scholarly journals Impact of COVID-19 on outpatient therapy with iloprost for Systemic Sclerosis digital ulcers

2020 ◽  
Author(s):  
Francesca Crisafulli ◽  
Maria-Grazia Lazzaroni ◽  
Stefania Zingarelli ◽  
Mara Rossi ◽  
Angela Tincani ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Assessment ◽  
Similar Rate ◽  
Infusion Therapy ◽  
Digital Ulcers ◽  
Outpatient Therapy ◽  
Therapy Discontinuation ◽  
The Individual ◽  
Iloprost Infusion ◽  
The Impact

Abstract Introduction and objectivesThe outbreak of COVID-19 epidemic imposed temporary changes in the management of patients with chronic diseases. We analyzed the impact of COVID-19 epidemic in patients with Systemic Sclerosis (SSc) receiving intravenous iloprost infusions for digital ulcers (DU) treatment.MethodsDuring the epidemic, iloprost infusion therapy in our Hospital was guaranteed; patients were regularly contacted by telephone before the scheduled infusions. DU were evaluated with DUCAS (DU Clinical Assessment Score).ResultsBetween 20th February and 31st May 2020, 47/64 SSc patients did not receive at least one of the scheduled infusions, for patients fear and/or logistical difficulties (43 cases), COVID-19 (2), or other intercurrent diseases (2). At the last evaluation before 20th February DUCAS was not different between patients who stopped the therapy and those who continued it. The 2 groups had similar rate of new DU during the emergency period, but DUCAS slightly increased during therapy discontinuation, decreasing after resuming it. After COVID-19, one patient underwent a fingertip sub-amputation, and an 85-year-old male with multiple comorbidities died for complications related to the infection.ConclusionsMost SSc patients briefly discontinued iloprost therapy, without increase of new DU number, but with a slight increase of their severity. The regular telephonic contact helped the management that, although not optimal, was adapted to the needs of the individual patient during this emergency period. DUCAS proved to be a useful tool for rheumatologists. COVID-19 had serious consequences in the two patients who contracted it.

scholarly journals Digital Ulcers as Presentation of Carpal Tunnel Syndrome

2020 ◽  
Vol 77 (4) ◽  
pp. 353-356
Author(s):  
T. F. Gomes ◽  
K Kieselova ◽  
F. Santiago ◽  
A. Daniel ◽  
M. Henrique
Keyword(s):  
Systemic Sclerosis ◽  
Carpal Tunnel Syndrome ◽  
Carpal Tunnel ◽  
Clinical Assessment ◽  
Gold Standard ◽  
Digital Ulcers ◽  
Tunnel Syndrome ◽  
Sensory Changes ◽  
Hand Radiography

Ulcerating and mutilating variant of carpal tunnel syndrome occurs in longstanding cases of untreated disease. Pa- tients present with painless ulcers of the second and third fingers, accompanied with other cutaneous and sensory changes. These patients are often misdiagnosed as having a Raynaud disease or systemic sclerosis. Clinical assessment is the gold standard for the diagnosis of carpal tunnel syndrome, but hand radiography and electromyography help supporting the diagnosis. The authors present two cases of this ulcerating variant of carpal tunnel syndrome.

scholarly journals THU0340 DURATION AND SYSTEMIC SCLEROSIS SUBTYPE ARE ASSOCIATED WITH DIFFERENT GUT MICROBIOME PROFILES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 401.2-401
Author(s):  
Y. Braun-Moscovici ◽  
S. Ben Simon ◽  
K. Dolnikov ◽  
S. Giryes ◽  
D. Markovits ◽  
...  
Keyword(s):  
Species Richness ◽  
Systemic Sclerosis ◽  
Gut Microbiota ◽  
Disease Duration ◽  
Alpha Diversity ◽  
Rrna Gene ◽  
Digital Ulcers ◽  
Diffuse Disease ◽  
Probiotic Treatment ◽  
The Impact

Background:A growing body of evidence suggests that the gut microbiota plays a significant role in the development of autoimmune diseases. Altered microbiota composition was associated with gastrointestinal and extraintestinal features in systemic sclerosis (SSc) patients.Objectives:To look for differences in gut microbiota between SSc patients regarding disease duration, disease subset and occurrence of digital ulcers (DU).Methods:SSc patients seen at our center were recruited in a prospective study. The exclusion criteria included antibiotic or probiotic treatment during the month prior to recruitment, recent hospitalization, BMI>30, diabetes mellitus or concomitant inflammatory bowel disease. Fecal samples were processed and 16S rRNA gene sequences were analyzed using the QIIME2 packageWeighted (quantitative) and unweighted (qualitative) UniFrac distances, alpha diversity for richness and homogeneity, taxa plots for species and phyla and ANCOM analyses were performed.Results:During July 2018-May 2019, 26 SSc patients (mean age [SD] 53[12.7] years) and disease duration 8.8 [7.1] years) fulfilled the criteria and were willing to participate in the study. Thirteen patients had diffuse SSc, 16 patients had active DU, 8 patients had Raynaud’s phenomenon only without DU, 2 patients had past DU. The microbiota was significantly more similar between patients without active DU compared to those with active DU (P=0.024), but species richness did not differ. Patients with SSc duration less than 6 years had significantly different microbiota compared to long-lasting SSc (unweighted PCoA – q=0.031). Significant variations concerning quantitative and qualitative UniFrac distances (q=0.063, q=0.005) and species richness (q=0.009) were found among patients with diffuse compared to limited SSc. Limited SSc was associated with greater species richness. Taxa plot analysis revealed higher relative abundance of Firmicutes in diffuse disease and of Actinobacteria and Bacteroidetes in limited SSc.Conclusion:Disease duration, disease subset and active DU were associated with shifts in the microbiome of SSc patients. The impact of these changes on disease progression needs further elucidation.Figure:Disclosure of Interests:Yolanda Braun-Moscovici: None declared, Shira Ben Simon: None declared, Katya Dolnikov: None declared, Sami Giryes: None declared, Doron Markovits: None declared, Yonit Tavor: None declared, Kohava Toledano: None declared, Alexandra Balbir-Gurman Consultant of: Novartis, Omry Koren: None declared

Impact of COVID-19 on outpatient therapy with iloprost for systemic sclerosis digital ulcers

2020 ◽  
pp. 239719832095229
Author(s):  
Francesca Crisafulli ◽  
Maria-Grazia Lazzaroni ◽  
Stefania Zingarelli ◽  
Mara Rossi ◽  
Angela Tincani ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Digital Ulcers ◽  
Outpatient Therapy

scholarly journals Impact of Covid-19 on clinical care and lived experience of systemic sclerosis: An international survey from EURORDIS-Rare Diseases Europe

2021 ◽  
pp. 239719832199992
Author(s):  
Michael Hughes ◽  
John D Pauling ◽  
Andrew Moore ◽  
Jennifer Jones
Keyword(s):  
Systemic Sclerosis ◽  
Lived Experience ◽  
Online Survey ◽  
Care Delivery ◽  
Clinical Care ◽  
Immunosuppressive Treatment ◽  
Well Being ◽  
Personal Threat ◽  
The Individual ◽  
The Impact

Introduction: Outcomes related to Covid-19 in systemic sclerosis patients could be influenced by internal organ involvement and/or immunosuppressive treatment, leading to efforts to shield patients from Covid-19 transmission. We examined the impact of Covid-19 on the lived experience of systemic sclerosis with regards to other aspects of daily living including occupation and emotional well-being. Method: Individuals with systemic sclerosis or relatives/carers participated in an online survey, disseminated through international patient associations and social media pages, designed to examine the impact of Covid-19 on living with a rare disease. Results: Responses from 121 individuals (98% were patients with systemic sclerosis) from 14 countries were evaluable. Covid-19 was considered a probable/definite personal threat (93%) or threat for the individual they care for (100%). Approximately two-thirds of responders reported either cancellation or postponement/delay to appointments, diagnostic tests, medical therapies at home (e.g. infusions), surgery or transplant, psychiatry follow-up or rehabilitation services. Twenty-six percent reported at least one systemic sclerosis medicine/treatment had been unavailable, and 6% had to either stop taking usual medications or use an alternative. Most reported online consultations/telemedicine via phone (88%) and online (96%) as being ‘fairly’ or ‘very’ useful. Respondents reported tensions among family members (45%) and difficulty overcoming problems (48%). Restrictions on movement left around two-thirds feeling isolated (61%), unhappy and/or depressed (64%), although the majority (85%) reported a strengthening of the family unit. Conclusion: Covid-19 has resulted in significant impact on the clinical-care and emotional well-being of systemic sclerosis patients. Changes to clinical care delivery have been well-received by patients including telemedicine consultations.

scholarly journals Preliminary Validation of the Digital Ulcer Clinical Assessment Score in Systemic Sclerosis

2018 ◽  
Vol 46 (6) ◽  
pp. 603-608 ◽  
Author(s):  
Cosimo Bruni ◽  
Tanaka Ngcozana ◽  
Francesca Braschi ◽  
Tiziana Pucci ◽  
Guya Piemonte ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Construct Validity ◽  
Clinical Assessment ◽  
Hand Function ◽  
Assessment Tools ◽  
Face Validity ◽  
Pain Patient ◽  
Digital Ulcers ◽  
Assessment Score ◽  
Patient Reported

Objective.To date, “healed/non-healed” and clinical judgment are the only available assessment tools for digital ulcers (DU) in patients with systemic sclerosis (SSc). The aim of our study is to examine a preliminary composite DU clinical assessment score (DUCAS) for SSc for face, content, and construct validity.Methods.Patients with SSc presenting at least 1 finger DU were enrolled and assessed with the Health Assessment Questionnaire–Disability Index, Cochin scale, visual analog scale (VAS) for DU-related pain, patient global DU status, and global assessment as patient-reported outcomes (PRO), and physician VAS for DU status (phyGDU) as an SSc-DU expert physician/nurse measure. The DUCAS included 7 DU-related variables selected by a committee of SSc DU experts and weighted on a clinical basis. Face validity was examined by consensus and partial construct validity was tested through convergent correlation with other measures of hand function, using Spearman’s correlations. A range of patients with SSc was examined. A linear regression model with backward stepwise analysis was used to determine the relationship of individual variables with the primary clinical parameter, phyGDU.Results.Forty-four patients with SSc (9 males, mean age 55 ± 15 yrs, mean disease duration 9.9 ± 5.8 yrs) were enrolled in the study. Overall DUCAS showed significant positive correlations with all abovementioned PRO (r > 0.4, p < 0.01). When all scores and scales were modeled, only DUCAS significantly predicted phyGDU (r = 0.59, R2 = 0.354, Akaike information criterion = 385.4).Conclusion.Preliminarily, we suggest that the DUCAS may be a new clinical score for SSc-related DU, having face and content validity and convergent/divergent correlations (construct validity). These early data suggest that this score deserves further evaluation.

scholarly journals Ischemic Digital Ulcers Affect Hand Disability and Pain in Systemic Sclerosis

2014 ◽  
Vol 41 (7) ◽  
pp. 1317-1323 ◽  
Author(s):  
Luc Mouthon ◽  
Patrick H. Carpentier ◽  
Catherine Lok ◽  
Pierre Clerson ◽  
Virginie Gressin ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Hand Function ◽  
Time Interval ◽  
Digital Ulcers ◽  
Raynaud Phenomenon ◽  
Hand Disability ◽  
The Mean ◽  
Noninterventional Study ◽  
The Impact ◽  
Mean Time

Objective.Ischemic digital ulcers (DU) are frequent and severe complications of systemic sclerosis (SSc). The purpose of our study was to assess the effect of DU on hand disability and pain in patients with SSc.Methods.The Evaluation of the Impact of Recurrent Ischemic DU on Hand Disability in Patients with SSc (ECLIPSE) is a prospective, multicenter, noninterventional study with a 2-year followup. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan therapy were included between October 2009 and March 2011. This cohort is described at the time of inclusion.Results.There were 190 patients (132 females) from 53 centers. Mean age ± SD was 43 ± 15 years at SSc diagnosis and 53 ± 15 years at inclusion. In 105 patients (56.2%), DU were the first non-Raynaud symptoms of SSc. The mean time interval between the occurrence of Raynaud phenomenon and the first DU episode was 6.6 ± 9.1 years. The mean numbers of active DU and fingers affected per patient for both hands were 2.3 ± 1.8 and 2.2 ± 1.6, respectively. Presence of active DU at inclusion was significantly associated with pain and impaired hand function: Visual Analog Scale for pain (0 to 10) was 6.2 ± 2.6 versus 2.5 ± 2.4 (p < 0.0001) and Cochin Hand Function Scale for hand disability (0 to 90) was 38 ± 20 versus 25 ± 19 (p < 0.0001), respectively.Conclusion.DU represent a major sign of SSc, often affecting multiple fingers and both hands. They are significantly associated with pain and hand disability.

