Al-Harthy Fayez, Esdaile John, Berean Kenneth W, Chalmers Andrew
Division of Rheumatology and the Department of Pathology, University of British Columbia, Vancouver, Canada.
J Rheumatol. 2006 Feb;33(2):358-61.
We describe 2 cases of multifocal idiopathic fibrosclerosis treated successfully with cyclosporine. The first patient presented with chronic abdominal pain and was subsequently found to have retroperitoneal fibrosis with ureteral obstruction. Other findings included cholangiolar fibrosis, retroorbital pseudotumors, submandibular gland enlargement, subcutaneous fibrotic masses, and elevated erythrocyte sedimentation rate (ESR) and serum creatinine. He initially responded well to temporary ureteral stenting followed by combination therapy with steroids and cyclosporine. He relapsed when cyclosporine was stopped, but subsequently remitted completely when cyclosporine was reintroduced. The second patient presented with long-standing abdominal pain with retroperitoneal fibrosis, submandibular gland enlargement, and an enlarged pancreas with a localized mass, all of which improved significantly with 6 months of therapy with cyclosporine. Cyclosporine was well tolerated with no significant side effects in the 2 patients. A review of the literature is presented.
我们描述了2例采用环孢素成功治疗的多灶性特发性纤维硬化症病例。首例患者表现为慢性腹痛，随后被发现患有伴有输尿管梗阻的腹膜后纤维化。其他发现包括胆小管纤维化、眶后假瘤、颌下腺肿大、皮下纤维性肿块以及红细胞沉降率（ESR）和血清肌酐升高。他最初对临时输尿管支架置入术反应良好，随后接受了类固醇和环孢素联合治疗。停用环孢素后病情复发，但再次使用环孢素后病情完全缓解。第二例患者长期存在腹痛，伴有腹膜后纤维化、颌下腺肿大以及胰腺肿大并伴有局部肿块，接受6个月的环孢素治疗后所有症状均有显著改善。2例患者对环孢素耐受性良好，未出现明显副作用。本文还对相关文献进行了综述。
