228 Cardiac damage in the advanced phase of systemic sclerosis evolves differently in diffuse and limited disease forms: an echocardiographic study

2006 ◽  
Vol 5 (1) ◽  
pp. 45-45
Author(s):  
I MOYSSAKIS ◽  
E GIALAFOS ◽  
V VASILIOU ◽  
M DASKALAKI ◽  
D PAPADOPOULOS ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Limited Disease ◽  
Cardiac Damage ◽  
Advanced Phase ◽  
Echocardiographic Study

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.

OP0092 The Arrhythmic Burden in Systemic Sclerosis: Ventricular Ectopic Beat Rate Correlates with Cardiac Damage and Bad Prognosis

2014 ◽  
Vol 73 (Suppl 2) ◽  
pp. 96.1-96
Author(s):  
G. De Luca ◽  
S. Bosello ◽  
F. Parisi ◽  
G. Berardi ◽  
M. Rucco ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Ectopic Beat ◽  
Cardiac Damage ◽  
Ventricular Ectopic Beat ◽  
Beat Rate

Soluble P selectin in systemic sclerosis: relationship with von Willebrand factor, autoantibodies and diffuse or localised/limited disease

2003 ◽  
Vol 109 (4) ◽  
pp. 203-206 ◽  
Author(s):  
A.D. Blann ◽  
J. Constans ◽  
P. Carpentier ◽  
M. Renard ◽  
B. Satger ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Von Willebrand Factor ◽  
Limited Disease ◽  
Soluble P ◽  
Von Willebrand ◽  
Willebrand Factor

scholarly journals AB0093 CLINICAL AND SEROLOGICAL CHARACTERISTICS OF SYSTEMIC SCLEROSIS: EXPERIENCE FROM A TERTIARY CARE CENTER IN PAKISTAN

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1075.2-1076
Author(s):  
S. Asif ◽  
M. Haroon ◽  
A. Khan ◽  
M. Faiq
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Memorial Hospital ◽  
Limited Disease ◽  
Raynaud Phenomenon ◽  
Female Ratio ◽  
Diffuse Disease ◽  
Gi Symptoms

Background:Systemic sclerosis (SS) is less studied Connective tissue disease in our population. It is characterized by different manifestations which if left undiagnosed and untreated lead to serious complications. The hallmark of this disease is fibrosis of various organs including skin and also involving pulmonary, gastrointestinal and cardiovascular system. We aim to evaluate the clinical and serological characteristics of SS in our population. In addition, we evaluated the prevalence of Fibromyalgia and Depression in patients with SS.Objectives:We aimed to determine Clinical and Serological Characteristics of Systemic Sclerosis visiting our department of Rheumatology, Fatima Memorial hospital, Lahore.Methods:It is a cross-sectional study of 38 patients visiting outdoor and indoor in Rheumatology department of Fatima Memorial Hospital from September 2019 to January 2020. All systemic sclerosis patients with age 16 years or above were included in this study. After taking informed consent, we filled the performas to record all the required information. We evaluated the clinical, serological and radiological features of Systemic Sclerosis.Results:A total of 38 patients were included in the study with mean age was 34.47 ± 1.53 years. Female gender comprised of 32(84.2%), with male to female ratio of 1:5.3. Thirty-four (89.5%) had developed Raynaud phenomenon and sclerodactyly was found in 34(89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Skin tightness proximal to elbow was present in 9 (23.7%). Microstomia (decreased mouth opening) was present in 32(84.2%) patients. Twenty-eight (73.7%) patients had arthritis.Respiratory symptoms comprised of shortness of breath in 36(94.7%), palpitation in 24 (63.2%) and chest pain in 7(18.4%). Gastrointestinal (GI) symptoms comprised of regurgitation in 31 (81.5%) and dysphagia in 14 (36.8%). Interstitial lung disease was present in 30 (78.9%) patients, with higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients, with significantly higher prevalence in diffuse disease (57.1%), that was secondary to interstitial lung disease and in limited disease it was found in (11.8%) (p<0.01). Thirty (78.9%) patients were found to have restricted disease on pulmonary function tests.Obstetric history showed a higher prevalence of primary infertility in at least 6 (15.8%) patients, with significantly higher prevalence in limited systemic sclerosis disease as compared to diffuse disease (23.5% vs 9.5%, and p=0.05). Fibromyalgia diagnosed as per ACR criteria was present in 7 (18.4%) patients, and depression assessed by Hospital Anxiety and depression (HADS) score was present in 10 (26.3%) patients.Anti nucleic acid antibody (ANA) was found positive in 30 (78.9%) patients. Anti Scl-70 antibodies were in 24 (63.2%) patients, with significant association with diffuse disease (85% vs 35.3% and p<0.01), while anti centromere antibodies were present in 20 (52.6%) patients; significantly higher in limited disease (94.2% vs 19.0%, and p<0.01).Conclusion:Scleroderma is a very important, autoimmune multisystem disease. It has female preponderence. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of variable course and disease severity. Interstitial lung disease and pulmonary hypertension were the most important complication found in our patients which has poor prognosis. So, It is imperative to early diagnose and treat the disease manifesations to prevent future complications.Keywords:Scleroderma, Systemic sclerosis.Disclosure of interest:Sadia Asif: None declared, Muhammad Haroon: None declared, Dr Asadullah Khan: None declared, Dr. Muhammad Faiq: None declareDOI: 10.1136/annrheumdis-2021-eular.1635

