Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication

2019 ◽  
Vol 47 (2) ◽  
pp. 241-248 ◽  
Author(s):  
Pia Moinzadeh ◽  
Kathrin Kuhr ◽  
Elise Siegert ◽  
Norbert Blank ◽  
Cord Sunderkoetter ◽  
...  
Keyword(s):  
Risk Factors ◽  
Enzyme Inhibitors ◽  
Rare Complication ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme Inhibitors ◽  
Life Threatening ◽  
History Of ◽  
Mean Time ◽  
Renal Crisis

Objective.Scleroderma renal crisis (SRC) is a severe life-threatening manifestation in patients with systemic sclerosis (SSc). However, the knowledge about risk factors for SRC is limited. We determined here the frequency of SRC and identified risk factors for the prediction of SRC.Methods.Based on regular followup data from the German Network for Systemic Scleroderma, we used univariate and multivariate generalized estimating equations to analyze the association between clinical variables, SSc subsets, therapy [i.e., angiotensin-converting enzyme inhibitors (ACEi), corticosteroids], and the occurrence of SRC.Results.Data of 2873 patients with 10,425 visits were available for analysis with a mean number of registry visits of 3.6 ± 2.8 and a mean time of followup of 3.6 ± 3.8 years. In total, 70 patients developed SRC (70/2873, 2.4%). Of these patients, 57.1% (40/70) were diagnosed with diffuse cutaneous SSc, 31.4% (22/70) with limited cutaneous SSc, and 11.4% (8/70) with SSc-overlap syndromes. Predictive independent factors with the highest probability for SRC were positive anti-RNA polymerase antibodies (RNAP), a history of proteinuria prior to SRC onset, diminished DLCO, and a history of hypertension. Interestingly, positive antitopoisomerase autoantibodies did not predict a higher risk for SRC. Further, patients with SRC were significantly more frequently treated with ACEi and corticosteroids without being independently associated with SRC.Conclusion.In this cohort, SRC has become a rare complication. By far the highest risk for SRC was associated with the detection of anti-RNAP and proteinuria.

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.

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.

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 Scleroderma nephropathy: unsolved problems

2021 ◽  
Vol 7 (5) ◽  
pp. 5-18
Author(s):  
I. E. Bulavko ◽  
E. V. Timofeev ◽  
K. J. U. Alkak ◽  
V. A. Isakov
Keyword(s):  
Risk Factors ◽  
Blood Pressure ◽  
Systemic Sclerosis ◽  
Rna Polymerase Iii ◽  
Kidney Injury ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme Inhibitors ◽  
Treatment Approaches ◽  
Renal Crisis

Kidney injury is a common pathology in the group of patients with systemic sclerosis. At least half of the patients show histological signs of it. Acute condition is known as scleroderma renal crisis. Although discussions regarding the risk factors for scleroderma renal crisis are open, most researchers consider the following factors: female sex, previous proteinuria and hypertension, the presence of anti-RNA polymerase III antibodies, and a decrease in lung diffusion capacity ≤75%. Diagnostic criteria for scleroderma renal crisis include an acute increase in blood pressure, accompanied by acute renal failure and abnormalities in the urinary sediment, anemia, and thrombocytopenia. Treatment of scleroderma renal crisis entails decreasing blood pressure, mainly with short-acting angiotensin-converting enzyme inhibitors, followed by selecting effective antihypertensive therapy. Further research of new treatment approaches is being carried on: the use of endothelin receptor antagonists (bosentan), monoclonal antibodies against the complement component 5 (eculizumab). Despite the approved strategies for identifying risk factors for scleroderma renal crisis development and treatment approaches, this group of patients is still characterized by high rates of mortality, the need for renal replacement therapy, and kidney transplantation. Thus, the problem of kidney injury in systemic sclerosis remains relevant.

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 Scleroderma Renal Crisis in a Case of Mixed Connective Tissue Disease Treated Successfully with Angiotensin-Converting Enzyme Inhibitors

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Jomana Madieh ◽  
Iman Khamayseh ◽  
Alaa Hrizat ◽  
Abdurrahman Hamadah ◽  
Kamel Gharaibeh
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Ace Inhibitors ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Enzyme Inhibitors ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme Inhibitors ◽  
Renal Crisis

Mixed connective tissue disease (MCTD) is a rheumatic disease syndrome with overlapping features of scleroderma, systemic lupus erythematosus, and polymyositis. An extremely rare but serious complication that can occur in MCTD is scleroderma renal crisis (SRC). There have been different approaches to the treatment of SRC associated with MCTD. We present a case of MCTD with chronic features of Raynaud’s phenomenon, dermatomyositis, and thrombocytopenia complicated with acute SRC which showed a great response to ACE inhibitors. Here, we advise the early and aggressive use of ACE inhibitors as soon as SRC is suspected.

scholarly journals The vicious cycle: a history of obesity and COVID-19

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jacek Bil ◽  
Olga Możeńska
Keyword(s):  
Enzyme Inhibitors ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Obesity Paradox ◽  
Lifestyle Changes ◽  
Angiotensin Ii Receptor ◽  
Converting Enzyme Inhibitors ◽  
History Of ◽  
Receptor Blockers ◽  
History Of Obesity

AbstractRecently, we face a surge in the fast-forward Coronavirus Disease 2019 (COVID-19) pandemic with nearly 170 million confirmed cases and almost 3.5 million confirmed deaths at the end of May 2021. Obesity, also known as the pandemic of the 21st century, has been evolving as an adverse prognostic marker. Obesity is associated with a higher risk of being SARS-CoV-2-positive (46%), as well as hospitalization (113%) and death (48%) due to COVID-19. It is especially true for subjects with morbid obesity. Also, observational studies suggest that in the case of COVID-19, no favorable “obesity paradox” is observed. Therefore, it is postulated to introduce a new entity, i.e., coronavirus disease-related cardiometabolic syndrome (CIRCS). In theory, it applies to all stages of COVID-19, i.e., prevention, acute proceedings (from COVID-19 diagnosis to resolution or three months), and long-term outcomes. Consequently, lifestyle changes, glycemic control, and regulation of the renin-angiotensin-aldosterone pathway have crucial implications for preventing and managing subjects with COVID-19. Finally, it is crucial to use cardioprotective drugs such as angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers and statins. Nevertheless, there is the need to conduct prospective studies and registries better to evaluate the issue of obesity in COVID-19 patients.

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