Faculty Opinions recommendation of UK Scleroderma Study Group (UKSSG) guidelines on the diagnosis and management of scleroderma renal crisis.

Author(s):  
Martin Aringer
2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1004.1-1004
Author(s):  
D. Xu ◽  
R. Mu

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


2021 ◽  
Vol 16 (S2) ◽  
Author(s):  
Eric Hachulla ◽  
Christian Agard ◽  
Yannick Allanore ◽  
Jerome Avouac ◽  
Brigitte Bader-Meunier ◽  
...  

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.


2007 ◽  
Vol 32 (2) ◽  
pp. 165-167 ◽  
Author(s):  
I. Peyrot ◽  
S. Boulinguez ◽  
A. Sparsa ◽  
Y. Le Meur ◽  
J.-M. Bonnetblanc ◽  
...  

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Eliza F. Chakravarty

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.


2001 ◽  
Vol 7 (2) ◽  
pp. 115-119 ◽  
Author(s):  
Thomas M. Herndon ◽  
Theodore T. Kim ◽  
Bruce E. Goeckeritz ◽  
Lisa K. Moores ◽  
Robert J. Oglesby ◽  
...  

2017 ◽  
Vol 32 (suppl_3) ◽  
pp. iii127-iii128
Author(s):  
Anne-Lyse Langlois ◽  
Véronique Fremeaux-Bacchi ◽  
Luc Mouthon ◽  
André Pruna ◽  
Yahsou Delmas ◽  
...  

2021 ◽  
Vol 8 (03) ◽  
pp. 168-171
Author(s):  
Ajay Chanakya V ◽  
Sreekanth Kotagiri ◽  
Gogineni Tarun Chowdary ◽  
Sriram K.V.V.S.D. ◽  
Ch. Vasu Reddy ◽  
...  

BACKGROUND Benign breast diseases (BBDs) are a common cause of breast problems in up to 30 % of women. The pathogenesis of BBD involves disturbances in the breast physiology. There is a necessity for thorough comprehensive, prompt diagnosis and management of the benign conditions to reduce the physical and psychological illness of the patients who are anxious about the possibility of the disease being a malignant one. METHODS This was a prospective study conducted in the Department of Surgical Oncology, GSL medical college from October 2016 to July 2018. Females presenting with breast lump, confirmed with fine needle aspiration cytology (FNAC), aged between 12 – 55 years and fit for surgery were included in the study. Those who did not submit the informed consent were excluded. Statistical analysis was performed by SPSS version-21 and MS Excel 2013. RESULTS Most (74 %) of the patients were between 20 and 40 years of age, 56 % were diagnosed to be fibroadenoma. In the study group, 82 % (41) participants with BBD were married. The most common presenting complaint in the study group was lump (50 %) in the breast followed by lump associated with pain (28 %). Patients with fibroadenoma mass had well defined features. CONCLUSIONS Lump was the most common clinical presentation followed by lump with pain. Based on the FNAC report, decision is made for management. Comprehensive and prompt preoperative diagnosis and management of the benign conditions is necessary to reduce the physical and psychological burden on the patients who are anxious about the possibility of malignancy. KEYWORDS Breast, Participants, Study, Age


2019 ◽  
Vol 58 (3) ◽  
pp. 441-445 ◽  
Author(s):  
Toshimasa Shimizu ◽  
Naoki Iwamoto ◽  
Momoko Okamoto ◽  
Yushiro Endo ◽  
Sosuke Tsuji ◽  
...  

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