scholarly journals Iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis and the quality of life: a new therapeutic protocol

Rheumatology ◽  
2006 ◽  
Vol 45 (8) ◽  
pp. 999-1004 ◽  
Author(s):  
G. Milio ◽  
E. Corrado ◽  
C. Genova ◽  
C. Amato ◽  
F. Raimondi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Therapeutic Protocol

scholarly journals Raynaud’s phenomenon

2019 ◽  
Vol 4 (2) ◽  
pp. 89-101 ◽  
Author(s):  
Ariane L Herrick
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
General Population ◽  
Raynaud’S Phenomenon ◽  
Underlying Disease ◽  
Raynaud's Phenomenon ◽  
Recent Advances ◽  
Primary Raynaud’S Phenomenon ◽  
Critical Ischaemia

Raynaud’s phenomenon can be either primary (idiopathic) or secondary to underlying disease including systemic sclerosis. Primary Raynaud’s phenomenon is very common, affecting approximately 3%–5% of the general population. Although much rarer, systemic sclerosis–related Raynaud’s phenomenon can be particularly severe, progressing to digital ulceration in approximately 50% of patients. Raynaud’s phenomenon can have a major impact on quality of life. This review has a focus on the systemic sclerosis–related Raynaud’s phenomenon (which is the most researched form of Raynaud’s phenomenon and probably the most challenging to treat) and on recent advances. Epidemiology (including transition from ‘isolated’ to systemic sclerosis–related Raynaud’s phenomenon), pathogenesis, diagnosis and assessment are discussed, followed by the treatment of both ‘uncomplicated’ and ‘complicated’ Raynaud’s phenomena (i.e. Raynaud’s phenomenon which has progressed to digital ulceration and/or critical ischaemia). Finally, some of the major challenges for the next 5–10 years are highlighted.

scholarly journals FRI0601-HPR IMPACT OF LIFE STYLE MODIFICATION TECHNIQUE IN SYSTEMIC SCLEROSIS (SSC) PATIENTS: A STUDY BY RHEUMATOLOGY NURSES COUNSELOR

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 907.1-907
Author(s):  
S. Baghel ◽  
R. Thakran ◽  
C. Messi ◽  
V. Yadav ◽  
S. Kapoor ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Life Style ◽  
Lifestyle Modification ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Lifestyle Modifications ◽  
Modification Technique

Background:Systemic sclerosis (SSc) is an autoimmune the disease of the connective tissue that is clinically characterized by the involvement of skin (fibrosis, contractures of the finger joints), microvascular abnormalities (Raynaud’s phenomenon and complications), gastrointestinal involvement (gastroesophageal reflux disease - GERD, the lower GI tract involvement), musculoskeletal involvement (polyarthralgia, muscle disease), and involvement of internal organs (especially lungs, heart, and kidneys). Lifestyle modification techniques could have significant impact on various aspects of the disease including early disease control, increased drug adherence, positive attitude towards life, decreased financial burden of treatment, maintaining mobility and joints range of motion, minimizing or delaying joint contractures and decreased dependency with regular physical therapy. Counselling explaining the benefits of lifestyle modification related to these aspects of daily living may make a major difference in the quality of life of the patients with SSc.Objectives:To assess the benefits of lifestyle modification technique in improving the quality of life in patients with SSc.Methods:Patients with SSc attending the Rheumatology clinic of this institution, willing to participate in the survey, were enrolled in this study. All the information including the follow-up details were recorded in a pre-designed form. Their demographic information (age, gender) and disease characteristics (diagnosis, duration, treatment) were recorded, All the patients were explained the lifestyle modifications and their benefits, reinforced at each follow-up visit using posters (visual), written lifestyle modification techniques (using printed material) to raise their awareness of how to improve several of the above manifestations of SSc.Results:One hundred fifty (n=150) consecutive SSc patients were included in the study. It was observed that with repeated counselling 125 (83.3 %) patients adopted the lifestyle modification technique according to the advice imparted and felt a positive benefit in their daily life. However, 25 (16.6%) could not or did not follow the imparted lifestyle change advice on a regular basis. Those who were able to modify the life-style as counselled showed the following results:80 % were able to avoid exposure to cold by adopting the following measures: Wearing gloves and extra woolen socks, using mittens most of the time, wearing woollen undergarments to keep the central regions of the body region warm. These patients noted 55% decrease in the episodes of Raynaud’s phenomenon.Early evening meals and raising the head-end of the bed: 60% decrease in gastrointestinal symptoms.Regular physiotherapy: 65 % decrease dependency on others; 55% could maintain flexibility with physical exercises.Regular application and rubbing of the skin with lanoline-containing skin moisturizers 60 % improve your skin’s health80% were able to avoid active and passive tobacco use.Conclusion:The lifestyle modification techniques are important to control disease and its complications. Thus, after intense and regular counselling by the specialist rheumatology nurses on the lifestyle modification technique (83.3%) adapted the advised lifestyle modifications. The study showed the important role specialist rheumatology nurses can play in educating patients and helping them improving their quality of life.References:[1] Hudson M, Thombs BD, Steele R. at ell. Canadian Scleroderma Research G. QOL in patients with systemic sclerosis compared to the general population and patients with other chronic conditions. J Rheumatol. 2009;36(4):768–72.Disclosure of Interests:None declared

Raynaud’s phenomenon and digital ischaemia - pharmacologic approach and alternative treatment options

VASA ◽  
2016 ◽  
Vol 45 (3) ◽  
pp. 201-212 ◽  
Author(s):  
Birgit Linnemann ◽  
Matthias Erbe
Keyword(s):  
Quality Of Life ◽  
Angiotensin Receptor Blockers ◽  
Raynaud’S Phenomenon ◽  
Treatment Options ◽  
Raynaud's Phenomenon ◽  
Tissue Loss ◽  
Receptor Antagonists ◽  
Skin Ulcers ◽  
Digital Ischaemia

Abstract. The primary goal of therapy is to reduce the frequency and intensity of Raynaud’s attacks and to minimize the related morbidity rather than to cure the underlying condition. Treatment strategies depend on whether Raynaud’s phenomenon (RP) is primary or secondary. All patients should be instructed about general measures to maintain body warmth and to avoid triggers of RP attacks. Pharmacologic intervention can be useful for patients with severe and frequent RP episodes that impair the patient’s quality of life. Calcium channel blockers are currently the most prescribed and studied medications for this purpose. There has been limited evidence for the efficacy of alpha-1-adrenergic receptor antagonists, angiotensin receptor blockers, topical nitrates or fluoxetine to treat RP. The intravenously administered prostacyclin analogue iloprost can reduce the frequency and severity of RP attacks and is considered a second-line therapy in patients with markedly impaired quality of life, critical digital ischaemia and skin ulcers who are at risk for substantial tissue loss and amputation. Phosphodiesterase inhibitors (e.g., sildenafil) can also improve RP symptoms and ulcer healing whereas endothelin-1 receptor antagonists (e.g., bosentan) are mainly considered treatment options in secondary prevention for patients with digital skin ulcers related to systemic sclerosis. However, their use in clinical practice has been limited by their high cost. Antiplatelet therapy with low-dose aspirin is recommended for all patients who suffer from secondary RP due to ischaemia caused by structural vessel damage. Anticoagulant therapy can be considered during the acute phase of digital ischaemia in patients with suspected vascular occlusive disease attributed to the occurrence of new thromboses. In patients with critical digital ischaemia, consideration should be given to hospitalisation, optimisation of medical treatment in accordance with the underlying disease and evaluation for a secondary, possibly reversible process that is causing or aggravating the clinical symptoms.

scholarly journals Assessment of quality of life in patients with Raynaud’s phenomenon after procedures with physical therapy

2017 ◽  
Vol 7 (3) ◽  
pp. 213-222
Author(s):  
Katarzyna Gacuta ◽  
Mateusz Cybulski ◽  
Jacek Kita ◽  
Elżbieta Krajewska-Kułak
Keyword(s):  
Quality Of Life ◽  
Physical Therapy ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon

scholarly journals Raynaud’s Phenomenon Impact on Quotidian Quality of Life

2020 ◽  
Vol 16 (09) ◽  
pp. 88
Author(s):  
Isobel Taylor ◽  
Ivone Silva ◽  
Susana Barreto ◽  
César Soares ◽  
Joaquim Mendes
Keyword(s):  
Quality Of Life ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
General Analysis ◽  
Daily Activities ◽  
Central Hospital ◽  
Design Specification ◽  
Daily Experience ◽  
Patient Questionnaires

<strong>Objective: </strong>To gain further understanding into the needs of Raynaud’s phenomenon (RP), patient questionnaires were conducted in England, UK 2017 at an SRUK patient conference and Porto, Portugal 2018 at Central Hospital Universitário Porto (CHUP). The questions focused on daily activities and experience. <strong>Method</strong>: Sixty-four participants answered questions on their daily experience with RP, specific and general. Analysis grouped results into topics quantified by number and %.<strong> Results:</strong> Although many tasks trigger RP episodes, some are more problematic than others, e.g. peeling vegetables; for many, the pain (46.9%) is not the worst part, but instead the hinderance in function, inability (73.4%) especially regarding hands and fingers. <strong>Conclusion:</strong> A Design Specification (DS) criteria for RP patients’ hands is formed through the listed activities. The key needs identified for RP patients fingers and hands are maintenance of: Dexterity, Tactility and Comfort whilst cohering to aesthetic needs &amp; wellbeing.

