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management and raynaud phenomenon

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https://www.readbyqxmd.com/read/27833984/a-case-of-a-generalized-symptomatic-calcinosis-in-systemic-sclerosis
#1
Cristian Radu Jecan, Ştefan Iulian Bedereag, Ruxandra Diana Sinescu, Valentin Titus Grigorean, Cristina Nicoleta Cozma, Anca Bordianu, Ioan Petre Florescu
Calcinosis consists of abnormal calcium deposition in soft tissues, which appears often in patients with limited systemic sclerosis, being one of the criteria of CREST (calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndrome. With a long evolution, the aim of the treatment is to control the symptoms and prevent complications. In this article, we present the challenging management of a profuse lesion of calcinosis in a patient with systemic sclerosis. We describe the case of a 52-year-old woman with systemic sclerosis and CREST syndrome who was admitted in our Department with multiple painful and disabling tumoral masses, situated in nearly all joints...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27706206/incidences-and-risk-factors-of-organ-manifestations-in-the-early-course-of-systemic-sclerosis-a-longitudinal-eustar-study
#2
Veronika K Jaeger, Elina G Wirz, Yannick Allanore, Philipp Rossbach, Gabriela Riemekasten, Eric Hachulla, Oliver Distler, Paolo Airò, Patricia E Carreira, Alexandra Balbir Gurman, Mohammed Tikly, Serena Vettori, Nemanja Damjanov, Ulf Müller-Ladner, Jörg H W Distler, Mangtao Li, Ulrich A Walker
OBJECTIVE: Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc. METHODS: In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset...
2016: PloS One
https://www.readbyqxmd.com/read/27682894/interstitial-lung-disease-in-primary-sj%C3%A3-gren-s-syndrome
#3
F Roca, S Dominique, J Schmidt, A Smail, P Duhaut, H Lévesque, I Marie
Interstitial lung disease (ILD) has been reported in 3 to 11% of patients with primary Sjögren's syndrome (pSS). The aims of this retrospective multicenter study were to: 1) analyze characteristics and outcome of ILD in pSS; and 2) evaluate predictive factors associated with ILD onset and deterioration. Twenty-one of 263 patients with pSS (8%) developed ILD. ILD onset preceded pSS diagnosis (n=5), was concurrently identified in association with pSS (n=6) and developed after pSS onset (n=9). Presenting ILD manifestations were: acute/subacute (n=11) onset of ILD, symptomatic progressive onset of ILD (n=5), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT-scan (n=5)...
September 25, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27594391/nailfold-capillaroscopy-microscopy-an-interdisciplinary-appraisal
#4
Peter Franz Klein-Weigel, Cord Sunderkötter, Oliver Sander
Nailfold capillaroscopy is a method of great diagnostic value in the differential diagnosis of primary versus secondary Raynaud´s phenomenon, of systemic sclerosis versus other so called connective tissue diseases and of additional diagnostic value in other entities. Rheumatologists, dermatologists, and angiologists in Germany have convened in an interdisciplinary working group in which they synergistically combined their expertise to develop a common nomenclature and standards for the technical performance of nailfold capillary microscopy...
September 2016: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/27548652/aberrant-immune-response-with-consequent-vascular-and-connective-tissue-remodeling-causal-to-scleroderma-and-associated-syndromes-such-as-raynaud-phenomenon-and-other-fibrosing-syndromes
#5
Nedim Durmus, Sung-Hyun Park, Joan Reibman, Gabriele Grunig
PURPOSE OF REVIEW: Scleroderma and other autoimmune-induced connective tissue diseases are characterized by dysfunctions in the immune system, connective tissue and the vasculature. We are focusing on systemic sclerosis (SSc)-associated pulmonary hypertension, which remains a leading cause of death with only a 50-60% of 2-year survival rate. RECENT FINDINGS: Much research and translational efforts have been directed at understanding the immune response that causes SSc and the networked interactions with the connective tissue and the vasculature...
November 2016: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27541182/recent-advances-in-the-pathogenesis-and-management-of-raynaud-s-phenomenon-and-digital-ulcers
#6
Ariane L Herrick
PURPOSE OF REVIEW: Systemic sclerosis (SSc)-related digital vasculopathy can progress from severe Raynaud's phenomenon to digital ulceration, is a major cause of pain and disability, and impacts negatively on quality of life. Current treatments are often ineffective and poorly tolerated. This review summarises some of the progress which has been made in the last 12 to 18 months in terms of our understanding of disease process, measurement and treatment. RECENT FINDINGS: The most important findings include that we can now better predict which patients with SSc are most likely to develop digital ulcers...
