Iatrogenic scleroderma renal crisis: A case report and mini literature review for 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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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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Kidney involvement in systemic sclerosis: From pathogenesis to treatment

Journal of Scleroderma and Related Disorders ◽

10.1177/2397198318758607 ◽

2018 ◽

Vol 3 (1) ◽

pp. 43-52 ◽

Cited By ~ 14

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.

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Favourable Outcome of Scleroderma Renal Crisis

Journal of the Royal Society of Medicine ◽

10.1177/014107689608900114 ◽

1996 ◽

Vol 89 (1) ◽

pp. 49P-50P ◽

Cited By ~ 5

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.

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SCLERODERMA RENAL CRISIS: MORE EVIDENCE OF ANGIOTENSIN CONVERTING ENZYME INHIBITORS SUPERIORITY OVER ANGIOTENSIN-RECEPTOR BLOCKERS

American Journal of Kidney Diseases ◽

10.1053/j.ajkd.2015.02.012 ◽

2015 ◽

Vol 65 (4) ◽

pp. A17

Keyword(s):

Angiotensin Converting Enzyme ◽

Enzyme Inhibitors ◽

Angiotensin Receptor Blockers ◽

Angiotensin Converting Enzyme Inhibitors ◽

Scleroderma Renal Crisis ◽

Converting Enzyme ◽

Angiotensin Receptor ◽

Converting Enzyme Inhibitors ◽

Receptor Blockers ◽

Renal Crisis

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Scleroderma nephropathy: unsolved problems

Juvenis scientia ◽

10.32415/jscientia_2021_7_5_5-18 ◽

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.

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

The Journal of Rheumatology ◽

10.3899/jrheum.180582 ◽

2019 ◽

Vol 47 (2) ◽

pp. 241-248 ◽

Cited By ~ 2

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.

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

Case Reports in Nephrology ◽

10.1155/2021/8862405 ◽

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.

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POS1439 A MULTI-PREDICTOR MODEL TO PREDICT RISK OF SCLERODERMA RENAL CRISIS IN SYSTEMIC SCLEROSIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3613 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1004.1-1004

Author(s):

D. Xu ◽

R. Mu

Keyword(s):

Systemic Sclerosis ◽

Prediction Model ◽

Renal Involvement ◽

Area Under The Curve ◽

Individual Risk ◽

Multivariable Logistic Regression Analysis ◽

Scleroderma Renal Crisis ◽

Lasso Regression ◽

Patients At Risk ◽

Renal Crisis

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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Worldwide Expert Agreement on Updated Recommendations for the Treatment of Systemic Sclerosis

The Journal of Rheumatology ◽

10.3899/jrheum.181173 ◽

2019 ◽

Vol 47 (2) ◽

pp. 249-254 ◽

Cited By ~ 8

Author(s):

Jeska K. de Vries-Bouwstra ◽

Yannick Allanore ◽

Marco Matucci-Cerinic ◽

Alexandra Balbir-Gurman

Keyword(s):

Systemic Sclerosis ◽

Online Survey ◽

Geographical Area ◽

Phosphodiesterase Inhibitors ◽

Angiotensin Converting Enzyme Inhibitors ◽

Pressure Control ◽

Scleroderma Renal Crisis ◽

Converting Enzyme Inhibitors ◽

Hematopoietic Stem ◽

Level Of Agreement

Objective.To evaluate agreement of the updated European League Against Rheumatism and European Scleroderma Trials and Research group (EUSTAR) recommendations for treatment of systemic sclerosis (SSc) among international experts. In addition, to determine factors that might influence agreement.Methods.Level of agreement (10-point scale: 0 = not at all, 10 = completely agree) and local drug availability (yes/no) were assessed using an online survey. The Web link to the survey was shared with 481 unique e-mail addresses and SSc networks (Scleroderma Clinical Trials Consortium, Australian Scleroderma Interest Group, International Systemic Sclerosis Inception Cohort). Level of agreement was compared between subgroups stratified for participant characteristics.Results.In total, 263 experts participated, of whom n = 209 (79%) completed each single item. The majority were rheumatologists (n = 200, 76%) working in Europe (n = 185; 71%); 59% (n = 156) were EUSTAR members; and 57% (n = 151) had > 10 years of clinical experience. Overall level of agreement was high (mean 8.0, SD 2.5). The 3 highest mean agreements included (1) angiotensin-converting enzyme inhibitors for scleroderma renal crisis (9.2, SD 2.1); (2) blood pressure control in SSc-patients treated with corticosteroids (9.0, SD 2.2); (3) proton pump inhibitors to prevent reflux complications (9.0, SD 2.2). The 3 lowest mean agreements included (1) fluoxetine for Raynaud phenomenon (RP; 4.6, SD 2.8); (2) hematopoietic stem cell transplantation (HSCT) for severe SSc (7.1, SD 2.9); (3) phosphodiesterase inhibitors 5 for RP (7.3, SD 2.7). Agreement differed between Europe and non-Europe for the use of iloprost, bosentan, methotrexate, HSCT, and cyclophosphamide. Treatment availability could partially explain differential agreement for iloprost, bosentan, and HSCT.Conclusion.In general, worldwide expert agreement on updated recommendations for treatment of SSc is high, supporting their value. Differences in agreement are partially explained by geographical area and treatment availability.

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