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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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Gelber AC. Lung transplantation for scleroderma: Strength in numbers. Joint Bone Spine 2018; 85:5-7. [DOI: 10.1016/j.jbspin.2017.09.002] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2017] [Accepted: 09/06/2017] [Indexed: 11/28/2022]
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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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Zulian F. Systemic Sclerodermas. TEXTBOOK OF PEDIATRIC RHEUMATOLOGY 2016:384-405.e9. [DOI: 10.1016/b978-0-323-24145-8.00027-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Bose N, Chiesa-Vottero A, Chatterjee S. Scleroderma renal crisis. Semin Arthritis Rheum 2014; 44:687-94. [PMID: 25613774 DOI: 10.1016/j.semarthrit.2014.12.001] [Citation(s) in RCA: 81] [Impact Index Per Article: 7.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2014] [Revised: 11/28/2014] [Accepted: 12/05/2014] [Indexed: 12/23/2022]
Abstract
OBJECTIVES To discuss the pathophysiology, risk factors, clinical manifestations, diagnosis, treatment, prevention, and outcomes of scleroderma renal crisis (SRC), a serious yet potentially treatable complication of scleroderma (systemic sclerosis). METHODS A PubMed search for articles published up until April 2014 was conducted using the following keywords: scleroderma, systemic sclerosis, scleroderma renal crisis, renal, treatment, and prognosis. Literature was carefully reviewed, and different risk factors, treatment options, prognostic factors, and survival data were assessed. RESULTS SRC occurs in about 10% of all patients with scleroderma. It is characterized by malignant hypertension and progressive renal failure. Around 10% of SRC cases may present with normal blood pressure, termed normotensive renal crisis. The etiopathogenesis is presumed to be a series of insults to the kidneys resulting in endothelial injury, intimal proliferation, and narrowing of renal arterioles leading to decreased blood flow, hyperplasia of the juxtaglomerular apparatus, hyperreninemia, and accelerated hypertension. Risk factors include rapid skin thickening, use of certain medications such corticosteroids or cyclosporine, new-onset microangiopathic hemolytic anemia and/or thrombocytopenia, cardiac complications (pericardial effusion, congestive heart failure, and/or arrhythmias), large joint contractures, and presence of anti-RNA polymerase III antibody. Since the 1970s, with the advent of angiotensin-converting enzyme (ACE) inhibitors, mortality associated with SRC decreased from 76% to <10%. Some patients may progress to end-stage renal disease and need dialysis. Renal transplantation has improved survival, though SRC may recur in transplanted kidneys. CONCLUSIONS More than 60 years after its initial description, SRC still remains an important cause of morbidity and mortality in scleroderma. Since the advent of ACE inhibitors, the prognosis of SRC has improved substantially. Prompt diagnosis and treatment may help prevent adverse outcomes and improve survival.
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Affiliation(s)
- Nilanjana Bose
- Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, 9500 Euclid Ave, Desk A50, Cleveland, OH 44195
| | - Andres Chiesa-Vottero
- Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, 9500 Euclid Ave, Desk A50, Cleveland, OH 44195
| | - Soumya Chatterjee
- Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, 9500 Euclid Ave, Desk A50, Cleveland, OH 44195.
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Schachna L, Medsger TA, Dauber JH, Wigley FM, Braunstein NA, White B, Steen VD, Conte JV, Yang SC, McCurry KR, Borja MC, Plaskon DE, Orens JB, Gelber AC. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. ACTA ACUST UNITED AC 2007; 54:3954-61. [PMID: 17133609 DOI: 10.1002/art.22264] [Citation(s) in RCA: 140] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
OBJECTIVE Lung transplantation is a viable, life-saving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention. METHODS Survival following lung transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pulmonary fibrosis (IPF) and 38 with idiopathic pulmonary arterial hypertension (IPAH), the latter groups representing pathologically related primary pulmonary disorders. The end point was death from any cause. Risk of mortality in patients with scleroderma was compared with that in patients with IPF or IPAH, with adjustment for demographic and clinical parameters. RESULTS During 2 years of followup, 11 patients with scleroderma (38%), 23 with IPF (33%), and 14 with IPAH (37%) died. Cumulative survival at 6 months posttransplantation was 69% in the scleroderma group compared with 80% in the IPF group (log-rank P = 0.21) and 79% in the IPAH group (P = 0.38). The estimated risk of mortality at 6 months was increased in patients with scleroderma compared with those with IPF (relative risk [RR] 1.70, 95% confidence interval [95% CI] 0.74-3.93) and those with IPAH (RR 1.52, 95% CI 0.59-3.96), but the differences were not statistically significant. Over the following 18 months, there was convergence in the survival rates such that cumulative survival at 2 years was comparable, at approximately 64%, among all 3 groups. CONCLUSION Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma.
