Clinical characterization and successful treatment of 6 patients with Churg-Strauss syndrome-associated neuropathy.
OBJECTIVE
To confirm the reported findings and clarify unknown clinical features of Churg-Strauss syndrome (CSS)-associated neuropathy and design appropriate treatment.


METHODS
We assessed the clinical features of 6 patients with CSS-associated neuropathy.


RESULTS
Mononeuritis multiplex was present in 4 cases and polyneuropathy in the remaining cases. Both groups progressed to sensori-motor polyneuropathy in an acute or subacute course. All cases showed bronchial asthma and eosinophilia. Two cases with serum antineutrophil cytoplasmic antibodies to myeloperoxidase (MPO-ANCA) had an acute clinical course and severe symptoms. Nerve conduction studies (NCS) of these 2 cases revealed conduction blocks at the initial stage, although NCS finally indicated sensori-motor axonopathy at the involved extremities. For treatment, high-dose corticosteroid therapy for 4 cases, and cyclophosphamide combined with corticosteroids for 1 case, were effective. For the remaining case, intravenous immunoglobulin (IVIg) at the chronic phase resulted in a slow improvement of neuropathy in the symptomatic aspect. There was no relapse of neuropathy with low-dose corticosteroid treatment for 14-24 months after the initial treatment, except 1 case. There was also no relapse in the other case that was treated with moderate-dose steroids.


CONCLUSIONS
Our study showed that CSS-associated neuropathy is a treatable disorder and that the first choice therapy is high-dose corticosteroid. In cases where corticosteroids are ineffective or for severe cases, immunosuppressive therapy (cyclophosphamide) with steroids should be considered, and IVIg might be a treatment option.
Nakamura
Masakazu
M
Department of Neurology, Hokkaido University Graduate School of Medicine, N15W7, Kita-ku, Sapporo 060-8638, Japan.
Yabe
Ichiro
I
Yaguchi
Hiroaki
H
Kishimoto
Riichiro
R
Mito
Yasunori
Y
Fujiki
Naoto
N
Houzen
Hideki
H
Tsuji-Akimoto
Sachiko
S
Niino
Masaaki
M
Sasaki
Hidenao
H
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004338:Drug Combinations; D004802:Eosinophilia / Q000150:complications / Q000628:therapy; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007964:Leukocytosis / Q000150:complications / Q000628:therapy; D008297:Male; D008875:Middle Aged; D020422:Mononeuropathies / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D009431:Neural Conduction; D011115:Polyneuropathies / Q000150:complications / Q000503:physiopathology / Q000628:therapy
D000305:Adrenal Cortex Hormones; D004338:Drug Combinations; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide