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management and raynaud phenomenon

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https://www.readbyqxmd.com/read/29324934/stem-cells-therapy-the-future-in-the-management-of-systemic-sclerosis-a-case-report
#1
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
https://www.readbyqxmd.com/read/29320807/overlap-of-interstitial-pneumonia-with-autoimmune-features-with-undifferentiated-connective-tissue-disease-and-contribution-of-uip-to-mortality
#2
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
https://www.readbyqxmd.com/read/29201156/evidence-based-management-of-raynaud-s-phenomenon
#3
REVIEW
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
https://www.readbyqxmd.com/read/29198478/the-evaluation-of-a-home-based-program-for-hands-in-patients-with-systemic-sclerosis
#4
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
https://www.readbyqxmd.com/read/29173135/a-64-year-old-woman-with-interstitial-lung-disease-and-positive-antibodies-against-aminoacyl-transfer-rna-synthetases-in-the-absence-of-myositis-presentation-of-an-anti-pl-12-positive-antisynthetase-syndrome
#5
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
https://www.readbyqxmd.com/read/28980901/long-term-treatment-of-scleroderma-related-digital-ulcers-with-iloprost-a-cohort-study
#6
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
https://www.readbyqxmd.com/read/28913687/non-atherosclerotic-vascular-disease-in-women
#7
REVIEW
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
https://www.readbyqxmd.com/read/28901690/hypothenar-hammer-syndrome-in-an-office-worker
#8
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...
September 13, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28895508/esvm-guidelines-the-diagnosis-and-management-of-raynaud-s-phenomenon
#9
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
https://www.readbyqxmd.com/read/28869417/efficacy-of-intensive-hand-physical-therapy-in-patients-with-systemic-sclerosis
#10
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
https://www.readbyqxmd.com/read/28869415/a-novel-iloprost-administration-method-with-portable-syringe-pump-for-the-treatment-of-acral-ulcers-and-raynaud-s-phenomenon-in-systemic-sclerosis-patients-a-pilot-study-iloporta
#11
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%)...
August 31, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28780561/behaviour-change-interventions-for-the-management-of-raynaud-s-phenomenon-a-systematic-review-protocol
#12
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
https://www.readbyqxmd.com/read/28752373/skin-manifestations-of-rheumatoid-arthritis-juvenile-idiopathic-arthritis-and-spondyloarthritides
#13
REVIEW
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
https://www.readbyqxmd.com/read/28712039/cutaneous-manifestations-of-scleroderma-and-scleroderma-like-disorders-a-comprehensive-review
#14
REVIEW
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
https://www.readbyqxmd.com/read/28641553/capillaroscopy-in-routine-diagnostics-potentials-and-limitations
#15
Francesca Ingegnoli, Vanessa Smith, Alberto Sulli, Maurizio Cutolo
Nailfold capillaroscopy is a safe and useful investigational tool that allows an early detection and a qualitative description of the microvascular abnormalities in patients with Raynaud's phenomenon secondary to scleroderma-spectrum disorders. Nowadays, the role of capillaroscopy in the diagnosis of systemic sclerosis is well known. Capillaroscopy has been included in the new 2013 classification criteria for systemic sclerosis and it is considered a key investigation in the very early phases of the disease...
June 14, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28624897/corrosion-of-harrington-rod-in-idiopathic-scoliosis-long-term-effects
#16
Beth Sherman, Tanya Crowell
PURPOSE: Metal implants have been used to treat adolescent idiopathic scoliosis since the 1960s. Only recently, however, it has the issue of metal-bone breakdown secondary to metal corrosion in situ come to light, raising concerns of possible long-term complications from the resulting metallosis and inflammation of spinal tissues. We present a case of a patient with neurological deficit, pain, and disability with Harrington rod in place for over 30 years, to bring attention to the issue of bio-corrosion of metal implants and its effect on human tissue...
June 17, 2017: European Spine Journal
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#17
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28523890/handy-hints-about-raynaud-s-phenomenon-in-children-a-critical-review
#18
REVIEW
Donato Rigante, Michele Fastiggi, Francesco Ricci, Francesca D'Errico, Benedetta Bracci, Cristina Guerriero
Raynaud's phenomenon (RP) is a vasospastic disorder characterized by recurrent self-limited episodes of skin pallor, cyanosis, and hyperemia caused by paroxysmal spasms in the small arteries of the fingers and toes and can occur in any age group. Hands, feet, nose, ears, and nipples can be affected. The diagnosis is made clinically, assessing varying degrees of ischemia in the involved areas of skin, but this transient ischemia may also herald the onset of connective tissue disease. Investigation is recommended when RP starts in childhood to exclude an underlying autoimmune condition and close follow-up for its development...
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28507447/managing-refractory-cryoglobulinemic-vasculitis-challenges-and-solutions
#19
REVIEW
Predrag Ostojic, Ivan R Jeremic
Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III), vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation - cryoglobulinemic vasculitis (CryoVas). Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers), weakness, peripheral neuropathy, Raynaud's phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28479486/integrated-diagnosis-project-for-inflammatory-myopathies-an-association-between-autoantibodies-and-muscle-pathology
#20
REVIEW
Shigeaki Suzuki, Akinori Uruha, Norihiro Suzuki, Ichizo Nishino
Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve skeletal muscle as well as many other organs. The classification of inflammatory myopathies has been based on clinical diagnoses, pathological diagnoses, and autoantibodies, independently. The clinical phenotypes of inflammatory myopathies are characterized by various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, since the correlation between histological features and autoantibodies had not been fully elucidated, we created the "Integrated Diagnosis Project for Inflammatory Myopathies" in October 2010...
July 2017: Autoimmunity Reviews
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