenous immunoglobulin therapy for peripheral neuropathy in the remission stage of eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome)

Abstract

BACKGROUND: Most patients with eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome) suffer from peripheral neuropathy. The neuropathy affects ADLs in not only the active stage, but also the remission stage of EGPA. Recently, high-dose intravenous immunoglobulin therapy (IVIg) is known to be effective for peripheral neuropathy in the active stage of EGPA. However, the effect of IVIG for peripheral neuropathy in the remission stage remains obscure. OBJECTIVE: This study assessed the efficacy of high-dose IVIg for peripheral neuropathy in the remission stage of EGPA. PATIENTS & METHODS: Six patients with peripheral neuropathy (duration: 3 months~7 years) in the remission stage of EGPA were investigated. IVIg was performed with an immunoglobulin dose of 400 mg/kg daily (intravenous drip) for 5 days. Neuropathy was evaluated with the manual muscle strength test (MMT) and the visual analogue scale (VAS). RESULTS: MMT improved in 4 of 7 patients (57.1%). MMT score increased 10.0±7.2 after IVIG. VAS (Numbness and neuralgia) improved in 6 of 7 patients (85.7%). Average VAS improved in the range of 46 mm to 61 mm. Side effect was detected in one patient (headache) only even though all patients underwent this therapy. CONCLUSION: These results suggested that IVIg therapy was safe and effective in patients with persistent peripheral neuropathy even in the remission stage of EGPA. © 2013 The Japan Society for Clinical Immunology.