[Churg-Strauss syndrome: clinical features and long-term follow-up of 16 patients].
OBJECTIVE
To enhance the knowledge about Churg-Strauss syndrome (CSS).


METHODS
The clinical data of 16 CSS patients were analyzed. Follow-up was conducted for 42 (14 - 76) months.


RESULTS
All patients had the symptom of asthma. As in other case series, the lung, peripheral nervous system, and skin were the most commonly involved organs. During the active stage of the disease, most of the patients exhibited peripheral blood eosinophilia and elevated serum total IgE level. Two patients were treated with pulse treatment of methylprednisolone followed by prednisone with tapering dose and cyclophosphamide, nine patients were treated with corticosteroids and cyclophosphamide, and the others were treated with corticosteroids alone. Several patients were treated with the following drugs in combination: methotrexate, hydroxychloroquine, azathioprine, ciclosporin, common Threewingnut root, and intravenous injection of IgG. Symptom relief was seen in all patients after treatment. Two patients were lost to follow-up. Seven patients showed sustained remission, of which 3 had discontinued the medication, 3 were treated with low-dose corticosteroids and immunity inhibitors, and 1 continued to be treated with low-dose corticosteroids. Six patients suffered from relapse 1.7 times due to withdrawal of corticosteroid treatment or tapering of corticosteroid dose and they regained remission once more after increasing the corticosteroid dose and combination of cyclophosphamide. Four of these 6 patients were treated with corticosteroids of low or medium dose as maintenance doses, and the other 2 with medium-dose corticosteroids and cyclophosphamide. One patient committed suicide because of family dissension 1 year after treatment.


CONCLUSIONS
The clinical manifestations and course of CSS vary considerably. Its clinical manifestations may range from mild symptoms to life-threatening conditions. Corticosteroids and cyclophosphamide are both effective remedies. Despite relapses in several cases, the general prognosis is good.
Lou
Jian
J
Department of Respiratory Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
Xu
Wen-Bing
WB
D000293:Adolescent; D000328:Adult; D000368:Aged; D001249:Asthma / Q000188:drug therapy*; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult