Scleroderma renal crisis and renal involvement in systemic sclerosis

Background:Scleroderma renal crisis (SRC) is a life-threatening syndrome. The early identification of patients at risk is essential for timely treatment to improve the outcome[1].Objectives:We aimed to provide a personalized tool to predict risk of SRC in systemic sclerosis (SSc).Methods:We tried to set up a SRC prediction model based on the PKUPH-SSc cohort of 302 SSc patients. The least absolute shrinkage and selection operator (Lasso) regression was used to optimize disease features. Multivariable logistic regression analysis was applied to build a SRC prediction model incorporating the features of SSc selected in the Lasso regression. Then, a multi-predictor nomogram combining clinical characteristics was constructed and evaluated by discrimination and calibration.Results:A multi-predictor nomogram for evaluating the risk of SRC was successfully developed. In the nomogram, four easily available predictors were contained including disease duration <2 years, cardiac involvement, anemia and corticosteroid >15mg/d exposure. The nomogram displayed good discrimination with an area under the curve (AUC) of 0.843 (95% CI: 0.797-0.882) and good calibration.Conclusion:The multi-predictor nomogram for SRC could be reliably and conveniently used to predict the individual risk of SRC in SSc patients, and be a step towards more personalized medicine.References:[1]Woodworth TG, Suliman YA, Li W, Furst DE, Clements P (2016) Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol 12 (11):678-91.Disclosure of Interests:None declared

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Renal Involvement in Systemic Sclerosis: An Update

Kidney & Blood Pressure Research ◽

10.1159/000507886 ◽

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.

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AB0831 FREQUENCY OF RENAL CRISIS IN PATIENTS WITH SYSTEMIC SCLEROSIS: NEW DATA FROM A CHINESE COHORT AND A SYSTEMIC REVIEW WITH META-ANALYSIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3456 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1440.1-1440

Author(s):

D. Xu ◽

J. He ◽

T. LI ◽

R. Mu

Keyword(s):

Systemic Sclerosis ◽

Meta Analysis ◽

Renal Involvement ◽

Geographic Origin ◽

Systemic Review ◽

Scleroderma Renal Crisis ◽

Pooled Prevalence ◽

Chinese Cohort ◽

Life Threatening ◽

Renal Crisis

Background:Scleroderma renal crisis (SRC) is a rare but life-threatening complication in SSc, which defined as malignant hypertension and progressive renal failure [1]. Previous reports of the prevalence for scleroderma renal crisis (SRC) show variable outcomes.Objectives:We aim to find out the prevalence for SRC in Chinese, and conduct a meta-analysis to assess the prevalence of SRC in worldwide and investigate possible factors of variability.Methods:We evaluated the prevalence for SRC and compared the clinical characteristics in SSc patients with or without SRC. A systematic review on the prevalence of SRC was carried out in PubMed, Embase and Cochrane. Meta-analysis was performed using available data on the prevalence for SRC.Results:302 patients were enrolled in PKUPH-SSc cohort and the prevalence of SRC was 4.97%. In our cohort, SRC was found in 3.60% limited cutaneous SSc (lcSSc) and 7.75% diffused cutaneous SSc (dcSSc) patients. Twenty-nine studies including a total population of 22686 SSc patients were analyzed in the meta-analysis. The overall pooled prevalence of SRC was 4.0%, but heterogeneity among the studies was substantial (I2=86.1%, P <0.00001). Meta-regression revealed that subtype of SSc and geographic factors partially explained this heterogeneity. Sub-group analyses demonstrated that the prevalence of SRC in dcSSc and lcSSc were 9.0% and 2.0%. The SRC prevalence in SSc was 3.0% in Asia, 5.0% in Europe, and 3.0% in North America, respectively.Conclusion:Data on our new cohort showed the prevalence of SRC was around 5% in China, and meta-analysis confirmed that the prevalence of SRC varies among centers. The marked heterogeneity of SRC prevalence was partially explained by subtype of SSc and geographic origin of patients.References:[1]Woodworth TG, Suliman YA, Li W, Furst DE, Clements P (2016) Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol 12 (11):678-91.Disclosure of Interests:None declared

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Erratum: Scleroderma renal crisis and renal involvement in systemic sclerosis

Nature Reviews Nephrology ◽

10.1038/nrneph.2017.183 ◽

2018 ◽

Vol 14 (2) ◽

pp. 137-137 ◽

Cited By ~ 6

Author(s):

Thasia G. Woodworth ◽

Yossra A. Suliman ◽

Wendi Li ◽

Daniel E. Furst ◽

Philip Clements

Keyword(s):

Systemic Sclerosis ◽

Renal Involvement ◽

Scleroderma Renal Crisis ◽

Renal Crisis

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

Discussion of Clinical Cases ◽

10.5430/dcc.v8n3p4 ◽

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.

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Faculty Opinions recommendation of Scleroderma renal crisis and renal involvement in systemic sclerosis.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.726756543.793527345 ◽

2017 ◽

Author(s):

Martin Aringer

Keyword(s):

Systemic Sclerosis ◽

Renal Involvement ◽

Scleroderma Renal Crisis ◽

Renal Crisis

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French recommendations for the management of systemic sclerosis

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01844-y ◽

2021 ◽

Vol 16 (S2) ◽

Author(s):

Eric Hachulla ◽

Christian Agard ◽

Yannick Allanore ◽

Jerome Avouac ◽

Brigitte Bader-Meunier ◽

...

Keyword(s):

Systemic Sclerosis ◽

Proximal Region ◽

Scleroderma Renal Crisis ◽

Cardiac Damage ◽

Favorable Prognosis ◽

Causes Of Mortality ◽

Cutaneous Form ◽

Pulmonary Arterial ◽

Renal Crisis ◽

Severe Pulmonary Arterial Hypertension

AbstractSystemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.

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Vascular Complications of Systemic Sclerosis during Pregnancy

International Journal of Rheumatology ◽

10.1155/2010/287248 ◽

2010 ◽

Vol 2010 ◽

pp. 1-5 ◽

Cited By ~ 14

Author(s):

Eliza F. Chakravarty

Keyword(s):

Systemic Sclerosis ◽

Pulmonary Arterial Hypertension ◽

Pregnancy Outcomes ◽

Vascular Complications ◽

Autoimmune Disorder ◽

Morbidity And Mortality ◽

Scleroderma Renal Crisis ◽

Hemodynamic Changes ◽

Pulmonary Arterial ◽

Renal Crisis

Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by progressive fibrosis of the skin and visceral tissues as well as a noninflammatory vasculopathy. Vascular disease in systemic sclerosis is a major cause of morbidity and mortality among nonpregnant patients with SSc and is even a bigger concern in the pregnant SSc patient, as the underlying vasculopathy may prevent the required hemodynamic changes necessary to support a growing pregnancy. Vascular manifestations including scleroderma renal crisis and pulmonary arterial hypertension should be considered relative contraindications against pregnancy due to the high associations of both maternal and fetal morbidity and mortality. In contrast, Raynaud's phenomenon may actually improve somewhat during pregnancy. Women with SSc who are considering a pregnancy or discover they are pregnant require evaluation for the presence and extent of underlying vasculopathy. In the absence of significant visceral vasculopathy, most women with SSc can expect to have reasonable pregnancy outcomes.

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