From Raynaud Phenomenon to Systemic Sclerosis in COVID-19

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Dilia Giuggioli ◽  
Amelia Spinella ◽  
Marco de Pinto ◽  
Mascia Maria Teresa ◽  
Carlo Salvarani
Keyword(s):  
Systemic Sclerosis ◽  
Raynaud Phenomenon

Treatment of Raynaud phenomenon and ischemic ulcers associated to systemic sclerosis with hyperbaric oxygen

2021 ◽  
Author(s):  
María Ahijón-Lana ◽  
Elia Baragaño-Ordóñez ◽  
Raúl Veiga-Cabello ◽  
Carmen de la Cruz-Tapidor ◽  
Patricia E. Carreira
Keyword(s):  
Systemic Sclerosis ◽  
Hyperbaric Oxygen ◽  
Raynaud Phenomenon ◽  
Ischemic Ulcers

scholarly journals Raynaud Phenomenon in Systemic Sclerosis: Does Digital Thermal Monitoring Correlate to Specific Nailfold Videocapillaroscopy Abnormalities?

2020 ◽  
pp. jrheum.191371
Author(s):  
Julie Thomas ◽  
Mislav Radic ◽  
Jordan R. Tucker ◽  
Rebecca Overbury ◽  
Tracy M. Frech
Keyword(s):  
Systemic Sclerosis ◽  
Vascular Function ◽  
Vascular Reactivity ◽  
Clinical Care ◽  
Density Measurement ◽  
Nonparametric Tests ◽  
Reactivity Index ◽  
Thermal Monitoring ◽  
Nailfold Videocapillaroscopy ◽  
Raynaud Phenomenon

Objective Early diagnosis of systemic sclerosis (SSc) is imperative, and Raynaud phenomenon (RP) is an important component of progressive vasculopathy. Nailfold videocapillaroscopy (NVC) is a well-established tool that can quantify structural vascular abnormalities. Digital thermal monitoring (DTM) assesses microvascular functional dysfunction related to thermoregulation. In this study, we investigated the correlation of NVC patterns and DTM variables in patients with SSc. Methods Patients with SSc according to the 2013 American College of Rheumatology/European League Against Rheumatism criteria who consented and enrolled in the clinical care registry had NVC and DTM performed. For NVC, the number of capillaries (density), measurement of apical diameter (dimension), presence or absence of hemorrhages, and number of abnormal shapes were assessed to categorize 3 different qualitative patterns: early, active, and late. For DTM, Doppler ultrasound hyperemic, low frequency, blood velocity of radial artery, and fingertip vascular function were assessed, and a vascular reactivity index (VRI) measurement was automated. Statistical evaluation was performed by nonparametric tests to assess the correlation of NVC and VRI. Results Thirty-one SSc subjects with interpretable NVC and DTM performed on the same day were included in the study. VRI was progressively higher in SSc patients with early, active, and late NVC patterns of microangiopathy (P < 0.0001). There was a significant negative correlation between VRI and microhemorrhages scores (r = –0.363, P = 0.044). Conclusion Our study suggests that more advanced vasculopathy correlates to reduced microvascular function as detected by DTM and more advanced structural abnormalities detected by NVC. NVC and DTM may provide different aspects of vasculopathy quantification and complement each other as investigative tools.

Tako-tsubo-like syndrome in systemic sclerosis: a sign of myocardial Raynaud phenomenon?

2008 ◽  
Vol 67 (6) ◽  
pp. 898-899 ◽  
Author(s):  
D Melchiorre ◽  
P Bernardo ◽  
M L Conforti ◽  
C Comunian ◽  
F Nacci ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Raynaud Phenomenon

scholarly journals AB0615 MORTALITY AND CAUSES OF DEATH AMONG ROMANIAN SYSTEMIC SCLEROSIS PATIENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1603.2-1603
Author(s):  
A. Petcu ◽  
M. M. Tamas ◽  
L. Muntean ◽  
S. P. Simon ◽  
I. Filipescu ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Systemic Sclerosis ◽  
Survival Rate ◽  
Causes Of Death ◽  
Age At Onset ◽  
Male Gender ◽  
Raynaud Phenomenon ◽  
Cardiovascular Involvement ◽  
The Mean

