Renal Involvement In Systemic Sclerosis Research Articles

Renal pathology and clinical associations in systemic sclerosis: a historical cohort study.

BackgroundThe information guiding the treatment decision(s) for renal diseases in systemic sclerosis (SSc) is the renal pathological finding. This study aimed to evaluate the renal pathological diagnosis and its clinical feature among SSc.MethodA historical cohort study was performed on adult Thai SSc patients who underwent renal biopsy during January 2005–December 2016. The renal pathologic findings and patient clinical characteristics were reviewed. Chi-square or Fisher’s exact test was applied to analyze the association between clinical manifestation and renal pathology.ResultsOf the 26 SSc patients identified (77% female), 46% had the diffuse cutaneous SSc subtype. The mean age at the time of biopsy was 53.2±14.4 years and median duration of disease was 2.4 years (IQR 0.5–7.0). Rapidly progressive glomerulonephritis (RPGN) was the most common renal manifestation (53.9%) followed by nephrotic syndrome (19.2%) and nephritis (11.5%). The pathological diagnosis included lupus nephritis (LN) class IV (26.9%), LN class V (19.2%), scleroderma renal crisis (SRC; 19.3%), progressive renal disease in scleroderma (7.7%), and IgA nephropathy (7.7%). The nephrotic syndrome was the most common renal feature among LN class V patients, whereas RPGN was the commonest renal presentation among LN class IV and SRC patients (p=0.001). Dialysis treatment at the time of kidney biopsy was significantly higher in SRC patients than in the other groups (p<0.001). The SRC tended to have more frequent cardiac involvement, pulmonary fibrosis, and shorter disease duration than the other groups.ConclusionThis is the first report of renal pathologic findings in Thai SSc patients. RPGN is the commonest renal manifestation among SSc who underwent kidney biopsy; for whom LN was the most common pathological finding. Nephrotic syndrome is a clinical feature of glomerular diseases other than renal involvement in SSc.

Points to consider in renal involvement in systemic sclerosis

This article discusses points to consider when undertaking a clinical trial to test therapy for renal involvement in SSc, not including scleroderma renal crisis. Double-blind, randomized controlled trials vs placebo or standard background therapy should be strongly considered. Inclusion criteria should consider a pre-specified range of renal functions or stratification of renal function. Gender and age limitations are probably not necessary. Concomitant medications including vasodilators, immunosuppressants and endothelin receptor antagonists and confounding illnesses such as diabetes, kidney stones, hypertension and heart failure need to be considered. A measure of renal function should be strongly considered, while time to dialysis, mortality, prevention of scleroderma renal crisis and progression of renal disease can also be considered, although they remain to be validated. Detailed, pre-planned analysis should be strongly considered and should include accounting for missing data.

Scleroderma renal crisis and renal involvement in systemic sclerosis.

Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3-5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients. The prognosis of SRC substantially improved in the 1980s with the introduction of angiotensin-converting-enzyme inhibitors for rapid blood pressure control, with additional antihypertensive agents as required. However, the survival of patients with SRC can still be improved. Current patient survival is 70-82% at 1 year, but decreases to 50-60% at 5 years despite dialysis support. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for transplantation. In this Review, we discuss progress made in the identification and proactive management of patients at risk of SRC and make recommendations aimed at optimizing management for those who progress to chronic kidney failure.

Profile of systemic sclerosis and associated renal involvement

Background: Systemic sclerosis (SSc) patients are encountered in all parts of the world. Few Indian studies are found in the literature on this connective tissue disorder of unknown etiology. The spectrum of sclerodermatous diseases comprises a wide variety of clinical entities. Renal involvement is not common in Indian patients when compared to western patient's in spite of the kidney being commonly affected in scleroderma due to vascular changes. Aim and Objective: To primarily study the prevalence of renal involvement in SSc and to correlate it with high-sensitivity C-reactive protein (hsCRP) over a period of 1 year in a tertiary care hospital in North-East India. Materials and Methods: A total of 38 consecutive scleroderma patients of both sexes, diagnosed by established criteria, were examined, and hsCRP was estimated in all. Evaluation for nephropathy by biochemical tests and sonography were carried out. Relevant clinical/biochemical examinations were carried out. Results: SSc was 3 times more common in mostly in middle aged females. About 50% presented within 1 year of disease onset. Renal involvement was uncommon (18%) and hsCRP was detected in 26%. Conclusion: SSc with nephropathy, not renal crisis, presents early and hsCRP is a good marker for the same.

