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.

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

scholarly journals AB0580 GENDER DIFFERENCES IN SYSTEMIC SCLEROSIS- IMPACT ON DISEASE PHENOTYPE AND PROGNOSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1586.1-1587
Author(s):  
L. Groseanu ◽  
A. Balanescu ◽  
V. Bojinca ◽  
D. Opris-Belinski ◽  
I. Saulescu ◽  
...  
Keyword(s):  
Gender Differences ◽  
Systemic Sclerosis ◽  
Disease Duration ◽  
Vascular Involvement ◽  
Scleroderma Renal Crisis ◽  
Peripheral Vascular ◽  
Male Patients ◽  
Male Sex ◽  
End Stage ◽  
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.Objectives:To evaluated sex influence on disease characteristics at baseline and then to estimate the effects of sex on disease progression and survival.Methods:We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096. 173 patients were analysed (26 males).The severity of organ system involvement was defined as described previously (1).Results:Males were significantly older at symptom onset (p=0.007) and at first center visit (p=0.009). There were no differences regarding disease duration at first visit or the interval between the onset of Raynaud syndrome and other non-Raynaud manifestations (p=0.06). Male patients were significantly more likely to have ever smoked (p<0.001), males more often had severe or end-stage peripheral vascular involvement (p=0.01). Modified Rodnan skin score (mRSS) was significantly higher in males (p=0.004). We found no difference regarding musculoarticular involvement, except for digital contractures (p=0.001) and tendon friction rubs (p=0,044). Males more often had interstitial lung disease (ILD) (p=0.013) which was also more frequently severe or end-stage (p = 0.003). Cardiac involvement was more common in males: pulmonary hypertension (PAH) (p = 0.018), arrhytmias (p=0.012), left ventricle ejection fraction<45% (p=0.014). The frequency of scleroderma renal crisis (SRC) was higher in males (p=0.025). Gastrointestinal involvement did not differ between groups EScSG (European Scleroderma Study Group) disease activity scores were higher in males (p=0.001). The isolated presence of antitopoisomerase-1 or anticentromere antibodies did not differ between groups. Mortality rate was similar between sexes, although male sex is a independent predictor for the death associated with ILD, SRC, arrythmiasIn multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.56, (1.35 to 1.84); p<0.001), a higher frequency of severe vascular disease (OR: 1.38 (1.11 to 1.67); p<0.001), severe digital contractures (OR:1.92(1.68 to 2,42); p<0.001), interstitial lung disease OR: 1.22 (0.9 to 1.47); p<0.001), severe heart involvement (OR: 1.56 (1.22 to 2,1); p<0.001) and SRC (OR: 3.31 (1.87 to 5620); p<0.003). In the longitudinal analysis, after a mean follow-up of 7.2 (±2.6) years, male sex was predictive of new onset of scleroderma renal crisis (HR: 3.66 (1.82 to 4.86); p=0.006) and heart failure (HR: 1.9 (1.36 to 3.18); p=0.01).Conclusion:In essence, the disease prophyle in females is that of younger age of onset, longer disease duration at first center visit, less severe peripheral vascular involvement, the most frequent cause of death being PAH. In contrast, males are older at onset, present earlier in their disease, have dcSSc, more severe peripheral vascular disease, higher mRSS, more frequent and severe ILD, more frequent heart involvement, higher risk of PAH and SRC, the most common cause of death being ILD. These results raise the point of including sex in the management and the decision-making process.References:[1]Peoples C, Medsger TA Jr, Lucas M et al Gender differences in systemic sclerosis: relationship to clinical features, serologic status and outcomes.J Scleroderma Relat Disord. 2016;1(2):177–240Disclosure of Interests:Laura Groseanu Speakers bureau: novartis, eli-lilly, ucb, pfizer,sandoz, Andra Balanescu Consultant of: pfizer, Speakers bureau: Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, UCB, Violeta Bojinca Speakers bureau: Eli-Lilly, Novartis, Pfizer, Daniela Opris-Belinski Speakers bureau: Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Ioana Saulescu Speakers bureau: Eli-Lilly, Pfizer, Diana Mazilu: None declared, Sanziana Daia-Iliescu Speakers bureau: sandoz, Andreea Borangiu: None declared, Florian Berghea Paid instructor for: abbvie, Speakers bureau: gideon richter, egis, novartis,ucb, cosmin-laurentiu constantinescu: None declared, CLAUDIA COBILINSCHI Speakers bureau: novartis, Maria Magdalena Negru: None declared, mihai abobului Speakers bureau: gideon richter, Ruxandra Ionescu Consultant of: Consulting fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Speakers bureau: Consulting and speaker fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz

scholarly journals Cerebral infarction caused by systemic sclerosis: a case report

2021 ◽  
Vol 49 (9) ◽  
pp. 030006052110440
Author(s):  
Qingqing Wang ◽  
Mengen Zhang ◽  
Mingfeng Zhai ◽  
Zongyou Li
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Cerebral Infarction ◽  
Vascular Lesions ◽  
Scleroderma Renal Crisis ◽  
Collagen Deposition ◽  
Cerebral Involvement ◽  
The Brain ◽  
Common Manifestation ◽  
Renal Crisis

Systemic sclerosis, also known as scleroderma, is a rare multisystem autoimmune disease characterized by vascular lesions caused by collagen deposition in the skin and viscera and damage to the endothelium. Endothelial injury and microvascular occlusion result in Raynaud’s phenomenon, finger ischemia, pulmonary hypertension, and scleroderma renal crisis. Scleroderma itself is a rare disease with an incidence ranging from 0.1 to 14 per 100,000 people in the general population. Cerebral involvement is not considered a common manifestation of systemic sclerosis, although studies have shown that the brain can be involved. Therefore, to deepen the understanding of this disease, we herein report a case of cerebral infarction associated with systemic sclerosis.

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.

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