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management and raynaud phenomenon

John D Pauling, Lesley Ann Saketkoo, Marco Matucci-Cerinic, Francesca Ingegnoli, Dinesh Khanna
RP is the most common manifestation of SSc and a major cause of disease-related morbidity. This review provides a detailed appraisal of the patient experience of SSc-RP and potential implications for disease classification, patient-reported outcome instrument development and SSc-RP clinical trial design. The review explores the clinical features of SSc-RP, the severity and burden of SSc-RP symptoms and the impact of SSc-RP on function, work and social participation, body image dissatisfaction and health-related quality of life in SSc...
March 12, 2018: Rheumatology
Ariane L Herrick, Andrea Murray
Most patients with Raynaud's phenomenon (RP) have "benign" primary RP (PRP), but a minority have an underlying cause, for example a connective tissue disease such as systemic sclerosis (SSc). Secondary RP can be associated with structural as well as functional digital vascular changes and can be very severe, potentially progressing to digital ulceration or gangrene. The first step in management is to establish why the patient has RP. This short review discusses the role of nailfold capillaroscopy and thermography in the assessment of RP...
March 8, 2018: Autoimmunity Reviews
Caterina Matucci-Cerinic, Vivek Nagaraja, Francesca Prignano, Bashar Kahaleh, Silvia Bellando-Randone
Raynaud's phenomenon (RP) is a functional vascular disorder involving extremities. In his practice, the dermatologist may frequently encounter RP which affects mainly women and is categorized into a primary benign form and a secondary form associated with different diseases (infections, drugs, autoimmune and vascular conditions, hematologic, rheumatologic and endocrinologic disorders). Still today, the differential diagnosis is a clinical challenge. Therefore, a careful history and a physical examination, together with laboratory tests and nailfold capillaroscopy are mandatory...
March 6, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Matylda Sierakowska, Stanisław Sierakowski, Justyna Sierakowska, Elżbieta Krajewska-Kułak, Mwidimi Ndosi
In the process of the planned and systematic education of patients/families, it is extremely important to identify patients' health problems as well as their needs and expectations. The objective of this study was to determine the relationship between functional disability, health problems and perceived educational needs in people with systemic sclerosis (SSc). This was a cross-sectional analytic study conducted in six rheumatology centers in Poland. Functional disability was measured using HAQ-DI, and the magnitude of other health problems (pain, fatigue, intestinal problems, breathing problems, Raynaud's phenomenon, finger ulcerations) was measured using 0-100 mm visual analogue scales...
March 1, 2018: Rheumatology International
John D Pauling, Robyn T Domsic, Lesley A Saketkoo, Celia Almeida, Jane Withey, Hilary Jay, Tracy M Frech, Francesca Ingegnoli, Emma Dures, Joanna Robson, Neil J McHugh, Ariane L Herrick, Marco Matucci-Cerinic, Dinesh Khanna, Sarah Hewlett
OBJECTIVES: Raynaud's phenomenon (RP) is the commonest manifestation of systemic sclerosis (SSc). RP is an episodic phenomenon, not easily assessed in the clinic, leading to reliance on self-report. A thorough understanding of the patient experience of SSc-RP is essential to ensuring patient-reported outcome (PRO) instruments capture domains important to the target patient population. We report the findings of an international qualitative research study investigating the patient experience of SSc-RP...
November 21, 2017: Arthritis Care & Research
Velu Nair, Vivek Vasdev, Abhishek Kumar, Subramanian Shankar, Vivek Nair, Ajay Sharma
AIM: To prospectively evaluate long term outcomes in a cohort of patients with Systemic sclerosis treated with Hematopoietic stem cell transplant (HSCT). METHOD: This is a prospective observational study of four SSc patients who underwent HSCT at a tertiary care center in India between 2008-2012. The selection criteria included young individuals with rapidly progressive disease and at least one major organ involvement. We used granulocyte colony-stimulating factor for peripheral blood stem cell mobilization, pre-transplant conditioning with fludarabine, cyclophosphamide and rabbit anti-thymocyte globulin followed by re-infusion of autologous stem cells as per standard institute protocol...
February 2, 2018: International Journal of Rheumatic Diseases
Jung In Song, Silvanie Volz, Maria Eirini Liodaki, Peter Mailänder, Konstantinos Kalousis
OBJECTIVE: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology, with heterogeneous clinical manifestations and chronic and often progressive course. The diffuse cutaneous form of SSc (dcSSc) is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs. Patients with limited cutaneous SSc (lcSSc) generally have long-standing Raynaud's phenomenon before other manifestations of SSc appear. Over the last decade the Interest of adipose-derived cell therapy in regenerative medicine has increased continuously...
September 2017: Hellenic Journal of Nuclear Medicine
Bryan T Kelly, Teng Moua
BACKGROUND AND OBJECTIVE: Criteria for interstitial pneumonia with autoimmune features (IPAF) were recently established for research purposes in a joint statement from the European Respiratory Society (ERS) and American Thoracic Society (ATS). We reviewed the utility of these criteria in patients previously diagnosed as broadly defined undifferentiated connective tissue disease (UCTD) and noted overlapping IPAF findings. Additional review was given to IPAF patients with usual interstitial pneumonia (UIP) on histopathology or radiology in terms of survival and outcome...
January 10, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
Ariane L Herrick
Raynaud's phenomenon (RP) is relevant to the rheumatologist because it may signify an underlying connective tissue disease and also because it can be very challenging to treat, especially when it has progressed to digital ulceration or critical ischaemia. This review article discusses diagnosis (does this patient have an underlying connective tissue disease?), including the role for nailfold capillaroscopy, and treatment. Management of 'uncomplicated' RP is first described and then treatment of RP complicated by progression to digital ulceration or critical ischaemia, highlighting recent advances (including phosphodiesterase type 5 inhibition, and endothelin 1 receptor antagonism) and the evidence base underpinning these...
December 2017: Therapeutic Advances in Musculoskeletal Disease
Sibila Floriano Landim, Manoel Barros Bertolo, Marcos Felipe Marcatto de Abreu, Ana Paula Del Rio, Cecilia Carmen Mazon, João Francisco Marques-Neto, Janet L Poole, Eduardo de Paiva Magalhães
STUDY DESIGN: This study used a quasi-experimental design where patients were evaluated before and after participation in the self-management program. INTRODUCTION: Hands are commonly affected in systemic sclerosis (SSc). Strategies to maintain or improve hand function are indicated upon diagnosis and throughout the course of the disease. PURPOSE OF THE STUDY: The purpose of this study was to develop and evaluate a home-based program for hands in patients with SSc...
November 29, 2017: Journal of Hand Therapy: Official Journal of the American Society of Hand Therapists
Katrien Ghysen, Mathias Leys
Introduction The antisynthetase syndrome is a rare autoimmune disease described by the presence of inflammatory myositis, interstitial lung disease and antibodies against aminoacyl-transfer RNA synthetases. Interstitial lung disease can be the only manifestation in the absence of an inflammatory myositis. Other clinical signs are Raynaud phenomenon, hyperkeratotic skin lesions, fever and inflammatory polyarthritis. Case presentation We report the case of a 64-year old woman who complained of a dry cough, progressive dyspnea and arthralgia since 2 years, with no other systemic symptoms...
November 27, 2017: Acta Clinica Belgica
Michele Colaci, Federica Lumetti, Dilia Giuggioli, Serena Guiducci, Silvia Bellando-Randone, Ginevra Fiori, Marco Matucci-Cerinic, Clodoveo Ferri
OBJECTIVES: Raynaud's phenomenon and chronic/recurrent digital ulcers (DU) are main features of systemic sclerosis (SSc). Their treatment includes both systemic (i.e., iloprost) and local therapies. We report the therapeutic effects of iloprost in a cohort of SSc patients during a long-lasting follow-up period. METHODS: Fifty consecutive SSc patients (M/F 7/43, age at SSc diagnosis 43.5±12.7SD years) received iloprost infusions for 10±4.2SD years. Iloprost schedule consisted in monthly infusion at 0...
September 2017: Clinical and Experimental Rheumatology
Lee Joseph, Esther S H Kim
Takayasu arteritis, fibromuscular dysplasia (FMD), spontaneous arterial dissection, Raynaud's phenomenon, and chilblains are vascular conditions that are associated with an increased predisposition in women and are often underdiagnosed. Takayasu arteritis has an incidence rate of 2.6 cases per million individuals per year in the USA and predominantly affects women of childbearing age. HLA-B5 genetic locus is linked with Takayasu arteritis susceptibility. Methods to determine active disease are limiting; currently utilized clinical and imaging findings and laboratory tests are of limited value for this purpose...