Ascorbic Acid does not Improve Endothelium-Dependent Flow-mediated Dilatation of the Brachial Artery in Patients with Raynaud’s Phenomenon Secondary to Systemic Sclerosis

2003 ◽  
Vol 73 (1) ◽  
pp. 3-7 ◽  
Author(s):  
M. E. Mavrikakis ◽  
J. P. Lekakis ◽  
M. Papamichael ◽  
K. S. Stamatelopoulos ◽  
Ch. C. Kostopoulos ◽  
...  
Keyword(s):  
Ascorbic Acid ◽  
Smooth Muscle ◽  
Systemic Sclerosis ◽  
Endothelial Dysfunction ◽  
Brachial Artery ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Water Soluble ◽  
Placebo Administration ◽  
Flow Mediated Dilatation

Previous studies have shown that patients with Raynaud’s phenomenon secondary to systemic sclerosis present abnormal endothelial function; the mechanisms responsible for the endothelial dysfunction are unknown but increased vascular oxidative stress could be a possible cause. The hypothesis that a potent water-soluble antioxidant can reverse endothelial dysfunction in these patients was tested in the present study. We examined 11 female patients with Raynaud’s phenomenon secondary to systemic sclerosis and ten healthy control women by ultrasound imaging of the brachial artery to assess flow-mediated (endothelium-dependent) and nitrate-induced (endothelium-independent) vasodilatation. Flow-mediated dilatation and nitrate-induced dilatation were significantly reduced in patients with Raynaud’s phenomenon, indicating abnormal endothelial and smooth muscle cell function. Patients with Raynaud’s phenomenon entered a double-blind, randomized, crossover placebo-controlled trial and received orally 2 g of ascorbic acid or placebo; vascular studies were repeated two hours after ascorbic acid or placebo administration. Flow-mediated dilatation did not improve after ascorbic acid (1.6 ± 2.2% to 2.2 ± 2.5%, ns) or placebo administration (1.2 ± 1,9% to 1.7 ± 1.4%, ns); also nitrate-induced dilatation was similar after ascorbic acid or placebo (16 ± 7.4% vs 17 ± 8%, ns), suggesting no effect of ascorbic acid on endothelial and vascular smooth muscle function. In conclusion, ascorbic acid does not reverse endothelial vasomotor dysfunction in the brachial circulation of patients with Raynaud’s phenomenon secondary to systemic sclerosis. The use of different antioxidants or different dosing of ascorbic acid may be required to show a beneficial effect on endothelial vasodilator function.

scholarly journals ILD-specific health-related quality of life in systemic sclerosis-associated ILD compared with IPF

2020 ◽  
Vol 7 (1) ◽  
pp. e000598
Author(s):  
Michael T Durheim ◽  
Anna-Maria Hoffmann-Vold ◽  
Tomas M Eagan ◽  
Arnt-Ove Hovden ◽  
May Brit Lund ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Function ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related

IntroductionIdiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) are fibrotic ILDs with divergent disease populations. Little is known about health-related quality of life (HRQL) in SSc-ILD relative to IPF.MethodsWe used the Kings Brief Interstitial Lung Disease Questionnaire (K-BILD) to compare HRQL in a cross-sectional study of 57 patients with IPF and 29 patients with SSc-ILD. Analysis of covariance was used to adjust for age, gender and lung function.ResultsThe unadjusted mean K-BILD score was 63.1 (95% CI 57.1 to 69.1) among patients with SSc-ILD, as compared with 54.7 (51.8–57.5) among those with IPF (p=0.005). However, this difference in HRQL was attenuated after adjustment for age, gender and lung function. In a multivariable model, only forced vital capacity was associated with K-BILD scores. K-BILD scores were correlated with both forced vital capacity and with other relevant HRQL measures, regardless of ILD diagnosis.DiscussionPatients with SSc-ILD may have better ILD-specific quality of life than patients with IPF, but this difference appears to be driven primarily by better lung function. These results underscore the impact of lung function on HRQL in fibrotic ILD and the utility of K-BILD to assess HRQL in SSc-ILD.

scholarly journals FRI0233 IMPACT AND ADHERENCE TO THE MEDITERRANEAN DIET IN SYSTEMIC SCLEROSIS ITALIAN PATIENTS: CORRELATION WITH GASTROINTESTINAL SYMPTOMS, MOOD DISTURBANCES AND QUALITY OF LIFE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 700.2-700
Author(s):  
G. De Luca ◽  
G. Natalello ◽  
G. Abignano ◽  
C. Campochiaro ◽  
D. Temiz Karadağ ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Mediterranean Diet ◽  
Dietary Habits ◽  
Anxiety And Depression ◽  
Research Support ◽  
Work Impairment ◽  
Gi Symptoms ◽  
The Mediterranean