November 2016: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27494156/clinical-features-and-management-of-erythromelalgia-long-term-follow-up-of-46-cases
#7
Louise K Parker, Cristina Ponte, Kevin J Howell, Voon H Ong, Christopher P Denton, Benjamin E Schreiber
OBJECTIVES: To review our clinical experience of this rare condition and describe the clinical features and response to therapy in a cohort of patients with erythromelalgia (EM), a rare condition, characterised by paroxysmal hyperthermia of the extremities with erythema, pain and intense burning. METHODS: A review was made of the electronic and paper medical records of patients with the diagnosis of EM, with a telephone interview to verify and complete clinical information relating treatment and outcome...
August 2, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27478469/botulinum-toxin-a-in-the-treatment-of-raynaud-s-phenomenon-a-systematic-review
#8
Paweł Żebryk, Mariusz J Puszczewicz
INTRODUCTION: The management of Raynaud's phenomenon in its most severe form is challenging, and current medical and surgical treatment methods frequently do not lead to optimal symptom control and prevention of ischemic complications. The aim of the study was to critically evaluate all existing evidence on the use of botulinum toxin A in the management of Raynaud's phenomenon. MATERIAL AND METHODS: We adopted the PRISMA methodology and searched Cochrane Library, MEDLINE, SCOPUS, EULAR and ACR congresses abstract archives for Raynaud* AND botulinum toxin OR onabotulinum...
August 1, 2016: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/27465880/evidence-for-the-use-of-epoprostenol-to-treat-raynaud-s-phenomenon-with-or-without-digital-ulcers-a-review-of-the-literature
#9
Joseph E Cruz, Ashley Ward, Shannon Anthony, Susanna Chang, Hyun Billy Bae, Evelyn R Hermes-DeSantis
OBJECTIVE: To review the evidence for using intravenous (IV) epoprostenol to treat Raynaud's phenomenon (RP). DATA SOURCES: The databases MEDLINE (1946 to March 2016), PubMed, and International Pharmaceutical Abstracts were searched using the terms epoprostenol, Flolan, Raynaud's disease, and CREST syndrome. Further literature sources were identified by reviewing article citations. STUDY SELECTION AND DATA EXTRACTION: All English-language, clinical trials and case series evaluating IV epoprostenol for the management or treatment of RP were included...
July 26, 2016: Annals of Pharmacotherapy
https://www.readbyqxmd.com/read/27421220/raynaud-s-phenomenon
#10
REVIEW
Michael Hughes, Ariane L Herrick
Raynaud's phenomenon (RP) is a major cause of pain and disability in patients with autoimmune connective tissue diseases (CTDs), particularly systemic sclerosis (SSc). The clinician must perform a comprehensive clinical assessment in patients with RP to differentiate between primary (idiopathic) and secondary RP, in particular (for rheumatologists), secondary to an autoimmune CTD, as both the prognosis and treatment may differ significantly. Key investigations are nailfold capillaroscopy and testing for autoantibodies (in particular, those associated with SSc)...
February 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27413503/update-on-normal-tension-glaucoma
#11
REVIEW
Jyotiranjan Mallick, Lily Devi, Pradeep K Malik, Jogamaya Mallick
Normal tension glaucoma (NTG) is labelled when typical glaucomatous disc changes, visual field defects and open anterior chamber angles are associated with intraocular pressure (IOP) constantly below 21 mmHg. Chronic low vascular perfusion, Raynaud's phenomenon, migraine, nocturnal systemic hypotension and over-treated systemic hypertension are the main causes of normal tension glaucoma. Goldmann applanation tonometry, gonioscopy, slit lamp biomicroscopy, optical coherence tomography and visual field analysis are the main tools of investigation for the diagnosis of NTG...
April 2016: Journal of Ophthalmic & Vision Research
https://www.readbyqxmd.com/read/27178687/cutaneous-manifestations-of-genitourinary-malignancy
#12
REVIEW
Derek Raghavan
Genitourinary cancers are associated with a range of cutaneous syndromes, which can reflect direct metastatic spread, non-metastatic manifestations of malignancy or the consequences of treatment. More than 220,000 new cases of prostate cancer occur each year in the United States, and thus the associations with cutaneous involvement are quite well documented-rare metastatic spread, vasculitic and hemorrhagic syndromes. Cancers of the bladder and kidney may be associated with direct cutaneous metastases, vasculitic syndromes, hereditary leiomyomatosis, and other familial syndromes...
June 2016: Seminars in Oncology
https://www.readbyqxmd.com/read/27175977/thoracic-sympathectomy-for-upper-extremity-ischemia
#13
Frank Hoexum, Hans M Coveliers, Joyce J Lu, Vincent Jongkind, Kakkhee K Yeung, Willem Wisselink
INTRODUCTION: Thoracic sympathectomy is performed in the management of a variety of disorders of the upper extremity. To evaluate the contemporary results of thoracic sympathectomy for upper extremity ischemia a systematic review of the literature was conducted. EVIDENCE AQUISITION: We performed a PubMed, EMBASE and Cochrane search of the literature written in the English language from January 1975 to December 2015. All articles presenting original patient data regarding the effect of treatment on symptoms or on the healing of ulcers were eligible for inclusion...
