Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more f...Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more frequent and mainly characterized by moderate proteinuria,elevated serum creatinine levels,and hypertension.The most common kidney involvement in SSc is scleroderma renal crisis(SRC)that is fatal without prompt intervention.Case report:A 52-year-old Caucasian male with known diffuse cutaneous systemic sclerosis was hospitalized with communityacquired pneumonia.On the fifth day after appropriate antibiotic therapy and 60 mg/day methylprednisolone,decreased urine output,arterial hypertension,decreased renal function and pulmonary edema developed.The patient was diagnosed with a scleroderma renal crisis.Emergency hemodialysis was applied to the patient,and captopril 6×25 mg/day and nifedipine 2*60 mg/day treatment were given.He received a routine hemodialysis program for about three months.The hemodialysis program was terminated when the patient’s urine quality and quantity increased.Conclusions:SRC,characterized by malignant hypertension,azotemia,microangiopathic hemolytic anemia,and kidney failure,is one of the most important complications of systemic sclerosis with a poor prognosis without prompt intervention.Steroid use is one of the important risk factors that precipitate SRC development.With angiotensin-converting enzyme inhibitors,survival increased after SRC,the need for dialysis decreased,and usually allowed the discontinuation of dialysis treatment within about 6-18 months.Suspicion of SRC in the presence of the above-mentioned findings in patients with a diagnosis or suspected systemic sclerosis can be considered the most important treatment step.展开更多
The increased awareness of systemic sclerosis(SS)and its pathogenetic background made the management of this disease more amenable than previously thought.However,scleroderma renal crisis(SRC)is a rarely seen as an as...The increased awareness of systemic sclerosis(SS)and its pathogenetic background made the management of this disease more amenable than previously thought.However,scleroderma renal crisis(SRC)is a rarely seen as an associated disorder that may involve 2%-15%of SS patients.Patients presented with earlier,rapidly progressing,diffuse cutaneous SS disease,mostly in the first 3-5 years after non-Raynaud clinical manifestations,are more vulnerable to develop SRC.SRC comprises a collection of acute,mostly symptomatic rise in blood pressure,elevation in serum creatinine concentrations,oliguria and thrombotic microangiopathy in almost 50%of cases.The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis.In a scleroderma patient maintained on regular dialysis;every effort should be exerted to declare any possible evidence of renal recovery.A given period of almost two years has been suggested prior to proceeding in a kidney transplant(KTx).Of note,SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease(ESRD).KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients.Compared to other primary renal diseases,SS-related ESRD was considered for a long period of poor patient and allograft survivals.Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death.Recurrence of SRC after transplantation has been observed in some patients.However,an excellent post-transplant patient and graft outcome have been recently reported.Consequently,the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx.展开更多
文摘Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more frequent and mainly characterized by moderate proteinuria,elevated serum creatinine levels,and hypertension.The most common kidney involvement in SSc is scleroderma renal crisis(SRC)that is fatal without prompt intervention.Case report:A 52-year-old Caucasian male with known diffuse cutaneous systemic sclerosis was hospitalized with communityacquired pneumonia.On the fifth day after appropriate antibiotic therapy and 60 mg/day methylprednisolone,decreased urine output,arterial hypertension,decreased renal function and pulmonary edema developed.The patient was diagnosed with a scleroderma renal crisis.Emergency hemodialysis was applied to the patient,and captopril 6×25 mg/day and nifedipine 2*60 mg/day treatment were given.He received a routine hemodialysis program for about three months.The hemodialysis program was terminated when the patient’s urine quality and quantity increased.Conclusions:SRC,characterized by malignant hypertension,azotemia,microangiopathic hemolytic anemia,and kidney failure,is one of the most important complications of systemic sclerosis with a poor prognosis without prompt intervention.Steroid use is one of the important risk factors that precipitate SRC development.With angiotensin-converting enzyme inhibitors,survival increased after SRC,the need for dialysis decreased,and usually allowed the discontinuation of dialysis treatment within about 6-18 months.Suspicion of SRC in the presence of the above-mentioned findings in patients with a diagnosis or suspected systemic sclerosis can be considered the most important treatment step.
文摘The increased awareness of systemic sclerosis(SS)and its pathogenetic background made the management of this disease more amenable than previously thought.However,scleroderma renal crisis(SRC)is a rarely seen as an associated disorder that may involve 2%-15%of SS patients.Patients presented with earlier,rapidly progressing,diffuse cutaneous SS disease,mostly in the first 3-5 years after non-Raynaud clinical manifestations,are more vulnerable to develop SRC.SRC comprises a collection of acute,mostly symptomatic rise in blood pressure,elevation in serum creatinine concentrations,oliguria and thrombotic microangiopathy in almost 50%of cases.The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis.In a scleroderma patient maintained on regular dialysis;every effort should be exerted to declare any possible evidence of renal recovery.A given period of almost two years has been suggested prior to proceeding in a kidney transplant(KTx).Of note,SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease(ESRD).KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients.Compared to other primary renal diseases,SS-related ESRD was considered for a long period of poor patient and allograft survivals.Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death.Recurrence of SRC after transplantation has been observed in some patients.However,an excellent post-transplant patient and graft outcome have been recently reported.Consequently,the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx.
