scholarly journals AB0574 BENEFITS OF ILOPROST IN LONG – TERM STABILIZATION OR IMPROVEMENT IN NEOANGIOGENESIS IN SYSTEMIC SCLEROSIS: A 15 YEARS OBSERVATION COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1584.2-1584
Author(s):  
P. Faggioli ◽  
L. Castelnovo ◽  
A. Tamburello ◽  
A. Laria ◽  
A. M. Lurati ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Continuous Treatment ◽  
Digital Ulcers ◽  
Efficacy And Safety ◽  
Raynaud Phenomenon ◽  
Serious Adverse Events ◽  
Microvascular Damage ◽  
Skin Ulcers

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

scholarly journals OP0270 LONG-TERM EFFICACY AND SAFETY OF BOSENTAN IN PATIENTS WITH DIGITAL ULCERS RELATED TO SYSTEMIC SCLEROSIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 164-164
Author(s):  
N. Cristina ◽  
L. Groseanu ◽  
F. Berghea ◽  
A. Balanescu ◽  
V. Bojinca ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Control Group ◽  
Digital Ulcers ◽  
Efficacy And Safety ◽  
Term Efficacy ◽  
Elevated Liver Enzymes ◽  
Significant Difference ◽  
The Mean

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

scholarly journals Efficacy and Safety of Long-Term Low-Dose Clarithromycin in Patients With Refractory Chronic Sinusitis After Endoscopic Sinus Surgery: A Prospective Clinical Trial

2021 ◽  
pp. 014556132110320
Author(s):  
Han Chen ◽  
Bing Zhou ◽  
Qian Huang ◽  
Cheng Li ◽  
Yubin Wu ◽  
...  
Keyword(s):  
Oral Administration ◽  
Nasal Cavity ◽  
Endoscopic Sinus Surgery ◽  
Low Dose ◽  
Continuous Treatment ◽  
Sinus Surgery ◽  
Efficacy And Safety ◽  
Endoscopic Findings ◽  
Before And After

Objective: To observe the efficacy and safety of postoperative long-term low-dose oral administration of clarithromycin in patients with refractory chronic rhinosinusitis (RCRS), to explore the characteristics of postoperative microbiota in the nasal cavity in patients with RCRS, and to compare the differences and changes in microbiota in the nasal cavity before and after medication. Methods: This was a prospective, self-controlled study. Eighteen patients with RCRS who had persistent symptoms after endoscopic sinus surgery and standard therapy with normal immunoglobulin E and eosinophil level were included. Low dose (250 mg, once daily) clarithromycin was orally administrated for 12 weeks. Symptom severity and endoscopic findings were evaluated before, after 4 weeks, and 12 weeks of treatment, and nasal cavity microbiota was analyzed simultaneously. Results: A total of 18 patients with RCRS were enrolled and 17 patients completed the study. Four weeks after oral administration of clarithromycin, significant improvement was observed in subjective symptoms including nasal congestion, rhinorrhea, postnasal drip, and general discomfort, as well as endoscopic findings including general surgical cavity condition, rhinedema, and rhinorrhea ( P < .05). After continuous treatment to the 12th week, symptoms showed significant improvement compared with baseline, and endoscopic score showed significant improvement compared with both baseline and 4 weeks after treatment. Analysis of middle nasal meatus flora revealed a significant decrease of Streptococcus pneumoniae after 12 weeks of clarithromycin treatment ( P < .05), while the richness, composition, and diversity were similar before and after treatment. Patients enrolled experienced no adverse drug reaction or allergic reaction, nor clinical significant liver function impairment observed. Conclusion: Postoperative low-dose long-term oral administration of clarithromycin in patients with RCRS can improve the clinical symptoms and facilitate the mucosal epithelialization, with good tolerance and safety. The efficacy of clarithromycin in patients with RCRS may be related to its regulatory effect on nasal cavity microbiota.

scholarly journals Serum klotho concentrations inversely correlate with the severity of nailfold capillaroscopic patterns in patients with systemic sclerosis