Iloprost infusion through elastomeric pump in the treatment of Raynaud’s phenomenon and digital ulcers

2018 ◽  
Vol 4 (1) ◽  
pp. NP1-NP4
Author(s):  
Maria Helena Fartura Braga Temido ◽  
Manuel Gomes ◽  
Francisco Parente ◽  
Lèlita Santos
Keyword(s):  
Systemic Sclerosis ◽  
Case Reports ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Vascular Involvement ◽  
Systemic Autoimmune Disease ◽  
Portable Devices ◽  
Digital Ulcers ◽  
Critical Ischaemia ◽  
Iloprost Infusion

Introduction: Systemic sclerosis is a systemic autoimmune disease characterized by microangiopathy and fibroblast dysfunction resulting in fibrosis of the skin and internal organs. Raynaud’s phenomenon and digital ulcers are the main clinical features of vascular involvement. Treatment with iloprost is recommended to reduce the frequency and severity of Raynaud’s phenomenon attacks and to heal active digital ulcer. Classical forms of treatment require admission to a ward or day-hospital unit to ensure safety during infusion, causing disruption of the patient’s normal daily life and resulting in significant costs with hospitalization. Nowadays, new portable devices, of which the elastomeric pump is an example, are becoming available in order to avoid hospitalization. Case reports: We describe five cases of patients with systemic sclerosis or mixed connective tissue disease and severe Raynaud’s phenomenon/critical ischaemia or active digital ulcers that were successfully treated with iloprost infusion through elastomeric pump without experiencing any side effects. We present our unit’s protocol for ambulatory infusion. Discussion/conclusion: Our case reports and a brief review of literature prove that iloprost infusion through elastomeric pump is safe, easy and well tolerated and might even improve patient compliance with treatment. Meanwhile, it may also decrease the economic burden of hospitalization with these patients.

Impact of digital ulcers on disability and health-related quality of life in systemic sclerosis

2009 ◽  
Vol 69 (01) ◽  
pp. 214-217 ◽  
Author(s):  
L Mouthon ◽  
C Mestre-Stanislas ◽  
A Bérezné ◽  
F Rannou ◽  
P Guilpain ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Hand Function ◽  
Digital Ulcers ◽  
Health Related Quality ◽  
Disability And Health ◽  
Related Quality ◽  
Health Related ◽  
The Impact

Objective:To assess the impact of digital ulcers (DUs) on disability and health-related quality of life (HRQoL) in systemic sclerosis (SSc).Methods:Two hundred and thirteen patients with SSc were evaluated at four annual meetings of a patient society between 2004 and 2007 (n = 177) or during hospital stay (n = 36). HRQoL was assessed by the SF-36, global disability by the health assessment questionnaire (HAQ), hand disability by the Cochin Hand Function Scale (CHFS) and global hand and wrist mobility by the Kapandji index.Results:Sixty-seven patients (31.4%) had at least one DU at the time of evaluation. Patients with DUs showed significantly more pitting scars (p<0.001) and calcinosis (p<0.0001) than others. Patients with DU had significantly greater HAQ (mean (SD) 1.218 (0.723) vs 0.930 (0.717), p = 0.008), CHFS (mean (SD) 27.38 (20.68) vs 16.73 (18.19), p<0.0001) and aesthetic prejudice (mean (SD) 6.1 (2.2) vs 3.9 (2.5), p<0.0001) scores than others. Hand and wrist mobility were significantly diminished in patients with DU (mean (SD) Kapandji score 75.3 (22.8) vs 81.7 (19.2), p<0.0001). The presence of a DU did not significantly alter the physical component but influenced the mental component (mean (SD) 43.38 (12.53) vs 39.58 (9.54), p = 0.026) of the SF36.Conclusion:Patients with SSc with DUs have reduced wrist and hand mobility, increased global and hand disabilities and decreased mental component of HRQoL.

scholarly journals SAT0325 STELLATE BLOCKADE COMBINED TO ILOPROST AS SUPPORTIVE TREATMENT OPTION IMPROVES PAIN AND ISCHAEMIC SYMPTOMS IN PATIENTS WITH SYSTEMIC SCLEROSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1108.2-1108
Author(s):  
F. Höcketstaller ◽  
U. Henkemeier ◽  
M. Zimmermann ◽  
H. Burkhardt ◽  
F. Behrens ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Combined Treatment ◽  
Common Symptom ◽  
Digital Ulcers ◽  
Research Support ◽  
Pronounced Increase ◽  
Capillary Microscopy ◽  
Patient Reported ◽  
The Impact ◽  
Subjective Sensation

