Treatment Of Schnitzler's Syndrome With Colchicine: A Case Report

Robert Y. Lin; Sundar Jagannath

摘要

Schnitzler's syndrome was described in 1974(1) and is typically presents with chronic nonpruritic urticaria, bone pains, fever, and monoclonal IgM gammopathy(2). Although corticosteroids are usually used to treat these patients, long term treatment usually results in significant corticosteroid induced side effects. Other treatments that have been described include cyclosporine, thalidomide, and interferon alpha. The benefits of colchicines in this disorder have been stated by some experts to be minimal. In this report, we describe the clinical course of a patient with Schnitzler's syndrome who was treated with colchicine with marked improvement. Case Report A 57 year old White female presented to the hematology department of St Vincents Hospital in December 1999 with complaints of recurrent fever and rash since 1993. She had been diagnosed as having an IgM monoclonal gammopathy at another medical facility. The patient claimed to have been treated with corticosteroids and hydroxyzine without relief. A bone marrow biopsy examination at that time showed 11% plasma cells. Serum immunfixation showed an IgM kappa band. Serum IgG was 490 mg/dL, IgA was 90 mg/dL, and IgM was 710 mg/dL. The hemoglobin was 10 g/dL. The white blood count was 16,600/mL. The serum protein was 7.7 g/dL. A left upper arm lesional skin biopsy was interpreted as being consistent with leukocytoclastic vasculitis. Linear deposition of IgM kappa chain was observed in the vicinity of the inflamed blood vessels. The patient underwent plasmapheresis, which resulted in a decreased serum IgM of 477 mg/dL Rofecoxib 25 mg daily and prednisone 60 every other day was administered. In February 2000, the rash persisted and the serum IgM rose again to 805 mg/dL Rofecoxib was increased to 50 mg/d and the patient underwent plasmapheresis on a monthly basis through May 2000, at which time pentoxifylline 800 mg/d was administered. In July 2000 the patient had a serum IgM of 671 mg/dL, a C-reactive protein level of 10.3 mg/dL, and an IL6 level of 10 pgm/mL Prednisone was continued at 60 mg every other day and cimetidine 400 mg twice daily and loratadine 10 mg/d were prescribed. celecoxib 100 mg twice daily was prescribed in place of rofecoxib. In September 2000, the patient was given etanercept 25 mg twice weekly for 10 weeks. In November 2000 the IgM rose to 1500 mg/dL. The patient continued to have rash but no fever. Rituximab 460 mg was administered intravenously on a weekly basis for 4 doses. By the middle of January 2001,.the patient reported improvement and the IgM decreased to 404 mg/dL. Prednisone was discontinued. At the end of January 2001, the fever recurred and the temperature was 38.9C (102F) and rash was noted. At that time the IgM was 240 mg/dL Prednisone was restarted at 30 mg every other day. On 4/16/01 the C-reactive protein was 9.3 mg/dL.In December 2001 the patient still reported rashes and fever that would usually recur together. The IgM was 113 mg/dL, IgA 24 mg/dL, IgG 419 mg/dL. The bone marrow biopsy examination showed 5% plasma cells. The patient was referred for allergy/immunology consultation in March 2002. The patient reported that she had frequent “hives” that were followed with 1-2 2 hours by sheet soaking fever with chills and severe malaise and fatigue. This sequence of symptoms occurred every 2-3 days. The patient claimed that the rash was not very pruritic and individual lesions would sometimes last for up to 2 days. The patient reported having been treated in the past with colchicine at another medical facility without improvement of symptoms. Laboratory findings showed an ESR of 65 mm/hr, CRP of 6.9 mg/dL, a CH50 of 121 CAE units, white blood count of 7.8/mm3 and a C4 of 22.7 mg/dL. No cryoglobulins were detected in the serum. At that time she was taking prednisone 30 mg every other day, but still frequent outbreaks of rash and fever. Dapsone 100 mg per day was prescribed and the patient was instructed to reduce her prednisone dose to 25 mg eve

