Kidney involvement in systemic sclerosis: From pathogenesis to treatment

2018 ◽  
Vol 3 (1) ◽  
pp. 43-52 ◽  
Author(s):  
Cosimo Bruni ◽  
Giovanna Cuomo ◽  
Francesca W. Rossi ◽  
Emanuela Praino ◽  
Silvia Bellando-Randone
Keyword(s):  
Systemic Sclerosis ◽  
Angiotensin Converting Enzyme ◽  
Enzyme Inhibitors ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Prognostic Impact ◽  
Converting Enzyme ◽  
Converting Enzyme Inhibitors ◽  
Kidney Involvement ◽  
Renal Crisis

Among all possible systemic sclerosis internal organ complications, kidney involvement is frequently neglected or underestimated, except for the life-threatening scleroderma renal crisis. Fortunately, this severe clinical presentation is nowadays better controlled with available treatments, in particular angiotensin-converting enzyme inhibitors, and this has led to a reduction in its short- and longer-term mortality. Pathogenetic determinants are not well understood and many different other kidney involvements are possible in systemic sclerosis, including proteinuria, albuminuria, reduction of renal filtration, autoantibodies-related glomerulonephritis, and drug-related side effects. Different serological and radiological methods of evaluations are nowadays available, some representing promising diagnostic tool and prognostic outcome measure. Except for angiotensin-converting enzyme inhibitors in scleroderma renal crisis, no other treatment is currently recommended for treatment of kidney involvement in systemic sclerosis. For this reason, further studies are necessary to investigate its prognostic impact, in particular in combination with other systemic sclerosis–related internal organ manifestations. This review summarizes current available literature on kidney involvement in systemic sclerosis.

scholarly journals Renal Involvement in Systemic Sclerosis: An Update

2020 ◽  
Vol 45 (4) ◽  
pp. 532-548
Author(s):  
Magdalena Chrabaszcz ◽  
Jolanta Małyszko ◽  
Mariusz Sikora ◽  
Rosanna Alda-Malicka ◽  
Anna Stochmal ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Angiotensin Converting Enzyme ◽  
Enzyme Inhibitors ◽  
Renal Involvement ◽  
Significant Proportion ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme ◽  
Converting Enzyme Inhibitors ◽  
Renal Crisis

Background: Systemic sclerosis is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis and autoimmune phenomena. Summary: Renal disease occurring in patients with systemic sclerosis may have a variable clinicopathological picture. The most specific renal condition associated with systemic sclerosis is scleroderma renal crisis, characterized by acute onset of renal failure and severe hypertension. Although the management of scleroderma renal crisis was revolutionized by the introduction of angiotensin-converting enzyme inhibitors, there is still a significant proportion of patients with poor outcomes. Therefore, research on establishing disease markers (clinical, ultrasonographical and serological) and clear diagnostic criteria, which could limit the risk of developing scleroderma renal crisis and facilitate diagnosis of this complication, is ongoing. Other forms of renal involvement in systemic sclerosis include vasculitis, an isolated reduced glomerular filtration rate in systemic sclerosis, antiphospholipid-associated nephropathy, high intrarenal arterial stiffness and proteinuria. Key Messages: Scleroderma renal crisis is the most specific and life-threatening renal presentation of systemic sclerosis, albeit with declining prevalence. In patients with scleroderma renal crisis, it is mandatory to control blood pressure early with increasing doses of angiotensin-converting enzyme inhibitors, along with other antihypertensive drugs if necessary. There is a strong association between renal involvement and patients’ outcomes in systemic sclerosis; consequently, it becomes mandatory to find markers that may be used to identify patients with an especially high risk of scleroderma renal crisis.

scholarly journals Iatrogenic scleroderma renal crisis: A case report and mini literature review for renal crisis

2021 ◽  
Vol 8 (3) ◽  
pp. 4
Author(s):  
Gülşah Boz ◽  
Samet Karahan ◽  
Nurdan Yüksek
Keyword(s):  
Systemic Sclerosis ◽  
Enzyme Inhibitors ◽  
Day Treatment ◽  
Renal Involvement ◽  
Elevated Serum ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme Inhibitors ◽  
Kidney Involvement ◽  
Renal Crisis

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.

scholarly journals Favourable Outcome of Scleroderma Renal Crisis

1996 ◽  
Vol 89 (1) ◽  
pp. 49P-50P ◽  
Author(s):  
D A Collins ◽  
S Patel ◽  
J B Eastwood ◽  
B E Bourke
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Enzyme Inhibitors ◽  
Severe Hypertension ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Favourable Outcome ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme ◽  
Converting Enzyme Inhibitors ◽  
Renal Crisis

Severe hypertension and rapidly progressive acute renal failure is a well recognized complication of scleroderma, often referred to as the renal crisis, and widely thought to cause irreversible deterioration in renal function. With the advent of angiotensin-converting-enzyme inhibitors (ACE-I) the outlook for patients with this condition has dramatically improved. We report here one such patient.

Prognostic effect of angiotensin-converting-enzyme inhibitors in HCC patients treated with sorafenib.