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Pham PTT, Pham PCT, Danovitch GM, Gritsch HA, Singer J, Wallace WD, Hayashi R, Wilkinson AH. Predictors and risk factors for recurrent scleroderma renal crisis in the kidney allograft: case report and review of the literature. Am J Transplant 2005; 5:2565-9. [PMID: 16162209 DOI: 10.1111/j.1600-6143.2005.01035.x] [Citation(s) in RCA: 49] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Scleroderma renal crisis (SRC) can lead to end-stage renal disease (ESRD) and subsequent need for dialysis and/or renal transplantation. We review all reported cases of renal transplantations in scleroderma patients from PubMed search, present UNOS data on transplant outcomes, and identify predictors for allograft SRC. Of the five cases with recurrent SRC, all developed ESRD within a year of onset of native kidney SRC, whereas none of those who developed ESRD more than 1-2 years after the onset of SRC developed recurrence. Anemia preceded allograft SRC in two cases, pericardial effusion in one, and skin tightening in two others. UNOS data (October 1987-July 2004) documented 260 transplants performed for the renal diagnosis of scleroderma, with a 5-year graft survival rate of 56.7%. The risk for allograft SRC recurrence appears to correlate with early native renal function loss following the onset of SRC. Recurrent SRC in the allograft may be heralded by multiple clinical markers known to be predictive of severe scleroderma, including progression of diffuse skin thickening, new-onset anemia and cardiac complications.
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Affiliation(s)
- Phuong-Thu T Pham
- David Geffen School of Medicine at UCLA, Nephrology Division, Kidney and Pancreas Transplantation, Los Angeles, CA, USA.
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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
Although the outcome of renal transplantation in patients with systemic lupus erythematosus (SLE) has been studied, there are few reports about the outcome of patients with systemic sclerosis who have undergone renal transplantation. We retrospectively collected data from the United Network for Organ Sharing (UNOS) Scientific Renal Transplant Registry from a 10-year period. From 1987 to 1997, 86 patients with systemic sclerosis who had renal transplantation were identified. Of these 86 patients, 70% were women, 86% were Caucasian, and the mean age at transplantation was 50.4 years. The overall mortality was 24% of the patient group; 44% (38/86) of renal grafts failed. First- through fifth-year graft survival rates were 62%, 60%, 57%, 50%, and 47%, respectively. The causes of graft failure could not be ascertained in 24 of 38 patients (63%). Among the known causes, 5 had acute rejection, 4 had chronic rejection, 3 had recurrence of scleroderma, and 1 each had infection and graft thrombosis. Immunosuppressive regimens used in the patients with systemic sclerosis consisted of antilymphocyte globulin in at least 25%. Sixty percent received a combination of steroids, azathioprine, and cyclosporine. The use of cyclosporine was not associated with either improvement of graft survival or an increased rate of graft failure. Graft survival at 5 years in patients with systemic sclerosis was comparable to that of patients with SLE who received renal transplantation, according to existent medical literature. Based upon these data, renal transplantation is as effective a treatment for restoring renal function in patients with systemic sclerosis as it is in patients with SLE. Those patients with systemic sclerosis whose renal function did not improve with angiotensin-converting enzyme (ACE)-inhibitor treatments after scleroderma renal crisis should be considered as transplant candidates. Although the data are incomplete, the use of cyclosporine may not confer the advantage of improving graft survival in patients with systemic sclerosis as compared with SLE patients.
