scholarly journals Elucidating the burden of recurrent and chronic digital ulcers in systemic sclerosis: long-term results from the DUO Registry

2015 ◽  
Vol 75 (10) ◽  
pp. 1770-1776 ◽  
Author(s):  
Marco Matucci-Cerinic ◽  
Thomas Krieg ◽  
Loic Guillevin ◽  
Barbara Schwierin ◽  
Daniel Rosenberg ◽  
...  
2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Michał Waszczykowski ◽  
Bożena Dziankowska-Bartkowiak ◽  
Michał Podgórski ◽  
Jarosław Fabiś ◽  
Arleta Waszczykowska

AbstractThe aim of this study was to estimate the long-term results of complex and supervised rehabilitation of the hands in systemic sclerosis (SSc) patients. Fifty-one patients were enrolled in this study: 27 patients (study group) were treated with a 4-week complex, supervised rehabilitation protocol. The control group of 24 patients was prescribed a home exercise program alone. Both groups were evaluated at baseline and after 1-, 3-, 6-, and 12-months of follow-up with the Disability of the Arm, Shoulder and Hand Questionnaire (DAHS) as the primary outcome, pain (VAS—visual analog scale), Cochin Hand Function Scale (CHFS), Health Assessment Questionnaire Disability Index (HAQ-DI), Scleroderma-HAQ (SHAQ), range of motion (d-FTP—delta finger to palm, Kapandji finger opposition test) and hand grip and pinch as the secondary outcomes. Only the study group showed significant improvements in the DASH, VAS, CHFS and SHAQ after 1, 3 and 6 months of follow-up (P = 0.0001). Additionally, moderate correlations between the DASH, CHFS and SHAQ (R = 0.7203; R = 0.6788; P = 0.0001) were found. Complex, supervised rehabilitation improves hand and overall function in SSc patients up to 6 months after the treatment but not in the long term. The regular repetition of this rehabilitation program should be recommended every 3–6 months to maintain better hand and overall function.


1990 ◽  
Vol 13 (2) ◽  
pp. 125-129 ◽  
Author(s):  
L. Guillevin ◽  
Z. Amoura ◽  
Ph. Merviel ◽  
J. Pourrat ◽  
A. Bussel ◽  
...  

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1584.2-1584
Author(s):  
P. Faggioli ◽  
L. Castelnovo ◽  
A. Tamburello ◽  
A. Laria ◽  
A. M. Lurati ◽  
...  

Background:In Systemic Sclerosis (SSc) fibrosis is due to microcirculation damage with capillary necrosis, arteriolar intimal proliferation and local ischemia. loprost (ILO) is used IV for the treatment of severe Raynaud phenomenon (RP) and digital ulcers (DU) in (SSc). We have already described (1) an improvement of peripheral vascularization with ILO, observed after 3 years treatment by capillaroscopy with an increase in the capillary number and mild regression of avascular areas and pericapillar oedemaObjectives:Our aim was to observe capillaroscopic changes in a cohort of 26 patients treated with ILO, once a month (25 – 50 ng each infusion) for an average time of 15 yearsMethods:We evaluated the initial and 2019 capillaroscopic picture of 26 SSc patients (24 W,2 M; median age 63.8Y) in continuous treatment with monthly infusion of ILO from 2004 to today. 6/26 were SCL70 positive;the remainder was positive for anticentromere AbResults:We documented stability of capillaroscopic picture in 62% of patients,an improvement in 19% and a worsening (mainly from early to active pattern) in 19%. Low adherence to therapy was observed among the worsened patients. Out of 8 patients with onset ulcers, only 3 patients still have skin ulcers, all with late stable capillaroscopic picture from onset.We have not documented serious adverse eventsConclusion:Our observations confirm the efficacy and safety of ILO in the treatment of SSc even after many years of treatment, resulting in a stabilization of microvascular damage, independent of disease severity.References:[1]Possible role of iloprost (stable analog of PG12) in promoting neoangiogenesis in systemic sclerosis. Faggioli P, Giani L, Mazzone A. Clin Exp Rheumatol. 2006 Mar-Apr;24(2):220-1. No abstract availableDisclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 164-164
Author(s):  
N. Cristina ◽  
L. Groseanu ◽  
F. Berghea ◽  
A. Balanescu ◽  
V. Bojinca ◽  
...  

