硬皮病肾危象5例并文献复习
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摘要
目的分析硬皮病肾危象的临床特点,探讨早期诊治的重要性。方法搜集收治的5例硬皮病肾危象(SRC)患者的临床表现、实验室指标,治疗转归,总结疾病的特点并文献复习。结果本组例1患者,通过很好的控制血压及长期血液透析治疗,病情稳定。例2患者,开始诊断为"皮肌炎",后病情转化并恶化为硬皮病肾危象,经抢救无效而死亡。例3患者,血压控制不佳,发展至尿毒症期且合并肺间质病变,经抢救无效而死亡。例4患者及时发现高血压及肾功能不全,积极使用血管紧张素转化酶抑制剂(ACEI),肾功能恢复良好。例5患者合并多器官损害,入院后抢救无效而死亡。结论硬皮病必须关注尿常规、血压及肾功能,一旦出现肾危象,需及时使用ACEI,合并多脏器损害者,预后差。结缔组织病容易转化及误诊,进一步检查如皮肤活检及肾活检等是必要的。
Objective To analyze the clinical characteristics of scleroderma renal crisis,and discuss the importance of early diagnosis and therapy.Methods The clinical manifestations of five patients with scleroderma renal crisis were collected,laboratory indexes,treatments and outcomes,disease characteristics and review literature were analyzed.Results Case one was in stable condition under good control of blood pressure and continuous hemodialysis treatment.Case two firstly was diagnosed as"dermatomyositis",which transformed or worsened to scleroderma renal crisis and the patient dead after emergency treatment.Case three accompanied with hypertension which was not well controlled,the disease developed to uremic syndrome in combination with interstitial lung disease,the patient dead after emergency treatment.Case four was promptly found to be with hypertension and renal insufficiency,after being administered actively with angiotensin converting enzyme inhibitor(ACEI),the renal function was well recovered.Case five was accompanied with multiple organs impairment and dead after emergency treatment.Conclusion As for sclerodermas,attention must be paid to urine routines,blood pressure changes and renal function.Once renal crisis appeared,ACEI should be administered promptly.Scleroderma accompanied with more injured viscera had unfavorable prognosis.Connective tissue disease might be transformed and misdiagnosed easily,it was necessary to perform further inspections such as skin biopsy and renal biopsy.
Objective To analyze the clinical characteristics of scleroderma renal crisis,and discuss the importance of early diagnosis and therapy.Methods The clinical manifestations of five patients with scleroderma renal crisis were collected,laboratory indexes,treatments and outcomes,disease characteristics and review literature were analyzed.Results Case one was in stable condition under good control of blood pressure and continuous hemodialysis treatment.Case two firstly was diagnosed as"dermatomyositis",which transformed or worsened to scleroderma renal crisis and the patient dead after emergency treatment.Case three accompanied with hypertension which was not well controlled,the disease developed to uremic syndrome in combination with interstitial lung disease,the patient dead after emergency treatment.Case four was promptly found to be with hypertension and renal insufficiency,after being administered actively with angiotensin converting enzyme inhibitor(ACEI),the renal function was well recovered.Case five was accompanied with multiple organs impairment and dead after emergency treatment.Conclusion As for sclerodermas,attention must be paid to urine routines,blood pressure changes and renal function.Once renal crisis appeared,ACEI should be administered promptly.Scleroderma accompanied with more injured viscera had unfavorable prognosis.Connective tissue disease might be transformed and misdiagnosed easily,it was necessary to perform further inspections such as skin biopsy and renal biopsy.
引文
[1]姜廷新,李冰.硬皮病肾危象的临床研究[J].中国误诊学杂志,2012,12(6):1280-1281.
[2]Akoglu H,Atilgan GK,Ozturk R,et al.A“silent”course of normotensive scleroderma renal crisis:case report and review of the literature[J].Rheumatol Int,2009,29(12):1223-1229.
[3]Bussone G,Berezne A,Pestre V,et al.The scleroderma kidney:the scleroderma kidney:progress in risk factors,therapy,and prevention[J].Curr Rheumatol Rep,2011,13(1):37-43.
[4]葛均波,徐永健.内科学[M].8版.北京:人民卫生出版社,2014:847.
[5]Bose N,Chiesa-Vottero A,Chatteriee S.Scleroderma renal crisis[J].Semin Arthritis Rheum,2015,44(6):687-694.
[6]Mouthon L,Bussone G,Berezne A,et al.Scleroderma renal crisis[J].J Rheumatol,2014,41(6):1040-1048.
[7]Steen VD.Kidney involvement in systemic sclerosis[J].Presse Med,2014,43(10Pt 2):305-314.