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Affiliation(s)
- Y J Chang
- Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
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Caplin NJ, Dikman S, Winston J, Spiera H, Uribarri J. Recurrence of scleroderma in a renal allograft from an identical twin sister. Am J Kidney Dis 1999; 33:e7. [PMID: 10196038 DOI: 10.1016/s0272-6386(99)70248-9] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
A patient with scleroderma developed renal failure secondary to recurrence of scleroderma in a renal allograft from an identical twin. This report reviews the previous reports of scleroderma recurrence in renal allografts; the differential diagnosis of scleroderma renal crisis, including cyclosporine toxicity, malignant hypertension, and allograft rejection; and the pathophysiology of this disease.
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Affiliation(s)
- N J Caplin
- Departments of Internal Medicine and Pathology, The Mount Sinai Hospital, New York, NY 10028, USA.
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Ruggenenti P, Remuzzi G. Malignant vascular disease of the kidney: nature of the lesions, mediators of disease progression, and the case for bilateral nephrectomy. Am J Kidney Dis 1996; 27:459-75. [PMID: 8678055 DOI: 10.1016/s0272-6386(96)90155-9] [Citation(s) in RCA: 32] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Abstract
A case of thrombotic microangiopathy presenting as a hemolytic uremic syndrome complicated by untreatable hypertension and ultimately requiring bilateral nephrectomy is discussed. Severe hypertension and renal failure may complicate the course of vascular diseases of the kidney, including thrombotic microangiopathy, chronic hypertension, and scleroderma. Toxins, pressure stress, and immune material may trigger the initial injury to vascular endothelium. The malignant course of these renal vascular diseases seems linked to the severity of vascular injury. Endothelial injury manifests with swelling and detachment of endothelial cells from the basement membrane, expansion of the subendothelial space, and newly formed basement membrane-like material. In arterioles, endothelial injury precedes myointimal swelling and proliferation, leading to vascular lumina narrowing or obliteration and secondary glomerular ischemia, with glomerular tuft collapse and garland-like wrinkling and thickening of the capillary wall. Endothelial cell injury is very likely the common determinant of a cascade of events that lead to irreversible renal failure. When the initial insult (toxins, mechanical stress, antibodies) is promptly removed, lesions are self-limiting and the patient usually recovers. However, a severe insult persisting for some time can lead to chronic and irreversible vascular lesions that, through renal ischemia, trigger maximal activation of the renin angiotensin system with a brisk elevation in arterial blood pressure that may combine to further vascular injury and renal ischemia. Moreover, enhanced shear stress in the severely narrowed microcirculation, through abnormal von Willebrand factor processing, can also favor endothelial injury and platelet aggregation, which may further worsen the vascular lesions and sustain the microangiopathic process. Plasma manipulation, arteriolar vasodilators, and angiotensin-converting enzyme inhibitors normally control the vicious circle, but in few severe cases bilateral nephrectomy remains the last chance to save the patient's life.
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Affiliation(s)
- P Ruggenenti
- Mario Negri Institute for Pharmacological Research, Bergamo, Italy
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Abstract
Virtually all diseases affecting the native kidney recur in the kidney transplant with the exception of Alport syndrome, polycystic kidney disease, hypertension, chronic pyelonephritis, and chronic interstitial nephritis. Fortunately, in the majority of patients, recurrence of the original disease has minimal clinical impact, with only approximately 5% of all graft loss occurring as a result of recurrent disease. The primary renal diseases that commonly recur include membranoproliferative glomerulonephritis type II, IgA nephropathy, and focal and segmental glomerular sclerosis. The most common systemic disease that recurs is diabetic nephropathy. Living-related transplantation should be used with caution in patients with the hemolytic uremic syndrome, recurrent focal and segmental glomerular sclerosis, and membraneous glomerulonephritis. Fabry disease and primary hyperoxaluria type I are no longer absolute contraindications to kidney transplantation.