Background:Gastrointestinal involvement(GI) is a common feature of systemic sclerosis(SSc) and can be highly disabling, representing a major cause of morbidity and reduced quality of life(QoL). The impact of dietary habits on GI symptoms, mood and QoL has not been extensively evaluated.Objectives:To evaluate the adherence to the Mediterranean Diet(MD) in an Italian multicenter cohort of SSc patients, and its impact on GI symptoms and other disease features, depression, anxiety and overall QoL.Methods:Consecutive SSc(ACR/EULAR2013) patients from 4 Italian cohorts were enrolled. Dietary habits and adherence to the MD were assessed using the 14-item MEDAS and QueMD questionnaires. Presence and severity of depressive/anxious symptoms and QoL were evaluated with the Hospital Anxiety and Depression Scale(HADS) and the SSc-HAQ(S-HAQ). GI symptoms were assessed with the Reflux Disease Questionnaire(RDQ) and the UCLA SCTC GI Tract 2.0 questionnaire(USG). Associations with patients’ lifestyle, disease characteristics, and nutritional status were explored.Results:265 patients (94.7% females; age 55.8±13.6years; disease duration 9.1±7.0years; diffuse SSc 31.8%; Scl70 + 35.8%;ulcers 23.4%;ILD 29.4%;BMI 23.7±4.4 Kg/m2; obese 11.3%,overweight 23.4%,underweight 4.9%) were enrolled.Overall MD adherence was moderate(7.5±1.9) according to MEDAS and it correlated with QueMD score(4.53±1.96)(R=.371,p<0.001). MD adherence was optimal in 39 patients(14.7%), more frequently from Central-Southern Italy(p=0.036); 189 patients(71.3%) had a good and 37(14.0%) a poor MD adherence.GI symptoms were moderate/severe according to USG in 37(14.0%) patients(USG:0.41±0.40), and heartburn was the most common GI symptom(35.2%). The prevalence of significant anxiety and depression was 24.1% and 17.0%.An inverse correlation was found between MD adherence and mood disturbances at HADS(MEDAS; R=-0.181,p=0.04), work impairment(QueMD;R=-0.247,p=0.005) and reduced QoL, both for GI (constipation at USG: R=-0.133,p=0.032) and general S-HAQ items(bowel:R=-0.181,p=0.04;severity R=-0.202,p=0.01;Raynaud:R=-0.217,p<0.001;ulcers: R=-0.207,p=0.01). MD adherence directly correlated with lung function(MEDAS;R=0.181,p=0.023 for FVC and R=0.170,p=0.03 for DLCO). Patients with MD optimal adherence had lower HADS depression(p=0.04) and S-HAQ scores(HAQ,p=0.04; Raynaud,p=0.005; ulcers,p=0.02) and lower work impairment and lost work time(p=0.03). No significant correlation emerged between MD adherence and BMI, or specific symptoms. Depression and anxiety directly correlated with the severity of reported upper GI symptoms according to both scales (RDQ-GERD:R=0.261,p=0.001; USG:R=0.263,p<0.001) and general S-HAQ items (HAQ: R=0.136,p=0.032;severity R=0.233,p<0.001;bowel: R=0.135,p=0.04;breath: R=0.133,p=0.03; ulcers: R=0.132,p=0.037). Results were confirmed after exclusion of psychiatric(11.7%) and fibromyalgic(15.5%) patients.Conclusion:Unsatisfactory MD adherence is associated with a low mood, impaired QoL, work impairment, GI and vascular symptoms in Italian SSc patients. The promotion of a healthy lifestyle could positively impact on QoL and disease status of SSc patients.References:[1]Gnagnarella P, et a. NMCD 2018. DOI: 10.1016/j.numecd.2018.06.006[2]Jaeger VK et al. PLoS One, 2016.[3]Dinu M et al. Eur J Clin Nutr 2018. doi: 10.1038/ejcn.2017.58.[4]Khanna D, et al.Arthritis Rheum. 2009. doi: 10.1002/art.24730.Acknowledgments:GILSDisclosure of Interests:Giacomo De Luca Grant/research support from: SOBI, Speakers bureau: SOBI, Novartis, Pfizer, MSD, Celgene, Gerlando Natalello: None declared, Giuseppina Abignano: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Duygu Temiz Karadağ: None declared, Maria De Santis: None declared, Elisa Gremese Speakers bureau: Abbvie, BMS, Celgene, Jannsen, Lilly, MSD, Novartis, Pfizer, Sandoz, UCB, Silvia Laura Bosello Speakers bureau: Abbvie, Pfizer, Boehringer, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI

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