Low Frequency and Poor Outcome of Scleroderma Renal Crisis in The Follow-up of 306 Patients With Systemic Sclerosis

2006 ◽  
Vol 12 (Supplement) ◽  
pp. S2
Author(s):  
P D. Sampaio-Barros ◽  
A M. Samara ◽  
F J. Marques Neto
Keyword(s):  
Systemic Sclerosis ◽  
Low Frequency ◽  
Scleroderma Renal Crisis ◽  
Poor Outcome ◽  
Renal Crisis

scholarly journals POS0854 SEX DIFFERENCES IN SYSTEMIC SCLEROSIS PATIENTS IN A SINGLE CENTER IN EASTERN EUROPE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 681.2-681
Author(s):  
A. Petre ◽  
L. Groseanu ◽  
A. Balanescu ◽  
V. Bojinca ◽  
D. Opris-Belinski ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Sex Differences ◽  
Systemic Sclerosis ◽  
Vascular Involvement ◽  
Scleroderma Renal Crisis ◽  
Acute Phase Reactants ◽  
Ventricular Ejection Fraction ◽  
Data Extract ◽  
Renal Crisis

Background:The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in SSc patients, and contradictory results have often been observed.Objectives:The aim of the study was to assess differences in disease manifestations in a cohort of SSc patients according to gender.Methods:We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096.We looked at sex influence on disease characteristics at baseline and then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival.Results:173 patients with SSc were available for the baseline analyses. Males were older (52,96 vs 45,88, p=0.009), were more likely to smoke (73% vs 7%, p<0,001), had more frequent diffuse skin involvement (73,1% vs 56,5%,p<0.01), higher modified Rodnan skin score (34,61% vs 17%, p=0.01) and activity score(84,62% vs 46,26%,p<0.001) and were more often associed with positive acute phase reactants (65,38% vs 38,77%, p=0.01). Severe interstitial lung disease was more common in males (59,09% vs 27,53%, p=0.003), also the presence of tendon friction rubs was more frequent in this sex group (23,07% vs 8,84%, p=0.032).In the longitudinal analysis after a mean follow-up of 3,5(±0,65) years, male sex was associated with a higher risk of scleroderma renal crisis (OR:9.45 (1.49 to 59.69); p=0.004), digital contractures (OR:8,2 (3,1 to 21,9); p<0,001), arrhythmias (OR:3,37 (1.36 to 8,34); p=0.006), pulmonary fibrosis (OR: 3.56, (1.51 to 8.41); p=0.003), pulmonary hypertension (OR: 3.01 (1.19 to 7,59); p=0.016), severe vascular involvement (OR:2,86, (1,22 to 6,73); p=0.013) and low ventricular ejection fraction (OR: 2,84, (1.2 to 6,73); p=0.014). Males had significantly reduced survival time after diagnosis (p=0,004). The most frequent causes of death were scleroderma renal crisis in males and pulmonary hypertension in females.Conclusion:Although more common in women, SSc appears as strikingly more severe in men. Our results demonstrate a higher risk of severe organ involvement and poor prognosis in men. These results raise the point of including sex in the management and the decision-making process.Disclosure of Interests:None declared

scholarly journals Gender differences in organ involvement and survival in systemic sclerosis – experience of a EUSTAR center

2021 ◽  
Vol 30 (2) ◽  
pp. 68-76
Author(s):  
Laura Groseanu ◽  
◽  
Andreea Petre ◽  
Andra Balanescu ◽  
Violeta Bojinca ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Organ Involvement ◽  
Vascular Involvement ◽  
Scleroderma Renal Crisis ◽  
Ventricular Ejection Fraction ◽  
Data Extract ◽  
Using Data ◽  
Renal Crisis