Background:Systemic sclerosis (SSc) is associated with an increased risk of death compared to the general population. Survival in SSc patients has improved significantly over the last 20 years with a decrease in renal involvement as a cause of early death and an increase in death caused by cardiopulmonary involvement (1,2). Causes of death in SSc patients have not been described in a Romanian cohort so far.Objectives:To study the causes of death in patients with SSc prospectively followed-up from 2002 to 2018 in a single tertiary centre from Romania.Methods:The cohort consists of 197 patients who fulfill the American College of Rheumatology /EULAR 2013 criteria for SSc. We examined the data of patients who had died during follow up. Patients were reviewed at least twice a year and the cause of death was classified as SSc-related or nonSSc-related. SSc-related deaths were then attributed to the major organ involved. A univariate Cox proportional hazard (PH) regression was used to examine the association between each variable and mortality. Variables reported in the literature to associate with mortality were considered in the multiple Cox PH regression model.Results:Of 197 SSc patients (87.8% females), 47.7% had diffuse SSc and 52.2% had limited SSc. The mean age at diagnosis was 47 (SD 12) years and mean follow up duration was 6.75 years. There were 41 deaths (20.8%). Survival rate was substantially lower in men (P <0.003). The mean age at the time of death in those with diffuse SSc was lower compared to limited SSc (55.8 years vs 68.7 years). Sixty percent of deaths were SSc-related (pulmonary cause[n=11], cardiac cause[n=6], gastrointestinal involvement [GI, n=3], renal crisis [n=2] and others [n=4]). Deaths no related to SSc were associated with cancer and infections. Age at onset of Raynaud phenomenon [HR 1.05], male gender [HR 3.41], diffuse SSc [HR 0.48], presence of tendon friction rub [HR 4.54], digital ulceration [HR 3], esophagitis [HR 2.07] and cardiovascular involvement [HR 3.68], use of corticosteroids[HR 2.13] and cyclophosphamide [HR 2.02] were associated with poor prognosis in multivariate analysis.Conclusion:In our cohort the main causes of death were lung and cardiovascular involvement. Deaths occurred early after the onset of the disease and the survival rate was significantly reduced among men. Multivariate analysis showed that age at onset of Raynaud phenomenon, male gender, diffuse disease form, presence of tendon friction rub, digital ulceration, esophagitis and cardiovascular history, use of corticosteroids and cyclophosphamide were independently associated with mortality.References:[1]Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010 Oct;69(10):1809–15.[2]Rubio-Rivas M, Royo C, Simeon CP, Corbella X, Fonollosa V. Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum. 2014 Oct;44(2):208–19.Disclosure of Interests:None declared

Paradoxical Reaction of Raynaud Phenomenon following the Repeated Administration of Lloprost in a Patient with Diffuse Cutaneous Systemic Sclerosis

2012 ◽  
Vol 46 (10) ◽  
pp. 1439-1439 ◽  
Author(s):  
Rebeca Iglesias Barreira ◽  
Belén Bardán García ◽  
Mónica Granero López ◽  
Iria Rodríguez Legazpi ◽  
Hortensia Álvarez Díaz ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Repeated Administration ◽  
Vascular Involvement ◽  
Digital Ulcers ◽  
Probability Scale ◽  
Paradoxical Reaction ◽  
Raynaud Phenomenon ◽  
Naranjo Probability Scale ◽  
Case Summary ◽  
Diffuse Cutaneous Systemic Sclerosis