Background:Peripheral ischaemia is a common symptom in systemic sclerosis (SSc) patients with risk of development of digital ulcers (DU). For its treatment, intravenous iloprost is the most effective option. Accompanying pain symptoms worsen the ischaemic symptoms, so a combination with anaesthetic procedures may improve ischaemic status and the subjective sensation of raynaud and pain. The aim of this study was to observe the impact of a combined treatment of iloprost with stellate blockade (ILOST) in improvement of ischaemic symptoms compared to iloprost treatment only (ILO).Objectives:To evaluate efficacy of the ILOST treatment on changes in vascularisation and sensation of patients with SSc and indication for vasodilatative treatment with Iloprost.Methods:Twenty SSc-patients with indication for ILO-treatment (prophylactic or due to digital ulcerations (DU)) will be included in a prospective observational study. Patients will be offered to combine ILO with stellate blockade (ILOST). Beside documentation of disease activity characteristics (mRSS, number of DU, capillary microscopy at baseline, after ILO-treatment and at week 12), patients are assessed using fluorescence-optical imaging (FOI) as innovative method for illustration of changes in microvascularisation and patient reported outcomes (DASH, VAS) at week 12.Results:This interims analysis includes the result of the first 11 patients treated. Mean baseline characteristics (age and gender) are well balanced. Iloprost treatment was initiated due to prophylactic treatment to avoid DU in all patients. 100% of the patients in the ILOST-group were diagnosed as limited SSc compared to 60% in the ILO-group (diffuse type with 40%). All patients showed abnormalities in capillary microscopy (ILOST group: 83,3% late pattern 16.7% active pattern; ILO group: 80% late pattern, 20% early pattern). MRSS was low in both groups with 1.8, the disease duration in mean 15.3 years in the ILOST-group compared to 13.2 in the ILO-group, respectively. In both groups, no new DU occurred in the 12-week follow-up. Improvement in VAS pain was reported in 83% of the patients in the ILOST group compared to 60% in the ILO group. DASH improved with a mean of 5.5 points in the ILOST group compared to 3 points in the ILO group. FOI was compared individual at both arms in the ILOST group only. The arm with stellatum blockade showed a pronounced increase of FOI signals of 5% in mean whereas the opposite site showed a decrease of the signal shortly after ILO treatment indicating a pronounced increase of vascularisation in the ILOST treated body site.Conclusion:A new treatment approach to improve acute ischaemic symptoms was tested by combining stellate blockade to iloprost treatment. No new DU occurred up to 12 weeks after treatment in all patients of both groups indicating the relevance of iloprost as effective vascular dilatative therapy in SSc. The additional intervention was well tolerated and asked to repeat. Subjective sensation on pain of the hands as well as DASH was improved in the combined group. FOI showed a relevant increase in vascularisation in the blockade arm compared to the opposite site in which signals decreased indicating a stronger effect of the combined treatment for improvement of vascularisation.Disclosure of Interests:Franziska Höcketstaller Grant/research support from: Rheumazentrum Rhein-Main, Ulf Henkemeier: None declared, Michael Zimmermann: None declared, Harald Burkhardt Grant/research support from: Pfizer, Roche, Abbvie, Consultant of: Sanofi, Pfizer, Roche, Abbvie, Boehringer Ingelheim, UCB, Eli Lilly, Chugai, Bristol Myer Scripps, Janssen, and Novartis, Speakers bureau: Sanofi, Pfizer, Roche, Abbvie, Boehringer Ingelheim, UCB, Eli Lilly, Chugai, Bristol Myer Scripps, Janssen, and Novartis, Frank Behrens Grant/research support from: Pfizer, Janssen, Chugai, Celgene, Lilly and Roche, Consultant of: Pfizer, AbbVie, Sanofi, Lilly, Novartis, Genzyme, Boehringer, Janssen, MSD, Celgene, Roche and Chugai, Ulrich Drott: None declared, Michaela Köhm Grant/research support from: Pfizer, Janssen, BMS, LEO, Consultant of: BMS, Pfizer, Speakers bureau: Pfizer, BMS, Janssen, Novartis

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