机译：1974年对Schnitzler综合征进行了描述（1），通常表现为慢性非瘙痒性荨麻疹，骨痛，发烧和单克隆IgM丙种病（2）。尽管通常使用糖皮质激素治疗这些患者，但是长期治疗通常会导致明显的糖皮质激素诱发的副作用。已经描述的其他治疗方法包括环孢素，沙利度胺和干扰素α。一些专家认为秋水仙碱在这种疾病中的益处微乎其微。在本报告中，我们描述了接受秋水仙碱治疗并有明显改善的Schnitzler综合征患者的临床病程。病例报告一名57岁的白人女性于1999年12月被送往圣文森特医院血液科，自1993年以来因发烧和皮疹反复发作而被投诉。她在另一家医疗机构被诊断出患有IgM单克隆性丙种球蛋白病。该患者声称已接受皮质类固醇和羟嗪治疗，但无缓解。当时的骨髓活检显示11％的浆细胞。血清免疫固定显示IgMκ带。血清IgG为490 mg / dL，IgA为90 mg / dL，IgM为710 mg / dL。血红蛋白为10g / dL。白血球计数为16,600 / mL。血清蛋白为7.7g / dL。左上臂病变皮肤活检被解释为与白细胞碎裂性血管炎一致。在发炎的血管附近观察到IgMκ链的线性沉积。患者接受血浆置换，导致血清IgM降低至477 mg / dL罗非昔布每天25 mg，每两天一次泼尼松60。 2000年2月，皮疹持续存在，血清IgM再次上升至805 mg / dL，罗非考昔（Rofecoxib）上升至50 mg / d，该患者每月进行血浆置换，直至2000年5月，此时给予己酮可可碱800 mg / d。 2000年7月，患者的血清IgM为671 mg / dL，C反应蛋白水平为10.3 mg / dL，IL6水平为10 pgm / mL，泼尼松每隔一天持续60 mg，西咪替丁400 mg两次每天服用，氯雷他定10 mg / d。每天两次用塞来昔布100 mg代替罗非昔布开处方。在2000年9月，患者每周两次接受etanercept 25 mg的治疗，持续10周。 2000年11月，IgM升至1500 mg / dL。病人继续有皮疹但没有发烧。每周静脉注射利妥昔单抗460 mg，共4剂。到2001年1月中旬，患者报告病情好转，IgM降至404 mg / dL。泼尼松停药。在2001年1月底，发烧再次出现，温度为38.9摄氏度（102华氏度），并出现皮疹。当时，IgM为240 mg / dL泼尼松每隔一天以30 mg重新开始。在4/16/01时C反应蛋白为9.3 mg / dL.2001年12月，患者仍报告皮疹和发烧，通常会同时复发。 IgM为113 mg / dL，IgA 24 mg / dL，IgG 419 mg / dL。骨髓活检显示有5％的浆细胞。该患者于2002年3月被转介接受变态反应/免疫学咨询。该患者报告说，她经常有“荨麻疹”，随后是1-2个2小时的床单浸泡发烧，发冷，严重不适和疲劳。此症状序列每2-3天出现一次。该患者声称皮疹不是非常瘙痒，个别病变有时可持续长达2天。该患者报告说过去曾在另一家医疗机构接受过秋水仙碱的治疗，但症状并未改善。实验室检查结果显示，ESR为65 mm / hr，CRP为6.9 mg / dL，CH50为121 CAE单位，白细胞计数为7.8 / mm3，C4为22.7 mg / dL。在血清中未检测到冷球蛋白。当时她每两天服用一次泼尼松30毫克，但仍经常出疹子和发烧。每天服用氨苯砜100毫克，并指导患者将泼尼松的剂量减至25毫克前夕

Treatment Of Schnitzler's Syndrome With Colchicine: A Case Report

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