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Scheen M, Dominati A, Olivier V, Nasr S, De Seigneux S, Mekinian A, Issa N, Haidar F. Renal involvement in systemic sclerosis. Autoimmun Rev 2023; 22:103330. [PMID: 37031831 DOI: 10.1016/j.autrev.2023.103330] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2023] [Accepted: 04/03/2023] [Indexed: 04/11/2023]
Abstract
Systemic sclerosis is a rare autoimmune vasculopathy associated with dysregulated innate and adaptive immunity that leads to generalized systemic fibrosis. Renal involvement occurs in a significant proportion of systemic sclerosis patients, and is associated with worse outcome. Scleroderma renal crisis (SRC) is the most studied and feared renal complication described in systemic sclerosis. However, with the emergence of ACE inhibitors and better management, the mortality rate of SRC has significantly decreased. Renal disease in systemic sclerosis offers a wide array of differential diagnoses that may be challenging for the clinician. The spectrum of renal manifestations in systemic sclerosis ranges from an isolated decrease in glomerular filtration rate, increased intrarenal arterial stiffness, and isolated proteinuria due to SRC to more rare manifestations such as association with antiphospholipid antibody nephropathy and ANCA-associated vasculitis. The changes observed in the kidneys in systemic sclerosis are thought to be due to a complex interplay of various factors, including renal vasculopathy, as well as the involvement of the complement system, vasoactive mediators such as endothelin-1, autoimmunity, prothrombotic and profibrotic cytokines, among others. This literature review aims to provide an overview of the main renal manifestations in systemic sclerosis by discussing the most recent epidemiological and pathophysiological data available and the challenges for clinicians in making a diagnosis of renal disease in patients with systemic sclerosis.
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Affiliation(s)
- Marc Scheen
- Hôpitaux Universitaires de Genève, Service de Néphrologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland.
| | - Arnaud Dominati
- Hôpitaux Universitaires de Genève, Service d'allergologie et immunologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland
| | - Valérie Olivier
- Hôpitaux Universitaires de Genève, Service de Néphrologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland
| | - Samih Nasr
- Mayo Clinic College of Medicine and Science, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
| | - Sophie De Seigneux
- Hôpitaux Universitaires de Genève, Service de Néphrologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland
| | - Arsène Mekinian
- Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne, 75012 Paris, France
| | - Naim Issa
- Mayo Clinic College of Medicine and Science, Division of Nephrology and Hypertension, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
| | - Fadi Haidar
- Hôpitaux Universitaires de Genève, Service de Néphrologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland
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Maritati F, Provenzano M, Lerario S, Corradetti V, Bini C, Busutti M, Grandinetti V, Cuna V, La Manna G, Comai G. Kidney transplantation in systemic sclerosis: Advances in graft, disease, and patient outcome. Front Immunol 2022; 13:878736. [PMID: 35958558 PMCID: PMC9360313 DOI: 10.3389/fimmu.2022.878736] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/18/2022] [Accepted: 06/30/2022] [Indexed: 11/30/2022] Open
Abstract
Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis, and inflammation. Renal disease occurring in patients with SSc may have a variable clinicopathological picture. However, the most specific renal condition associated with this disease is the scleroderma renal crisis (SRC), characterized by acute onset of renal failure and severe hypertension. SRC develops in about 20% of cases of SSc, especially in those patients with diffuse cutaneous disease. The prognosis of this condition is often negative, with a rapid progression to end-stage renal disease (ESRD). The advent of the antihypertensive angiotensin-converting enzyme inhibitors in 1980 was associated with a significant improvement in patients’ survival and recovery of renal function. However, the prognosis of these patients can still be improved. The dialytic condition is associated with early death, and mortality is significantly higher than among patients undergoing renal replacement therapy (RRT) due to other conditions. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for kidney transplantation (KT). In this review, we reported the most recent advances in KT in patients with ESRD due to SSc, with a particular overview of the risk of disease recurrence after transplantation and the evolution of other disease manifestations.
