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首页> 外文期刊>Allergy >Schnitzler's syndrome: Diagnosis, treatment, and follow-up
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Schnitzler's syndrome: Diagnosis, treatment, and follow-up

机译：Schnitzler综合征：诊断，治疗和随访

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摘要

Schnitzler's syndrome is characterized by recurrent urticarial rash and monoclonal gammopathy, associated with clinical and biological signs of inflammation and a long-term risk of AA amyloidosis and overt lymphoproliferation. An extensive literature review was performed, and the following questions were addressed during an expert meeting: In whom should Schnitzler's syndrome be suspected? How should the diagnosis of Schnitzler's syndrome be established? How should a patient with Schnitzler's syndrome be treated? How should a patient with Schnitzler's syndrome be followed up?. A diagnosis of Schnitzler's syndrome is considered definite in any patient with two obligate criteria: a recurrent urticarial rash and a monoclonal IgM gammopathy, and two of the following minor criteria: recurrent fever, objective signs of abnormal bone remodeling, elevated CRP level or leukocytosis, and a neutrophilic infiltrate on skin biopsy. It is considered probable, if only 1 minor criterion is present. In patients with monoclonal IgG gammopathies, diagnosis is definite if three minor criteria are present and possible if two are present. First-line treatment in patients with significant alteration of quality of life or persistent elevation of markers of inflammation should be anakinra. Follow-up should include clinical evaluation, CBC and CRP every 3 months and MGUS as usually recommended.
机译：Schnitzler综合征的特征是反复发作的荨麻疹和单克隆丙种球蛋白病，与炎症的临床和生物学征兆以及AA淀粉样变性病和明显的淋巴细胞增殖的长期风险有关。进行了广泛的文献综述，在一次专家会议上提出了以下问题：应该怀疑谁患有Schnitzler综合征？怎样确定Schnitzler综合征的诊断？患有Schnitzler综合征的患者应如何治疗？患有Schnitzler综合征的患者应如何随访？任何有以下两个严格标准的患者均被诊断为Schnitzler综合征的诊断：复发性荨麻疹和IgM单克隆丙种球蛋白病，以及以下两个次要标准：复发性发烧，骨骼重构异常的客观体征，CRP水平升高或白细胞增多，并且嗜中性粒细胞浸润皮肤活检。如果仅存在一个次要条件，则认为是可能的。在患有单克隆IgG同性恋症的患者中，如果存在三个次要标准，则诊断是确定的，如果存在两个次要标准，则诊断是可能的。生活质量明显改变或炎症标志物持续升高的患者，应采取一线治疗。随访应包括临床评估，每3个月进行CBC和CRP以及通常推荐的MGUS。

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