Anti-proteinase 3 antibodies in diffuse systemic sclerosis (SSc) with normotensive renal impairment: is it suggestive for an overlapping between SSc and idiopathic vasculitis?

OBJECTIVE: To test the prevalence of anti-neutrophil cytoplasmic antibodies (ANCA) in systemic sclerosis (SSc) and to verify a possible association of ANCA with normotensive renal involvement in SSc. PATIENTS AND METHODS: 51 patients affected by SSc, 35 with diffuse scleroderma (dSSc) and 16 with limited scleroderma (lSSc), were tested for ANCA by indirect immunofluorescence (IIF) on human ethanol and formalin-acetone-fixed granulocytes (before and after DNase treatment), by conventional enzyme linked immuno-sorbent assay (ELISA) and by capture-ELISA. RESULTS: Six out of 51 selected SSc patients had ANCA by IIF (11.7%) and five presented a perinuclear/nuclear atypical ANCA pattern. In all cases we only found anti-proteinase3 (aPR3) antibodies. All ANCA positive patients had diffuse form of SSc (17.1%), all were anti-Scl70 positive (aScl70), five patients had proteinuria, three had microscopic haematuria. All ANCA positive patients were normotensive with normal renin plasma levels, the mean erythrocyte sedimentation rate (ESR) was higher in this group compared to the other SSc patients. CONCLUSIONS: Our study shows that aPR3 is not rare in dSSc. According to the clinical and serological findings and to the recent literature, we can hypothesise that when ANCA are found in SSc, an overlapping of scleroderma with systemic necrotizing vasculitis should be suspected.

Lack of activation of renal functional reserve predicts the risk of significant renal involvement in systemic sclerosis

ObjectiveTo evaluate if defective activation of renal functional reserve (RFR) in systemic sclerosis (SSc) without clinical signs of renal involvement predicts the risk of developing clinically relevant renal damage.MethodsTwenty-eight normotensive...

Intrarenal color Doppler sonography in studying patients affected by systemic sclerosis

Aim: Systemic sclerosis is a generalized disorder which affects the connective tissue of the skin and internal organs and is associated with alterations of the microvasculature. Scleroderma renal crisis involves almost 50% of patients with a 25% of which developing renal failure. In our study we evaluated utility of intrarenal quantitative parameters (Resistive Index: R. I.; Pulsatility Index: P. I.) during renal color Doppler sonography, as possible prognostic indicators of renal involvement in systemic sclerosis. Material and methods: In a period of 60 mounths, we examinated 78 patients (65 females and 13 males) affected by systemic sclerosis and 100 controls. All subjects underwent intrarenal color Doppler sonography with evaluation of R.I. and P.I. every 12 mounths. We then correlated these parameters with renal functional values (creatinine clearance and GFR), microalbuminuria and urine test. Results: In 52 patients (47 females and 5 males) in respect to controls R.I. (0.83±0.08 vs. 0.58±0.04) was significantly higher (p<0.05). There was no statistical difference in P.I. between patients and controls. Conclusion: Even if our preliminary findings have to be confirmed in larger patient groups, our results suggest that evaluation of R.I. by color Doppler sonography in patients with systemic sclerosis could allow a selection of those patients at risk of developing renal vascular involvement and, by consequence, renal crisis as a complication of systemic sclerosis.

