Berkun Yackov, Padeh Shai, Reichman Brian, Zaks Nurit, Rabinovich Einat, Lidar Merav, Shainberg Bracha, Livneh Avi
Department of Pediatrics, Safra Children's Hospital, Sheba Medical Center, Tel-Hashomer, Israel.
Semin Arthritis Rheum. 2007 Dec;37(3):182-8. doi: 10.1016/j.semarthrit.2007.03.005. Epub 2007 May 23.
In a significant proportion of patients with familial Mediterranean fever (FMF), serum amyloid A (SAA) remains elevated during attack-free periods, thereby increasing the risk of developing amyloidosis. The aim of the study was to determine various correlates of elevated SAA and evaluate the role of SAA measurement in the diagnosis and management of FMF.
We reviewed the medical files of all 204 patients from our FMF center in whom SAA measurements were performed. SAA levels and the resulting diagnostic and therapeutic decisions were analyzed in relation to the reasons of SAA testing and to several clinical and genetic parameters.
SAA measurements were made for diagnostic purposes in 29% of the patients. In the remainder, SAA measurements were used for adjustment of colchicine dose. Elevated SAA levels are found in a third of FMF patients during an attack-free period. The highest rate of elevated SAA levels was found in patients with proteinuria (60% of this patient group), followed by noncompliant (40%) and genetically positive asymptomatic patients (38%). Elevated SAA levels during remission were associated with family history of FMF, M694V homozygosity, and elevated C-reactive protein (CRP) (P<0.05 for each). Patients homozygous for the M694V mutation had the highest level of SAA. SAA measurement led to a change in colchicine dose in 30% of the patients, predominantly in noncompliant patients and patients with proteinuria or with atypical manifestations.
Measurement of SAA level may help in the diagnosis of FMF and in adjustment of the colchicine dose.
在相当一部分家族性地中海热（FMF）患者中，血清淀粉样蛋白A（SAA）在无发作期仍持续升高，从而增加了发生淀粉样变性的风险。本研究旨在确定SAA升高的各种相关因素，并评估SAA检测在FMF诊断和管理中的作用。
我们回顾了我们FMF中心所有进行过SAA检测的204例患者的病历。分析了SAA水平以及由此产生的诊断和治疗决策与SAA检测原因以及几个临床和遗传参数之间的关系。
29%的患者进行SAA检测是为了诊断。其余患者中，SAA检测用于调整秋水仙碱剂量。三分之一的FMF患者在无发作期SAA水平升高。蛋白尿患者中SAA水平升高的比例最高（该患者组的60%），其次是不依从患者（40%）和基因检测呈阳性的无症状患者（38%）。缓解期SAA水平升高与FMF家族史、M694V纯合子以及C反应蛋白（CRP）升高有关（每项P<0.05）。M694V突变纯合子患者的SAA水平最高。30%的患者因SAA检测导致秋水仙碱剂量改变，主要是不依从患者以及有蛋白尿或非典型表现的患者。
SAA水平检测可能有助于FMF的诊断以及秋水仙碱剂量的调整。