Background:Two pivotal studies, RAPIDS-1 and RAPIDS-2 revealed that bosentan reduces the development of new digital ulcers (DUs) in patients with systemic sclerosis (SSc). However data regarding the long-term use of this dual endothelin antagonist receptor in the treatment of DUs is scarce.Objectives:The aim of the present study was to evaluate long term efficacy and safety profile of bosentan in patients with DUs related to SSc.Methods:A prospective observational case-control study, conducted between 2014 and 2020 enrolled 65 SSc patients with ≥1 active DUs at baseline, who received bosentan therapy. Demographic and clinical features, including DUs incidence and patients subjective perception of DU pain and/or Raynaud’s Phenomenon, were collected. Nailfold videocapillaroscopy was performed in all patients.Results:The study included 51 females and 14 males, with a mean age of 52.6 years, 30 with diffuse subset, most of them with late scleroderma pattern (46/65). Number of DUs at baseline was 4.55 (±2.8), median duration of treatment was 25.95 (±19.4) months. Microangiopathy evolution score (MES) was 5.1 (2.19), visual analog scale (VAS) for DU was 77.9, VAS for Raynaud was 73.4.Patients receiving bosentan had clinically significant reduction in the mean number of DU (p<0.001). The effect was most powerful for the first 6 months of treatment, but the improvement was sustained until 24 months’ follow-up, when the mean DU number reached a plateau that was kept until end of study. 6 month and 24 month evaluations also revealed significant decrease in the VAS for DU (p<0.05) and in the VAS for Raynaud (p<0.01). Statistically significant difference was noted between bosentan-treated and the control group with respect to the decrease in the mean number of digital ulcers. (p=0.005).There was a clear trend towards an improvement in MES score, between baseline and the next follow-up assessments (p=0.003). The difference was statistically significant when compared to control group, but only for the first 18 months of treatment (p<0.001).14 patients (28.75%) discontinued bosentan therapy for administrative reasons. The median time among patients who interrupted the treatment was 6.9 months. An accelerated development of new DU was described 6 months after (p=0.02). Following recommencement of bosentan, the mean number of DU has rapidly decreased (p=0.008). There was no significant difference between patients who temporarily discontinued bosentan for 6 or 12 months.Bosentan was stopped due to lack of efficacy in 2 cases and due to side effects in 7 cases: 4 elevated liver enzymes, 1 severe trombocytopenia, 1 dyspneea agravation and low blood pressure.Conclusion:The present data suggest that treatment with endothelin receptor antagonist bosentan was associated with a significant reduction in the mean number of DU in patients with SSc. The beneficial effect of bosentan persisted throughout the study but was most evident in the first 6 months of treatment. Statistical analysis showed a significant improvement of the microangiopathy evolution score from baseline to end of therapy. 14 patients had a high relapse rate due to potential rebound effect, 6 months after bosentan withdrawal.The drug was reintroduced succesfully for 10 (70%) patients with a significant decrease in the number of DU.References:[1]UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis, Rheumatology, Volume 54, Issue 11, November 2015, Pages 2015[2]Groseanu L, Berghea F, Bojinca V, et al AB0779 Long term follow-up of a systemic sclerosis group treated with bosentan, Annals of the Rheumatic Diseases 2018;77:1523-1524.Disclosure of Interests:None declared


2005 ◽  
Vol 173 (4S) ◽  
pp. 116-117
Author(s):  
Hannes Steiner ◽  
Reinhard Peschel ◽  
Tilko Müller ◽  
Christian Gozzi ◽  
Georg C. Bartsch ◽  
...  

VASA ◽  
2011 ◽  
Vol 40 (6) ◽  
pp. 474-481 ◽  
Author(s):  
Radak ◽  
Babic ◽  
Ilijevski ◽  
Jocic ◽  
Aleksic ◽  
...  

Background: To evaluate safety, short and long-term graft patency, clinical success rates, and factors associated with patency, limb salvage and mortality after surgical reconstruction in patients younger than 50 years of age who had undergone unilateral iliac artery bypass surgery. Patients and methods: From January 2000 to January 2010, 65 consecutive reconstructive vascular operations were performed in 22 women and 43 men of age < 50 years with unilateral iliac atherosclerotic lesions and claudication or chronic limb ischemia. All patients were followed at 1, 3, 6, and 12 months after surgery and every 6 months thereafter. Results: There was in-hospital vascular graft thrombosis in four (6.1 %) patients. No in-hospital deaths occurred. Median follow-up was 49.6 ± 33 months. Primary patency rates at 1-, 3-, 5-, and 10-year were 92.2 %, 85.6 %, 73.6 %, and 56.5 %, respectively. Seven patients passed away during follow-up of which four patients due to coronary artery disease, two patients due to cerebrovascular disease and one patient due to malignancy. Limb salvage rate after 1-, 3-, 5-, and 10-year follow-up was 100 %, 100 %, 96.3 %, and 91.2 %, respectively. Cox regression analysis including age, sex, risk factors for vascular disease, indication for treatment, preoperative ABI, lesion length, graft diameter and type of pre-procedural lesion (stenosis/occlusion), showed that only age (beta - 0.281, expected beta 0.755, p = 0.007) and presence of diabetes mellitus during index surgery (beta - 1.292, expected beta 0.275, p = 0.026) were found to be significant predictors of diminishing graft patency during the follow-up. Presence of diabetes mellitus during index surgery (beta - 1.246, expected beta 0.291, p = 0.034) was the only variable predicting mortality. Conclusions: Surgical treatment for unilateral iliac lesions in patients with premature atherosclerosis is a safe procedure with a low operative risk and acceptable long-term results. Diabetes mellitus and age at index surgery are predictive for low graft patency. Presence of diabetes is associated with decreased long-term survival.


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