Introduction. The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in SSc patients, and contradictory results have often been observed. Material and method. We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096 . We looked at sex influence on disease characteristics at baseline and then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. Results. 173 patients with SSc were available for the baseline analyses. In the longitudinal analysis after a mean follow-up of 3.5(±0.65) years, male sex was associated with a higher risk of scleroderma renal crisis (OR:9.45 (1.49 to 59.69); p = 0.004), digital contractures (OR:8.2 (3.1 to 21.9); p < 0.001), arrhythmias (OR: 3.37 (1.36 to 8.34); p = 0.006), pulmonary fibrosis (OR: 3.56, (1.51 to 8.41); p = 0.003), pulmonary hypertension (OR: 3.01 (1.19 to 7.59); p = 0.016), severe vascular involvement (OR:2.86, (1.22 to 6.73); p = 0.013) and low ventricular ejection fraction (OR: 2.84, (1.2 to 6.73); p = 0.014). Males had significantly reduced survival time after diagnosis (p = 0.004). The most frequent causes of death were scleroderma renal crisis in males and pulmonary hypertension in females. Conclusions. Although more common in women, SSc appears as strikingly more severe in men. Our results demonstrate a higher risk of severe organ involvement and poor prognosis in men. These results raise the point of including sex in the management and the decision-making process.

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

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

scholarly journals French recommendations for the management of systemic sclerosis

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.

scholarly journals Vascular Complications of Systemic Sclerosis during Pregnancy

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
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.

Alveolar Hemorrhage and Pulmonary Hypertension in Systemic Sclerosis: A Continuum of Scleroderma Renal Crisis?

2001 ◽  
Vol 7 (2) ◽  
pp. 115-119 ◽  
Author(s):  
Thomas M. Herndon ◽  
Theodore T. Kim ◽  
Bruce E. Goeckeritz ◽  
Lisa K. Moores ◽  
Robert J. Oglesby ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Alveolar Hemorrhage ◽  
Scleroderma Renal Crisis ◽  
Renal Crisis

Analysis of Anti-RNA Polymerase III Antibody-positive Systemic Sclerosis and Altered GPATCH2L and CTNND2 Expression in Scleroderma Renal Crisis

2020 ◽  
Vol 47 (11) ◽  
pp. 1668-1677
Author(s):  
Edward P. Stern ◽  
Sandra G. Guerra ◽  
Harry Chinque ◽  
Vanessa Acquaah ◽  
David González-Serna ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Renal Biopsy ◽  
Genetic Susceptibility ◽  
Rna Polymerase Iii ◽  
Human Kidney ◽  
Scleroderma Renal Crisis ◽  
Nucleotide Polymorphisms ◽  
Polymerase Iii ◽  
Renal Crisis

ObjectiveScleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) strongly associated with anti-RNA polymerase III antibody (ARA) autoantibodies. We investigated genetic susceptibility and altered protein expression in renal biopsy specimens in ARA-positive patients with SRC.MethodsARA-positive patients (n = 99) with at least 5 years’ follow-up (49% with a history of SRC) were selected from a well characterized SSc cohort (n = 2254). Cases were genotyped using the Illumina Human Omni-express chip. Based on initial regression analysis, 9 single-nucleotide polymorphisms (SNP) were chosen for validation in a separate cohort of 256 ARA-positive patients (40 with SRC). Immunostaining of tissue sections from SRC or control kidney was used to quantify expression of candidate proteins based upon genetic analysis of the discovery cohort.ResultsAnalysis of 641,489 SNP suggested association of POU2F1 (rs2093658; P = 1.98 × 10−5), CTNND2 (rs1859082; P = 5.58 × 10−5), HECW2 (rs16849716; P = 1.2 × 10−4), and GPATCH2L (rs935332; P = 4.92 × 10−5) with SRC. Further, the validation cohort showed an association between rs935332 within the GPATCH2L region, with SRC (P = 0.025). Immunostaining of renal biopsy sections showed increased tubular expression of GPATCH2L (P = 0.026) and glomerular expression of CTNND2 (P = 0.026) in SRC samples (n = 8) compared with normal human kidney controls (n = 8), despite absence of any genetic replication for the associated SNP.ConclusionIncreased expression of 2 candidate proteins, GPATCH2L and CTNND2, in SRC compared with control kidney suggests a potential role in pathogenesis of SRC. For GPATCH2L, this may reflect genetic susceptibility in ARA-positive patients with SSc based upon 2 independent cohorts.

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