Clinical Correlates of CENP-A and CENP-B Antibodies in a Large Cohort of Patients with Systemic Sclerosis

2012 ◽  
Vol 39 (4) ◽  
pp. 787-794 ◽  
Author(s):  
MARIE HUDSON ◽  
MICHAEL MAHLER ◽  
JANET POPE ◽  
DANIEL YOU ◽  
SOLENE TATIBOUET ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Indirect Immunofluorescence ◽  
Disease Onset ◽  
Scleroderma Renal Crisis ◽  
Limited Disease ◽  
Anticentromere Antibody ◽  
Multicenter Cohort Study ◽  
Inflammatory Myositis ◽  
Diffuse Disease ◽  
Multicenter Cohort

Objective.To study the clinical phenotypes of centromeric proteins (CENP)-A- and CENP-B-positive patients with systemic sclerosis (SSc) and to compare them to anticentromere antibody (ACA)-positive and negative SSc patients.Methods.Sera samples were collected from 802 patients with SSc enrolled in a multicenter cohort study. Antibodies to CENP-A and B were detected by ELISA, and ACA by indirect immunofluorescence. Associations with clinical and other serological manifestations of SSc were investigated.Results.CENP-A antibodies were detected in 276 (34%), CENP-B in 286 (36%), and ACA in 279 (35%) patients. Patients having ACA, CENP-A, and/or CENP-B resembled each other and differed from the remainder of the cohort in the following respects: older chronologically and at disease onset; more commonly women; more likely to have limited disease and lower skin scores; less likely to have finger ulcers, digital tuft resorption, or finger contractures; more likely to have pulmonary hypertension; less likely to have interstitial lung disease, scleroderma renal crisis, inflammatory arthritis, and inflammatory myositis; and having lower overall disease severity. CENP-A and/or B status was predictive of the extent of skin involvement over time. Patients with limited disease who were CENP-A-negative at baseline were more likely to progress to diffuse disease compared to CENP-A-positive patients (OR 2.55, 95% CI 1.37, 4.85, p = 0.004).Conclusion.Clinical immunology laboratories are increasingly using high-throughput ELISA tests for CENP antibodies, with or without ACA detected by indirect immunofluorescence. The phenotype of CENP-A and/or B-positive patients is generally similar to that associated with ACA.