Reumatismo ◽  
2019 ◽  
Vol 71 (1) ◽  
pp. 19-23
Author(s):  
R. Talotta ◽  
F. Rigamonti ◽  
T. Letizia ◽  
S. Bongiovanni ◽  
M.C. Ditto ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Inverse Correlation ◽  
Enzyme Linked Immunosorbent Assay ◽  
Digital Ulcers ◽  
Concomitant Treatment ◽  
Microvascular Damage ◽  
Vascular Integrity ◽  
Median Serum ◽  
Concomitant Medications ◽  
Scleroderma Pattern

Klotho is a transmembrane and soluble glycoprotein that governs vascular integrity. Previous studies have demonstrated reduced serum klotho concentrations in patients with systemic sclerosis (SSc), and it is known that klotho deficiency can impair the healing of digital ulcers related to microvessel damage. The aim of this study was to evaluate the association between serum klotho levels and nailfold capillaroscopic abnormalities in SSc patients. We retrospectively enrolled 54 consecutive patients with SSc diagnosed on the basis of the 2013 EULAR/ACR criteria [11 with diffuse SSc; 47 females; median age 68.0 years (IQ 18); median disease duration 11.0 years (IQ 7)]. Serum klotho concentrations were determined by means of an enzyme-linked immunosorbent assay. On the basis of the 2000 classification of Cutolo et al., 14 patients had normal nailfold capillaroscopic findings, 8 had an early scleroderma pattern, 21 an active scleroderma pattern, and 11 a late scleroderma pattern. The median serum klotho concentration was 0.29 ng/mL (IQ 1). Regression analysis of variation showed an inverse correlation between serum klotho concentrations and the severity of the capillaroscopic pattern (p=0.02; t -2.2284), which was not influenced by concomitant treatment. Logistic regression did not reveal any significant association between the risk of developing digital ulcers and nailfold capillaroscopic patterns, serum klotho levels, or concomitant medications. The presence of avascular areas significantly correlated with calcinosis (p=0.006). In line with previous studies, our findings confirm that klotho plays a role in preventing microvascular damage detected with nailfold capillaroscopy.

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 ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Long Term Results ◽  
Digital Ulcers

Digital ulcers in systemic sclerosis: role of flow-mediated dilatation and capillaroscopy as risk assessment tools

2015 ◽  
Vol 25 (5) ◽  
pp. 444-451 ◽  
Author(s):  
Ivone Silva ◽  
Tiago Loureiro ◽  
Andreia Teixeira ◽  
Isabel Almeida ◽  
Armando Mansilha ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Systemic Sclerosis ◽  
Assessment Tools ◽  
Digital Ulcers ◽  
Risk Assessment Tools ◽  
Flow Mediated Dilatation

scholarly journals Microvascular damage evaluation in systemic sclerosis: the role of nailfold videocapillaroscopy and laser techniques

Reumatismo ◽  
2017 ◽  
Vol 69 (4) ◽  
pp. 147 ◽  
Author(s):  
B. Ruaro ◽  
A. Sulli ◽  
V. Smith ◽  
C. Pizzorni ◽  
S. Paolino ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Peripheral Blood ◽  
Imaging Technique ◽  
Blood Perfusion ◽  
Clinical Aspects ◽  
Damage Evaluation ◽  
Nailfold Videocapillaroscopy ◽  
Microvascular Damage ◽  
Laser Technologies

Microvascular damage and a decrease in peripheral blood perfusion are typical features of systemic sclerosis (SSc) with serious clinical implications, not only for a very early diagnosis, but also for disease progression. Nailfold videocapillaroscopy is a validated and safe imaging technique able to detect peripheral capillary morphology, as well as to classify and to score any nailfold abnormalities into different microangiopathy patterns. Capillaroscopic analysis is now included in the ACR/EULAR classification criteria for SSc. The decrease in peripheral blood perfusion is usually associated with microvascular damage in SSc, which may be studied by different methods. Several of these make use of safe laser technologies. This paper focuses on these new clinical aspects to assess SSc microvascular impairment.

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