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Abbas F, El Kossi M, Shaheen IS, Sharma A, Halawa A. Journey of a patient with scleroderma from renal failure up to kidney transplantation. World J Transplant 2021; 11:372-387. [PMID: 34631469 PMCID: PMC8465513 DOI: 10.5500/wjt.v11.i9.372] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2021] [Revised: 04/10/2021] [Accepted: 08/19/2021] [Indexed: 02/06/2023] Open
Abstract
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.
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Affiliation(s)
- Fedaey Abbas
- Department of Nephrology, Faculty of Health and Science, University of Liverpool, Institute of Learning and Teaching, School of Medicine, Liverpool L69 3GB, United Kingdom
| | - Mohsen El Kossi
- Doncaster Renal Unit, Doncaster Royal Infirmary, Doncaster DN2 5LT, United Kingdom
| | - Ihab Sakr Shaheen
- Department of Paediatric Nephrology, St James’s University Hospital, Glasgow G51 4TF, United Kingdom
| | - Ajay Sharma
- Department of Transplant Surgery, Royal Liverpool University Hospital, Liverpool L7 8XP, United Kingdom
| | - Ahmed Halawa
- Department of Transplant Surgery, Sheffield Teaching Hospital, Sheffield S5 7AU, United Kingdom
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Bertrand D, Dehay J, Ott J, Sberro R, Brunelle C, Kamar N, Colosio C, Chatelet V, Albano L, Girerd S, Audard V, Barbet C, Dantal J, Ducloux D, Durrbach A, Garrigue V, Hazzan M, Heng AE, Mariat C, Merville P, Rerolle JP, Moulin B, Guerrot D. Kidney transplantation in patients with systemic sclerosis: a nationwide multicentre study. Transpl Int 2017; 30:256-265. [DOI: 10.1111/tri.12923] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2016] [Revised: 09/30/2016] [Accepted: 12/12/2016] [Indexed: 12/19/2022]
Affiliation(s)
- Dominique Bertrand
- Nephrology Dialysis Transplantation; Centre Hospitalier Universitaire de Rouen; Rouen Haute-Normandie France
| | - Julien Dehay
- Nephrology Dialysis Transplantation; Centre Hospitalier Universitaire de Rouen; Rouen Haute-Normandie France
| | - Julien Ott
- Department of Nephrology and Renal Transplantation; Hopitaux Universitaires de Strasbourg; Strasbourg Alsace France
| | - Rebecca Sberro
- Service de Transplantation et Unité de soins intensifs; Hôpital Necker; Paris France
| | | | - Nassim Kamar
- Department of Nephrology and Organ Transplantation Toulouse; CHU Rangueil; Haute Garonne France
| | | | | | - Laetitia Albano
- Nephrology-Dialysis-Transplantation; Nice University Hospital; Nice PACA France
| | - Sophie Girerd
- Nephrology Dialysis Transplantation; CHU Nancy; Nancy France
| | | | | | - Jacques Dantal
- Nephrology Dialysis Transplantation; Centre Hospitalier Universitaire; Nantes France
| | | | | | | | - Marc Hazzan
- Service de Néphrologie; CHRU Lille; Lille France
| | - Anne-Elisabeth Heng
- Service de Néphrologie; Hôpital Gabriel Montpied; CHU Clermont-Ferrand; Clermont-Ferrand France
| | - Christophe Mariat
- Nephrology Dialysis Transplantation; CHU Saint Etienne; Saint Etienne France
| | | | | | - Bruno Moulin
- Department of Nephrology and Renal Transplantation; Hopitaux Universitaires de Strasbourg; Strasbourg Alsace France
| | - Dominique Guerrot
- Nephrology Dialysis Transplantation; Centre Hospitalier Universitaire de Rouen; Rouen Haute-Normandie France
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Bussone G, Bérezné A, Pestre V, Guillevin L, Mouthon L. The scleroderma kidney: progress in risk factors, therapy, and prevention. Curr Rheumatol Rep 2011; 13:37-43. [PMID: 21061100 DOI: 10.1007/s11926-010-0145-7] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Scleroderma renal crisis (SRC) is characterized by malignant hypertension, oliguric/anuric acute renal failure, and important mortality, with a 5-year survival rate of 65%. SRC occurs in 2% to 5% of patients with systemic sclerosis (SSc), particularly those with diffuse cutaneous SSc in the first years of disease evolution. Several retrospective studies have found high-dose corticosteroid therapy to be associated with increased risk of SRC, and anti-RNA-polymerase III antibodies have been detected in one third of patients with SRC. Treatment relies on the early control of blood pressure with increasing doses of angiotensin-converting enzyme inhibitors, eventually associated with calcium channel blockers together with dialysis if necessary. After 2 years on dialysis, eligible patients should be considered for renal transplantation. The strategy for prevention of SRC lacks consensus. However, corticosteroids and/or nephrotoxic drugs should be avoided in patients with diffuse cutaneous SSc.