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Affiliation(s)
- E L Ramos
- Department of Medicine, University of Florida, Gainesville 32610-0224
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Affiliation(s)
- J F Donohoe
- National Renal Centre, Beaumont Hospital, Dublin, Ireland
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Waeber B, Schaller MD, Wauters JP, Brunner HR. Deterioration of renal function in hypertensive patients with scleroderma despite blood pressure normalization with captopril. KLINISCHE WOCHENSCHRIFT 1984; 62:728-30. [PMID: 6387262 DOI: 10.1007/bf01725706] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/20/2023]
Abstract
The orally active angiotensin-converting enzyme inhibitor captopril was administered for up to 11 weeks to three patients with progressive systemic sclerosis presenting with hypertension and plasma creatinine levels of 3.1, 7.2 and 10.4 mg/100 ml. Only one patient had malignant phase hypertension. In this patient a diuretic had to be added to captopril in order to keep blood pressure under control. Despite sustained blood pressure control during captopril administration, renal function deteriorated and hemodialysis treatment had to be started in all patients. Up to that time no substantial improvement in skin lesions was observed. During the period of dialysis, blood pressure was normal in all patients even though administration of captopril was discontinued. All three patients died of respiratory failure while on chronic hemodialysis for 3 to 4 weeks. These observations confirm that angiotensin-converting enzyme inhibition may be helpful in controlling blood pressure of patients with scleroderma. However, in contrast to some earlier reports, they also indicate that converting enzyme inhibition does not always prevent the multivisceral vascular lesions of scleroderma.
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Fries JF, Wasner C, Brown J, Feigenbaum P. A controlled trial of antihypertensive therapy in systemic sclerosis (scleroderma). Ann Rheum Dis 1984; 43:407-10. [PMID: 6378105 PMCID: PMC1001359 DOI: 10.1136/ard.43.3.407] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/19/2023]
Abstract
Antihypertensive treatment may be life saving in scleroderma renal crisis. Patients surviving such crises frequently have had dramatic improvement in the dermal manifestations of their scleroderma. To investigate the potential role of antihypertensive treatment in nonhypertensive patients we randomly assigned 28 patients with systemic sclerosis into drug (14) and placebo (14) groups, using blocked randomisation , and followed them up in a prospective, double-blind clinical trial for 24 months. Overall, both groups improved slightly, with both subjective and objective markers. There were no statistically significant differences and no clinically meaningful trends between the 2 groups, except that the blood pressure was reduced in the group on the active drug.
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Barnett AJ. Medical treatment of hypertension and renal failure in scleroderma. AUSTRALIAN AND NEW ZEALAND JOURNAL OF MEDICINE 1981; 11:411-5. [PMID: 6946761 DOI: 10.1111/j.1445-5994.1981.tb03523.x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/22/2023]
Abstract
Until recently acute renal failure in scleroderma has been uniformly fatal. Since 1973 there have been several reports of successful treatment by peritoneal and haemodialysis and nephrectomy, sometimes followed by renal transplantation. During the past few years there have been several reports of patients managed successfully with drug treatment. The present paper reports on a patient with scleroderma who is clinically well with stable renal function with drug treatment three years after the onset of acute renal impairment and hypertension. Other similar cases are reviewed.