Prevalence of renal involvement in Indian patients with systemic sclerosis

Renal involvement in systemic sclerosis (SSc) either in the form of scleroderma renal crisis (SRC) or nonrenal crisis abnormalities has been reported to be in the range of 60-80%. Renal involvement is thought to be rare in Indian patients with SSc. However, there is paucity of data. To study the frequency and pattern of renal involvement in Indian patients with SSc. A single center prospective, cross sectional study. We prospectively evaluated the patients with SSc attending the Rheumatology Clinic. All patients were evaluated for renal involvement. All patients underwent measurement of blood pressure, urine examination, glomerular filtration rate (GFR) estimation using Cockcroft-Gault formula and kidney biopsy when indicated. Statistical analysis was performed using SAS 8.0 statistical package. Eighty-seven patients were included in the study from July 2001 to December 2004. Mean age of patients was 36.88 +/- 12.51 years. About 30% of patients had diffuse cutaneous SSc. None of these patients had SRC either at enrollment in the study or during follow-up. Eleven (12.6%) patients had hypertension. Six (6.9%) patients had abnormal urinary findings in the form of either active urinary sediment or significant proteinuria. Only one patient had azotemia (plasma creatinine > 1.8 mg/dl). Calculated GFR CONCLUSION: SRC is very rare in Indian patients with SSc. However, non-renal crisis abnormalities appear to be as common in Indian patients as compared to the western literature.

Failure of losartan to control blood pressure in scleroderma renal crisis

Losartan, a selective antagonist of the angiotensin-receptor subtype 1 is a recently introduced antihypertensive agent which may be of particular use for those patients intolerant of angiotensin-converting-enzyme (ACE) inhibitors. Losartan may be interchangeable with ACE inhibitors both in terms of efficacy and long-term patient benefit. We wish to report the failure of losartan to control hypertension in a patient with scleroderma renal crisis (the onset of accelerated arterial hypertension and/or rapidly progressive oliguric renal failure during the course of systemic sclerosis 1 Steen VD Renal involvement in systemic sclerosis. Clin Dermatol. 1994; 12: 253-258 Summary Full Text PDF PubMed Scopus (25) Google Scholar ), in whom ACE inhibitors controlled the blood pressure.

Renal involvement in systemic sclerosis

Renal crisis occurs in systemic sclerosis patients with rapidly progressive diffuse cutaneous thickening early in their disease course. SRC is characterized by malignant hypertension, hyperreninemia, azotemia, and microangiopathic hemolytic anemia. This complication was almost uniformly fatal but can now be treated successfully in most cases with ACE inhibitors. The result has been improved survival, reduced requirement for dialysis, and even discontinuation of dialysis after 6 to 18 months of treatment. Prompt diagnosis and early aggressive treatment of SRC with ACE inhibitors will result in the most optimal outcome.

Failure to control venereal disease.

<h3>Objective</h3> To evaluate if defective activation of renal functional reserve (RFR) in systemic sclerosis (SSc) without clinical signs of renal involvement predicts the risk of developing clinically relevant renal damage. <h3>Methods</h3> Twenty-eight normotensive SSc patients with normal renal function and no urinary abnormalities were submitted to an intravenous amino acid load to activate RFR. Nineteen patients (six with diffuse cutaneous SSc (dcSSc)) had an RFR activation defect, while nine (two with dcSSc) showed normal RFR. All patients were followed up for 5 years, with periodic evaluation of renal function, urinary protein excretion and systemic blood pressure (BP). <h3>Results</h3> At admission, patients with normal RFR had lower BP than those with abnormal RFR; no age, disease duration or creatinine clearance (CCr) differences were found. Five years later, patients with abnormal RFR showed, with respect to basal values, a significantly higher CCr reduction than patients with normal RFR (mean percent decrease 15.4±9.5 vs 2.6±3.8, p&lt;0.001). Among patients with abnormal RFR, 13 (68.4%) showed a CCr reduction of ≥2 ml/min/year, with a final CCr of ≤70 ml/min in eight cases; two patients developed microalbuminuria and 10 grade 1 or 2 systemic hypertension. Significant CCr reduction rates were found in eight patients with high BP and in five patients who remained normotensive. No patient with normal RFR had proteinuria or high BP during follow-up. <h3>Conclusions</h3> Lack of RFR activation is an early sign of renal involvement in SSc, and is a harbinger of an increased risk of developing renal insufficiency and systemic hypertension.