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Affiliation(s)
- Guillaume Bussone
- Pôle de Médecine Interne, Hôpital Cochin, 27 Rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France
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Hemolytic uremic syndrome with ischemic glomerulonephropathy and obliterative vasculopathy in a systemic sclerosis patient treated with cyclosporine-A. Rheumatol Int 2009; 29:821-4. [DOI: 10.1007/s00296-008-0826-y] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2008] [Accepted: 12/12/2008] [Indexed: 10/21/2022]
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Abstract
Scleroderma renal crisis (SRC) occurs in patients with systemic sclerosis (SSc) and is defined by otherwise unexplained rapidly progressive renal insufficiency associated with oliguria or rapidly progressive arterial hypertension or both. SRC is a rare and severe complication of SSc, most often encountered during the first 4 years of disease, almost only in patients with diffuse SSc. Factors predicting SRC were identified, including high-dose corticosteroid administration. Use of angiotensin-converting enzyme inhibitors (ACEI) has dramatically impressed the prognosis of SRC, but it mortality rate is still high. Treatment aims at normalizing blood pressure as soon as possible. ACEI should always be used, and additional anti-hypertensive agents, including calcium channel blockers and alpha- and beta-blockers, may be useful. Renal replacement therapy may be needed, but often (for almost half of patients) only temporarily.
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Affiliation(s)
- Luis Teixeira
- Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularites Nécrosantes et la Sclérodermie Systémique, Assistance Publique-Hôpitaux de Paris (AP-HP) et Université Paris-Descartes, Paris
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Abstract
Scleroderma renal crisis (SRC) was once a uniformly fatal complication of systemic sclerosis (SSc). With the introduction of angiotensin-converting enzyme inhibitors as treatment, outcomes have improved significantly, though 39% to 50% of SSc patients who develop SRC continue to have poor outcomes, including permanent dialysis and death. Early recognition and treatment with angiotensin-converting enzyme inhibitors are important in the effective management of SRC, though given the continuing morbidity and mortality caused by SRC, they are clearly not sufficient. Newer therapies based on the pathophysiologic mechanisms underlying the development and perpetuation of SRC are needed. This article reviews the epidemiology, pathogenesis, risk factors, clinical features, and treatment of SRC, with an emphasis on recent insights and developments.
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Affiliation(s)
- Elisa Y Rhew
- Department of Rheumatology, Feinberg School of Medicine, Northwestern University, 300 East Superior Street, Tarry 3-713, Chicago, IL 60611, USA.
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Abstract
Renal crisis occurs in systemic sclerosis patients with rapidly progressive diffuse cutaneous thickening early in their disease. SRC is characterized by malignant hypertension, hyperreninemia, azotemia, microangiopathic hemolytic anemia, and renal failure. This complication, which in the past has been almost uniformly fatal, is now successfully treated in most cases with ACE inhibitors. This therapy has improved survival, reduced requirement for dialysis, and in those on dialysis has often allowed discontinuation of this procedure 6 to 18 months later. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome. Chronic nonrenal crisis renal insufficiency is unusual and rarely progresses to significant renal dysfunction.
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Affiliation(s)
- V D Steen
- Division of Rheumatology, Immunology, and Allergy, Georgetown University Medical Center, Washington, DC 20007-2197, USA
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Affiliation(s)
- E C LeRoy
- Department of Medicine, Medical University of South Carolina, Charleston
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