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Simon NM, Graham MB, Kyser FA, Gashti EN. Resolution of renal failure with malignant hypertension in scleroderma. Case report and review of the literature. Am J Med 1979; 67:533-9. [PMID: 474603 DOI: 10.1016/0002-9343(79)90807-6] [Citation(s) in RCA: 19] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
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Lopez-Ovejero JA, Saal SD, D'Angelo WA, Cheigh JS, Stenzel KH, Laragh JH. Reversal of vascular and renal crises of scleroderma by oral angiotensin-converting-enzyme blockade. N Engl J Med 1979; 300:1417-9. [PMID: 220537 DOI: 10.1056/nejm197906213002505] [Citation(s) in RCA: 116] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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Waldherr R, Seelig HP, Klare B, Abigt J. Membranoproliferative glomerulonephritis in systemic sclerosis of childhood. VIRCHOWS ARCHIV. A, PATHOLOGICAL ANATOMY AND HISTOLOGY 1978; 379:169-79. [PMID: 150697 DOI: 10.1007/bf00432486] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
The renal lesions of a 5-year-old girl with progressive systemic sclerosis are described. The nephropathy was clinically characterised by moderate proteinuria, microscopic hematuria and transient hypertension. Light microscopy showed membranoproliferative glomerulonephritis of segmental character. On electron microscopy intramesangial, subendothelial and extramembranous glomerular deposits were observed. By immunofluorescence miscrosocpy deposit of IgG, Clq, C4, C3, C5, C8 and C9 in a predominantly subendothelial location were found in all glomeruli. Vascular lesions were of minor degree. Histological and immunohistological findings are compatible with an immune complex disease.
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LeRoy EC, Fleischmann RM. The management of renal scleroderma: experience with dialysis, nephrectomy and transplantation. Am J Med 1978; 64:974-8. [PMID: 350046 DOI: 10.1016/0002-9343(78)90452-7] [Citation(s) in RCA: 59] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
In 25 of 100 patients with scleroderma seen over a five year period irreversible renal failure developed; renal support was instituted in 17. Ten of 17 received peritoneal or hemodialysis, one survived. The remaining seven received hemodialysis plus nephrectomy; three survived. Two of these three underwent renal transplantation; one survived. This experience is presented to encourage improvement of these and other technics to increase the survival rate in the otherwise uniformly fatal renal failure associated with scleroderma (systemic sclerosis).
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Merino GE, Sutherland DE, Kjellstrand CM, Simmons RL, Najarian JS. Renal transplantation for progressive systemic sclerosis with renal failure: case report and review of previous experience. Am J Surg 1977; 133:745-9. [PMID: 326078 DOI: 10.1016/0002-9610(77)90170-2] [Citation(s) in RCA: 26] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Renal transplantation for terminal renal failure as a result of scleroderma (progressive systemic sclerosis [PSS]) seems justified if other organs have not been severely damaged by the disease. The recurrence of PSS in the graft, not observed in early reports, appears to have caused the rapid failure of a cadaveric graft in a forty year old woman with acute PSS. A similar case of rapid PSS recurrence in the transplant has been recently reported by others [7]. A more aggressive pattern of PSS seems to differentiate these two patients in whom recurrence was observed from four previous patients with long-term graft functions. The possibility of rapid renal transplant loss should be considered in the selection and prognosis of acute PSS patients.
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Cheigh JS, Kim SJ. Scleroderma kidney disease: a therapeutic approach with nephrectomy and hemodialysis. JOURNAL OF DIALYSIS 1977; 1:349-56. [PMID: 608857 DOI: 10.3109/08860227709038425] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
A 49 year old black female patient with progressive systemic sclerosis (scleroderma), multiple organ system involvement, uremia and malignant hypertension, was treated with maintenance hemodialysis and bilateral nephrectomy. Bilateral nephrectomy controlled refractory hypertension and appeared to alter the natural course of systemic sclerosis.
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Barker DJ, Farr MJ. Resolution of cutaneous manifestations of systemic sclerosis after haemodialysis. BRITISH MEDICAL JOURNAL 1976; 1:501. [PMID: 1252816 PMCID: PMC1638839 DOI: 10.1136/bmj.1.6008.501] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
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Medsger TA, Masi AT. Survival with scleroderma. II. A life-table analysis of clinical and demographic factors in 358 male U.S. veteran patients. JOURNAL OF CHRONIC DISEASES 1973; 26:647-60. [PMID: 4587746 DOI: 10.1016/0021-9681(73)90054-4] [Citation(s) in RCA: 105] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/11/2023]
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Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 32-1973. N Engl J Med 1973; 289:311-9. [PMID: 4352272 DOI: 10.1056/nejm197308092890610] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/10/2023]
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