September 14, 2017: Current Treatment Options in Cardiovascular Medicine
Fan Zhang, Yuranga Weerakkody, Patrik Tosenovsky
Hypothenar hammer syndrome (HHS) is an uncommon cause of unilateral Raynaud's phenomenon, splinter haemorrhages and hypothenar weakness. The typical patient is a male blue-collar worker who uses their hypothenar eminence to hammer objects as part of their work. The distal ulnar artery beyond Guyon's canal is superficial and vulnerable to blunt trauma. CTA and MRA are common initial investigations and can suggest the diagnosis. DSA is the gold standard imaging modality and offers therapeutic opportunities. Management is controversial, but unless there is critical digital ischaemia, conservative treatment is first line...
December 2017: Journal of Medical Imaging and Radiation Oncology
Jill Belch, Anita Carlizza, Patrick H Carpentier, Joel Constans, Faisel Khan, Jean-Claude Wautrecht, Adriana Visona, Christian Heiss, Marianne Brodeman, Zsolt Pécsvárady, Karel Roztocil, Mary-Paula Colgan, Dragan Vasic, Anders Gottsäter, Beatrice Amann-Vesti, Ali Chraim, Pavel Poredoš, Dan-Mircea Olinic, Juraj Madaric, Sigrid Nikol, Ariane L Herrick, Muriel Sprynger, Peter Klein-Weigel, Franz Hafner, Daniel Staub, Zan Zeman
Regarding the clinical diagnosis of Raynaud's phenomenon and its associated conditions, investigations and treatment are substantial, and yet no international consensus has been published regarding the medical management of patients presenting with this condition. Most knowledge on this topic derives from epidemiological surveys and observational studies; few randomized studies are available, almost all relating to drug treatment, and thus these guidelines were developed as an expert consensus document to aid in the diagnosis and management of Raynaud's phenomenon...
October 2017: VASA. Zeitschrift Für Gefässkrankheiten
József Horváth, Zsófia Bálint, Erika Szép, Aletta Deiszinger, Tünde Minier, Nelli Farkas, Enikő Török, Éva Horváthné Papp, Dalma Komjáti, Zsuzsanna Mándó, László Czirják, Cecilia Varjú
OBJECTIVES: To evaluate the efficacy of a three-week period of complex and intensive hand physical therapy on hand function in patients with systemic sclerosis (SSc). METHODS: Thirty-one patients with SSc were treated. Hand stretching exercises, ergotherapy supplemented with thermal and mud baths, whirlpool therapy and soft tissue massage were daily used during a three-week period. The control SSc group (n=22) received similar management for their large joints leaving out their hands...
September 2017: Clinical and Experimental Rheumatology
Paolo Fraticelli, Giuseppe Pio Martino, Marta Murri, Massimo Mattioli, Armando Gabrielli
OBJECTIVES: The objective of the study is to evaluate the feasibility, safety and efficacy of home infusion of iloprost with the new portable syringe pump Infonde®, for the treatment of scleroderma-related Raynaud's phenomenon and digital ulcers. METHODS: 12 scleroderma patients were treated with iloprost at home, using the pump, with infusion cycles of 2 days per month (24 hours a day), for 6 months. RESULTS: The home treatment proved feasible since ten patients (83%) completed the entire infusion cycle, thus satisfying the feasibility target imposed by the protocol (75%)...
September 2017: Clinical and Experimental Rheumatology
Jo Daniels, John D Pauling, Christopher Eccelston
INTRODUCTION: Raynaud's phenomenon (RP) describes excessive peripheral vasospasm to cold exposure and/or emotional stress. RP episodes are associated with digital colour changes, pain and reduced quality of life. Pharmacological interventions are of low to moderate efficacy and often result in adverse effects such as facial flushing and headaches. Recommended lifestyle and behavioural interventions have not been evaluated. The objectives of the proposed systematic review are to assess the comparative safety and efficacy of behaviour change interventions for RP and identify what we can learn to inform future interventions...
August 4, 2017: BMJ Open
Carolyn Jean Chua-Aguilera, Burkhard Möller, Nikhil Yawalkar
Extra-articular manifestations of rheumatoid arthritis, juvenile idiopathic arthritis, and various spondyloarthritides including psoriatic arthritis, ankylosing spondylitis, reactive arthritis, and inflammatory bowel disease-associated spondyloarthritis often involve the skin and may occur before or after diagnosis of these rheumatic diseases. Cutaneous manifestations encompass a wide range of reactions that may have a notable negative impact not only on the physical but especially on the emotional and psychosocial well-being of these patients...
December 2017: Clinical Reviews in Allergy & Immunology
Caterina Ferreli, Giulia Gasparini, Aurora Parodi, Emanuele Cozzani, Franco Rongioletti, Laura Atzori
Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations...
December 2017: Clinical Reviews in Allergy & Immunology
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