Objective TO report a paradoxical reaction of Raynaud phenomenon following the repeated administration of iloprost in a patient with diffuse cutaneous systemic sclerosis with vascular involvement. Case Summary In January 2006, a 40-year-old male was diagnosed with diffuse cutaneous systemic sclerosis with pulmonary, esophageal, cutaneous, and vascular involvement (Raynaud phenomenon, with digital ulcers on his hands). In December 2008, treatment with iloprost was started due to worsening disease. Nine cycles of iloprost were administered at a rate of 0.5–1 ng/kg/min (6 hours per day, for 5 days every 6–8 weeks); the patient tolerated this treatment well. However, on the fourth day of cycles 10 and 11, the patient developed paradoxical Raynaud phenomenon in the hand with perfusion when the infusion was increased to 1 ng/kg/min, requiring treatment to be stopped. Treatment was continued during cycles 12 and 13 at 0.5 ng/kg/min; the patient tolerated the treatment well, although paradoxical Raynaud phenomenon occurred when the rate of infusion was increased. Discussion Raynaud phenomenon is extremely common in patients with scleroderma, and often is severe. Iloprost has vasodilating, antiplatelet, cytoprotective, and immunomodulating properties, and has been found to be an efficacious alternative to nifedipine for the treatment of Raynaud phenomenon in patients with scleroderma. The Naranjo probability scale indicated that iloprost was the probable cause of the paradoxical Raynaud phenomenon in this patient. Conclusions This case demonstrates a probable relationship between the rate of infusion of iloprost and the paradoxical reaction of Raynaud phenomenon.

scholarly journals Osteonecrosis of carpal bones in systemic sclerosis

2021 ◽  
Vol 30 (3) ◽  
pp. 93-98
Author(s):  
Alexandra Chitac ◽  
◽  
Codrina Ancuta ◽  
◽  
Keyword(s):  
Systemic Sclerosis ◽  
Rare Complication ◽  
Vascular Supply ◽  
Clinical Findings ◽  
Carpal Bones ◽  
Digital Ulcers ◽  
Raynaud Phenomenon ◽  
Vascular Component ◽  
Anatomic Variants ◽  
Mechanical Factors

Background. Systemic sclerosis (SSc) is a rare chronic disease, with unknown aetiology and complex pathogenesis. The hand is often implied in the main clinical findings, being affected primarily by the vascular component (Raynaud phenomenon, digital ulcers). One of the possible complications of the hand vasculopathy is osteonecrosis (ON) of the carpal bones. In these cases, the lunate is the most frequently affected, followed by the scaphoid. In the ON process, not only the vascular supply is important, but the nearby mechanical factors and anatomic variants of the wrist. Our objective was to reveal the most important aspects regarding the ON of the carpal bones in patients with SSc. Methods. A systematic literature review was performed through July 31, 2021 on Pubmed and Cochrane databases. The eligible articles were read in full text and were included in this paper, in the absence of exclusion criteria and after consensus between two reviewers. Results. Ten articles met the inclusion criteria, their main results being described in this review. In all studies, ON of the lunate and the scaphoid is associated with advanced SSc and severe Raynaud phenomenon. Conclusions. In conclusion, carpal ON is a rare complication of SSc, especially of the diffuse type. There is limited data on this condition, its prevalence being difficult to estimate due to the lack of symptoms.

Esophageal Motor Abnormalities in Children and Adolescents With Scleroderma and Mixed Connective Tissue Disease

PEDIATRICS ◽  
1988 ◽  
Vol 82 (1) ◽  
pp. 107-111
Author(s):  
Jonathan A. Flick ◽  
John T. Boyle ◽  
David N. Tuchman ◽  
Balu H. Athreya ◽  
Robert A. Doughty
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Children And Adolescents ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Progressive Systemic Sclerosis ◽  
Sphincter Pressure ◽  
Raynaud Phenomenon ◽  
Linear Scleroderma ◽  
Motor Abnormalities

To determine the frequency and nature of esophageal motor abnormalities in children and adolescents with scleroderma syndromes and mixed connective tissue disease, esophageal manometry was performed on seven patients with progressive systemic sclerosis, four patients with mixed connective tissue disease, and two patients with linear scleroderma. A total of 73% of patients with progressive systemic sclerosis and mixed connective tissue disease had symptoms of esophageal dysfunction. A significant association between the presence of Raynaud phenomenon and esophageal symptoms was noted. Esophageal motor abnormalities were detected in 73% of patients with progressive systemic sclerosis and mixed connective tissue disease; these abnormalities were characterized by decreased lower esophageal sphincter pressure and abnormal peristalsis in the distal two thirds of the esophageal body. They resemble those described among adults with progressive systemic sclerosis and mixed connective tissue disease but were not related to disease duration or to the presence of Raynaud phenomenon. Patients with linear scleroderma did not have esophageal symptoms and demonstrated only nonspecific motor abnormalities that did not worsen during several years of follow-up.

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