scholarly journals Clinical and serological characteristics of systemic sclerosis: Experience of a tertiary care center in Pakistan

2021 ◽  
Vol 36 (4) ◽  
pp. 587-594
Author(s):  
Sadia Asif ◽  
Asadullah Khan ◽  
Muhammad Faiq ◽  
Zia Ud Din ◽  
Sarmad Zahoor ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Function Tests ◽  
Care Center ◽  
Tertiary Care ◽  
Disease Onset ◽  
Clinical Feature ◽  
Digital Ulcers ◽  
Limited Disease ◽  
Raynaud Phenomenon ◽  
Diffuse Disease

Objectives: This study aims to evaluate the clinical and serological characteristics of systemic sclerosis (SSc) in Pakistani population. Patients and methods: This prospective, cross-sectional study included a total of 38 patients (6 males, 32 females; mean age: 34.5±1.5 years; range, 16 to 60 years) with SSc who were admitted to our rheumatology clinic between November 2019 and January 2020. We evaluated the clinical, serological, and radiological features of SSc patients. Results: Thirty-four (89.5%) patients developed Raynaud phenomenon at the time of disease onset, while sclerodactyly was found in 34 (89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Interstitial lung disease was present in 30 (78.9%) patients with a higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients with a significantly higher prevalence in diffuse disease (57.1%) than limited disease (11.8%) (p<0.01). Thirty (78.9%) patients had impaired pulmonary function tests. Fibromyalgia was present in seven (18.4%) patients, and depression was present in 10 (26.3%) patients. Antinuclear antibody (ANA) was positive in 30 (78.9%) patients. Anti-Scl-70 antibodies were present in 24 (63.2%) patients with a significant association with diffuse disease (85% vs. 35.3%, respectively; p<0.01). The anti-centromere antibodies (ACA) were present in 20 (52.6%) patients with a significantly higher rate in limited disease (94.2% vs. 19.0%, respectively; p<0.01). Conclusion: Scleroderma has a female preponderance. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of a variable course and disease severity.

scholarly journals Lower Extremity Ulcers in Systemic Sclerosis: Features and Response to Therapy

2010 ◽  
Vol 2010 ◽  
pp. 1-8 ◽  
Author(s):  
Victoria K. Shanmugam ◽  
Patricia Price ◽  
Christopher E. Attinger ◽  
Virginia D. Steen
Keyword(s):  
Systemic Sclerosis ◽  
Lower Extremity ◽  
Antiphospholipid Antibodies ◽  
Disease Duration ◽  
Response To Therapy ◽  
Antiphospholipid Antibody ◽  
Limited Disease ◽  
Biopsy Specimens ◽  
Diffuse Scleroderma ◽  
Antibody Screen

Nondigital lower extremity ulcers are a difficult to treat complication of scleroderma, and a significant cause of morbidity. The purpose of this study was to evaluate the prevalence of nondigital lower extremity ulcers in scleroderma and describe the associations with autoantibodies and genetic prothrombotic states. A cohort of 249 consecutive scleroderma patients seen in the Georgetown University Hosptial Division of Rheumatology was evaluated, 10 of whom had active ulcers, giving a prevalence of 4.0%. Patients with diffuse scleroderma had shorter disease duration at the time of ulcer development (mean 4.05 years±0.05) compared to those with limited disease (mean 22.83 years±5.612,Pvalue .0078). Ulcers were bilateral in 70%. In the 10 patients with ulcers, antiphospholipid antibodies were positive in 50%, and genetic prothrombotic screen was positive in 70% which is higher than expected based on prevalence reports from the general scleroderma population. Of patients with biopsy specimens available(n=5), fibrin occlusive vasculopathy was seen in 100%, and all of these patients had either positive antiphospholipid antibody screen, or positive genetic prothrombotic profile. We recommend screening scleroderma patients with lower extremity ulcers for the presence of anti-phospholipid antibodies and genetic prothrombotic states.

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