[8]Redd D,Frech TM,Murtaugh MA,et al.Informatics can identify systemic sclerosis(SSc)patients at risk for scleroderma renal crisis[J].Comput Biol Med,2014,53(10):203-205.
[9]占永立,王丽,饶向荣,等.系统性硬化肾损伤误诊为狼疮性肾炎1例[J].中国中西医结合肾病杂志,2008,9(11):1006-1007.
[10]阎登富,王彪.系统性硬化病误诊1例[J].临床荟萃,2009,24(4):344.
[11]王建雄,李小生,赖燕蔚.系统性硬化病合并显微镜下多血管炎1例[J].临床荟萃,2007,22(22):封二.
[12]Cozzi F,Marson P,Cardarelli S,et al.Prognosis of scleroderma renal crisis:a long-term observational study[J].Nephrol Dial Transplant,2012,27(12):4398-4403.
[13]Hudson M,Baron M,Tatibouet S,et al.Exposure to ACE inhibitors prior to the onset of sclerodermarenal crisis-results from the international scleroderma renal crisis survey[J].Semin Arthritis Rheum,2014,43(5):666-672.
[14]Montanelli G,Beretta L,Santaniello A,et al.Effect of dihydropyridine calcium channel blockers and glucocorticoids on the prevention and development of scleroderma renal crisis in an Italian case series[J].Clin Exp Rheumatol,2013,31(2Suppl 76):135-139.
[15]Perez Campos D,Estevez Del Toro M,Pena Casanovas A.,et al.Are high doses of prednisone necessary for treatment of interstitial lung disease in systemic sclerosis[J].Reumatol Clin,2012,8(2):58-62.
[16]Dhaun N,MacIntyre IM,Bellamy CO,et al.Endothelin receptor antagonism and renin inhibition as treatment options for scleroderma kidney[J].Am J Kidney Dis,2009,54(4):726-731.
[2]Akoglu H,Atilgan GK,Ozturk R,et al.A“silent”course of normotensive scleroderma renal crisis:case report and review of the literature[J].Rheumatol Int,2009,29(12):1223-1229.
[3]Bussone G,Berezne A,Pestre V,et al.The scleroderma kidney:the scleroderma kidney:progress in risk factors,therapy,and prevention[J].Curr Rheumatol Rep,2011,13(1):37-43.
[4]葛均波,徐永健.内科学[M].8版.北京:人民卫生出版社,2014:847.
[5]Bose N,Chiesa-Vottero A,Chatteriee S.Scleroderma renal crisis[J].Semin Arthritis Rheum,2015,44(6):687-694.
[6]Mouthon L,Bussone G,Berezne A,et al.Scleroderma renal crisis[J].J Rheumatol,2014,41(6):1040-1048.
[7]Steen VD.Kidney involvement in systemic sclerosis[J].Presse Med,2014,43(10Pt 2):305-314.
[8]Redd D,Frech TM,Murtaugh MA,et al.Informatics can identify systemic sclerosis(SSc)patients at risk for scleroderma renal crisis[J].Comput Biol Med,2014,53(10):203-205.
[9]占永立,王丽,饶向荣,等.系统性硬化肾损伤误诊为狼疮性肾炎1例[J].中国中西医结合肾病杂志,2008,9(11):1006-1007.
[10]阎登富,王彪.系统性硬化病误诊1例[J].临床荟萃,2009,24(4):344.
[11]王建雄,李小生,赖燕蔚.系统性硬化病合并显微镜下多血管炎1例[J].临床荟萃,2007,22(22):封二.
[12]Cozzi F,Marson P,Cardarelli S,et al.Prognosis of scleroderma renal crisis:a long-term observational study[J].Nephrol Dial Transplant,2012,27(12):4398-4403.
[13]Hudson M,Baron M,Tatibouet S,et al.Exposure to ACE inhibitors prior to the onset of sclerodermarenal crisis-results from the international scleroderma renal crisis survey[J].Semin Arthritis Rheum,2014,43(5):666-672.
[14]Montanelli G,Beretta L,Santaniello A,et al.Effect of dihydropyridine calcium channel blockers and glucocorticoids on the prevention and development of scleroderma renal crisis in an Italian case series[J].Clin Exp Rheumatol,2013,31(2Suppl 76):135-139.
[15]Perez Campos D,Estevez Del Toro M,Pena Casanovas A.,et al.Are high doses of prednisone necessary for treatment of interstitial lung disease in systemic sclerosis[J].Reumatol Clin,2012,8(2):58-62.
[16]Dhaun N,MacIntyre IM,Bellamy CO,et al.Endothelin receptor antagonism and renin inhibition as treatment options for scleroderma kidney[J].Am J Kidney Dis,2009,54(4):726-731.