SCHNITZLER SYNDROME - A BROADER CLINICAL SPECTRUM

被引：40

作者：

BERDY, SS

BLOCH, KJ

机构：

[1] MASSACHUSETTS GEN HOSP,GEN MED SERV,CLIN IMMUNOL UNIT,55 FRUIT ST,BOSTON,MA 02114

[2] HARVARD UNIV,SCH MED,DEPT MED,BOSTON,MA 02115

[3] MASSACHUSETTS GEN HOSP,GEN MED SERV,ALLERGY UNIT,BOSTON,MA 02114

来源：

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY | 1991年 / 87卷 / 04期

关键词：

D O I：

10.1016/0091-6749(91)90132-8

中图分类号：

R392 [医学免疫学];

学科分类号：

100102 ;

摘要：

Schnitzler's syndrome is characterized by chronic urticaria, recurrent fever, bone pain, and lymphadenopathy in conjunction with a serum IgM M component in a concentration that is usually < 10,000 mg/L. Complement activation and cryoprecipitation do not appear to be involved. We report two additional patients who share many of the characteristics of this entity. These patients differ from patients previously reported because of the markedly elevated IgM M-component concentration in one patient and the severity of anemia in the second patient. An increased frequency of IgG autoantibodies to interleukin-1-alpha has been reported by other investigators; it has been suggested that an antibody-mediated prolongation of the half-life of interleukin-1-alpha might account for some of the symptoms and signs of this disorder. However, neither the mediators involved in the induction of nonpruritic urticaria nor the role of the IgM M component has been established.

引用

页码：849 / 854

页数：6

共 25 条

[1] SYSTEMIC CAPILLARY LEAK SYNDROME AND MONOCLONAL IGG GAMMOPATHY - STUDIES IN A 6TH PATIENT AND A REVIEW OF LITERATURE [J].

ATKINSON, JP ;

WALDMANN, TA ;

STEIN, SF ;

GELFAND, JA ;

MACDONALD, WJ ;

HECK, LW ;

COHEN, EL ;

KAPLAN, AP ;

FRANK, MM .

MEDICINE, 1977, 56 (03) :225-239

[2]

BARRIERE H, 1976, SEM HOP PARIS, V52, P221

[3]

BARRIERE H, 1974, B SOC FR DERMATOL SY, V81, P361

[4] CHRONIC URTICARIA WITH MONOCLONAL IGM - A REPORT OF 5 CASES [J].

CLAUVEL, JP ;

BROUET, JC ;

DANON, F ;

LEIBOWITCH, M ;

SELIGMANN, M .

CLINICAL IMMUNOLOGY AND IMMUNOPATHOLOGY, 1982, 25 (03) :348-353

[5]

DIDIERJEAN L, 1990, J INVEST DERMATOL, V94, P575

[6]

DOUTRE MS, 1987, ANN ALLERGY, V58, P413

[7]

FARHANGI M, 1986, SEMIN ONCOL, V13, P366

[8] ACQUIRED C1-INHIBITOR DEFICIENCY ASSOCIATED WITH ANTIIDIOTYPIC ANTIBODY TO MONOCLONAL IMMUNOGLOBULINS [J].

GEHA, RS ;

QUINTI, I ;

AUSTEN, KF ;

CICARDI, M ;

SHEFFER, A ;

ROSEN, FS .

NEW ENGLAND JOURNAL OF MEDICINE, 1985, 312 (09) :534-540

[9] ACQUIRED C1 ESTERASE INHIBITOR DEFICIENCY AND ANGIOEDEMA - REVIEW [J].

GELFAND, JA ;

BOSS, GR ;

CONLEY, CL ;

REINHART, R ;

FRANK, MM .

MEDICINE, 1979, 58 (04) :321-328

[10]

GOREVIC PD, 1988, IMMUNOLOGICAL DISEAS, P1687

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