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  • Title: [Hyper-IgD-syndrome].
    Author: Suschke J, Walther JU.
    Journal: Z Rheumatol; 1997; 56(1):40-2. PubMed ID: 9173750.
    Abstract:
    UNLABELLED: We report on a 6-year-old Romanian girl with recently diagnosed hyper-IgD-syndrome. The leading symptom of this rare disease are periodic pyrexia, joint involvements (arthralgias/arthritis) and swollen lymph nodes. A permanent increase of alpha 1-acid glycoprotein fucosylation indicates persisting inflammation. Most important in differential diagnosis in familial Mediterranean fever. Therapy is merely supportive as yet, the long-term outlook seems good despite duration of the illness. CONCLUSION: the hyper-IgD-syndrome must be considered in cases of otherwise unexplained periodic fever.
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