2013 ◽  
Vol 31 (4_suppl) ◽  
pp. 328-328 ◽  
Author(s):  
Khaled Abouelezz ◽  
Ahmed Omar Kaseb ◽  
Hesham Mohamed Hassabo ◽  
Chris R. Garrett ◽  
Jean-Nicolas Vauthey ◽  
...  
Keyword(s):  
Angiotensin Converting Enzyme ◽  
Median Survival ◽  
Enzyme Inhibitors ◽  
Angiotensin Receptor Blockers ◽  
Experimental Studies ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Prognostic Impact ◽  
Converting Enzyme ◽  
Converting Enzyme Inhibitors ◽  
Significant Difference

328 Background: Experimental studies indicated that angiotensin-converting-enzyme inhibitors (ACEs) or angiotensin receptor blockers (ARBs) has a role in reducing malignant changes of the liver and may significantly improve the mortality in patients with other cancers. However it is not known whether ACE and ARB inhibitors may have prognostic impact on HCC survival outcome. We aimed at evaluating the role of ACE inhibitors and ARB blockers on the prognosis of HCC patients who have been treated by Nexavar (sorafenib). Methods: Using resources of our case-control study at MD Anderson, total of 187 (males, 76.5%; females, 23.5%) pathologically confirmed HCC had been treated by sorafenib. The overall mean age (±SD) was 62.5years ± 9.6. The majority of patients were Caucasians (62.6%) who were diagnosed at TNM stage III and IV (79.2%). Total of 89 (47.6%) patients had no evidence of hepatitis C virus (HCV) or hepatitis B virus (HBV). Median survival was calculated using Kaplan Meier product-limit method and survival rates were compared using the log rank test. Cox proportional hazards model was used to estimate the univariate and multivariate Hazard Risk Ratios (HRR) and 95% Confidence Interval (CI). Results: The overall median survival of HCC patients was 19.9 months (95% CI, 16.1-23.6). Intake of ACEs or ARBs inhibitors was reported in 94 patients (50.3%). We observed significant difference in overall median survival (months) between patients who had ACE/ARB inhibitors versus those who did not, median survival (95% CI) were 21.5 (17.1-25.8) and 14.6 (12.4-16.7), respectively, p=0.04. After adjustment for HCC prognostic factors and demographic factors, we observed 30% reduction in HCC mortality among those who received ACEs/ARBs inhibitors, the estimated HR was 0.7 (95% CI, 0.4-1), p=0.05. Conclusions: Our results may indicate that HCC patients under anti-angiogenic therapy (sorafenib) and have been under anti-hypertensive therapy like ACE/ARB may assist in depleting the collagen and may improve host matrix and tumor microenvironment to enhance drug delivery. Future analysis in a larger sample is warranted

Mortality and morbidity in scleroderma renal crisis: A systematic literature review

2020 ◽  
pp. 239719832092042
Author(s):  
Hyein Kim ◽  
Frédéric Lefebvre ◽  
Sabrina Hoa ◽  
Marie Hudson
Keyword(s):  
Systemic Sclerosis ◽  
Angiotensin Converting Enzyme ◽  
Angiotensin Converting Enzyme Inhibitor ◽  
Enzyme Inhibitor ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme ◽  
Converting Enzyme Inhibitors ◽  
Mortality And Morbidity ◽  
Converting Enzyme Inhibitor ◽  
Renal Crisis

Objectives: The objective of this study was to systematically review the mortality and morbidity associated with scleroderma renal crisis and to determine temporal trends. Methods: We searched MEDLINE, Embase and the Cochrane Database of Systematic Reviews from database inception to 10 February 2020. Bibliographies of selected articles were hand-searched for additional references. Data were extracted using a standardized extraction form. Study quality was assessed using the Newcastle–Ottawa scale. Results were analysed qualitatively. Results: Twenty studies with 14,059 systemic sclerosis subjects, of which 854 had scleroderma renal crisis and 4095 had systemic sclerosis–associated end-stage renal disease, met inclusion criteria. Study quality was generally moderate. Cumulative mortality in the post-angiotensin-converting enzyme inhibitor era was approximately 20% at 6 months, 30%–36% at 1 year, 19%–40% at 3 years and almost 50% at 10 years from scleroderma renal crisis onset. Although the introduction of angiotensin-converting enzyme inhibitors in the early 1970s resulted in a 50% improvement in scleroderma renal crisis mortality, there was no further improvement thereafter. Scleroderma renal crisis mortality rates were proportionally higher than mortality rates associated with other systemic sclerosis organ involvement. The rate of permanent dialysis after scleroderma renal crisis in the post-angiotensin-converting enzyme inhibitor era ranged from 19%–40%. Three to 17% of systemic sclerosis patients underwent renal transplant. Survival was better in patients post-renal transplant (54%–91%) compared to those on dialysis (31%–56%). Graft survival improved over time and appeared similar to that of patients with other types of end-stage renal disease. Conclusion: While there has been considerable improvement in scleroderma renal crisis–related outcomes since the introduction of angiotensin-converting enzyme inhibitors, morbidity and mortality remain high for affected patients without convincing evidence of further improvement in the post-angiotensin-converting enzyme inhibitor era. Novel treatments are required to improve outcomes of scleroderma renal crisis.

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