December 2016: Minerva Cardioangiologica
https://www.readbyqxmd.com/read/27136918/symptoms-of-raynaud-s-phenomenon-rp-in-fibromyalgia-syndrome-are-similar-to-those-reported-in-primary-rp-despite-differences-in-objective-assessment-of-digital-microvascular-function-and-morphology
#14
M Scolnik, B Vasta, D J Hart, J A Shipley, N J McHugh, J D Pauling
Symptoms of Raynaud's phenomenon (RP) are common in fibromyalgia syndrome (FMS). We compared symptom characteristics and objective assessment of digital microvascular function using infrared thermography (and nailfold capillaroscopy where available) in patients with FMS (reporting RP symptoms) and primary RP. We retrospectively reviewed the outcome of microvascular imaging studies and RP symptom characteristics (captured using patient-completed questionnaire at the time of assessment) for patients with FMS (reporting RP symptoms) and patients with primary RP referred for thermographic assessment of RP symptoms over a 2-year period...
October 2016: Rheumatology International
https://www.readbyqxmd.com/read/27115104/systemic-sclerosis-markers-and-targeted-treatments
#15
M Cutolo, A Sulli, C Pizzorni, S Paolino, V Smith
<p>Systemic sclerosis (SSc) is characterized by autoantibody production, progressive microvasculopathy, and aberrant extracellular matrix protein (ECM) synthesis in tissues. The disease presents two major clinical hallmarks: Raynaud's phenomenon (RP) and skin involvement, followed by varying prevalences of internal organ involvement. Despite significant advances in the management of certain organ-specific involvements and symptoms, the research for efficient markers and targets, to be used for an optimized treatment, is still ongoing...
January 2016: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/26902555/severe-raynaud-s-phenomenon-a-streamlined-approach-to-acute-management
#16
Regent Lee, Oliver Lomas, Ashok Handa
Raynaud's phenomenon is an exaggerated vasospastic response to cold or emotional stress which not only may cause the patient severe pain but also critical ischaemia and necrosis of the digits. We report the case of a 69-year-old woman who presented with rest pain, impending ulceration and necrosis of finger tips due to an episode of left-sided Raynaud's phenomenon. Intravenous prostacyclin was administered successfully as a bridge to endoscopic thoracic sympathectomy. Vascular surgery units are ideally positioned for the acute management of severe Raynaud's phenomenon to provide continuity of care to patients with profound digital ischaemia and impending tissue loss...
2011: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/26886351/systemic-sclerosis-markers-and-targeted-treatments
#17
Maurizio Cutolo, Alberto Sulli, Carmen Pizzorni, Sabrina Paolino, Vanessa Smith
Systemic sclerosis (SSc) is characterized by autoantibody production, progressive microvasculopathy, and aberrant extracellular matrix protein (ECM) synthesis in tissues. The disease presents two major clinical hallmarks: Raynaud's phenomenon (RP) and skin involvement, followed by varying prevalences of internal organ involvement. Despite significant advances in the management of certain organ-specific involvements and symptoms, the research for efficient markers and targets, to be used for an optimized treatment, is still ongoing...
February 17, 2016: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/26659258/diagnosis-and-management-of-systemic-sclerosis-a-practical-approach
#18
REVIEW
Jason J Lee, Janet E Pope
Systemic sclerosis is a devastating multisystem rheumatologic condition that is characterized by autoimmunity, tissue fibrosis, obliterative vasculopathy and inflammation. Clinical presentation and course of the condition vary greatly, which complicates both diagnosis and corresponding treatment. In this regard, recent advances in disease understanding, both clinically and biochemically, have led to newer classification criteria for systemic sclerosis that are more inclusive than ever before. Still, significant disease modifying therapies do not yet exist for most patients...
February 2016: Drugs
https://www.readbyqxmd.com/read/26543766/a-case-of-co-existing-paraganglioma-and-thymoma
#19
G Bano, D Sennik, M Kenchaiah, Ye Kyaw, Katie Snape, V Tripathi, P Wilson, I Vlahos, I Hunt, S Hodgson
BACKGROUND: Head and neck paragangliomas are rare tumours and can arise as a part of inherited syndromes. Their association with thymic tumour is not well known. CASE DESCRIPTION: This report describes a female patient who presented with right sided neck paragangliomas. The histology of the tumour was consistent with paraganlioma. Few years later her MRI scan of the chest revealed presence of an anterior mediastinal mass that corresponded to the location of the thymus...
2015: SpringerPlus
https://www.readbyqxmd.com/read/26524895/-progress-of-autoantibody-examinations-for-connective-tissue-diseases
#20
REVIEW
Kengo Akashi, Jun Saegusa, Akio Morinobu
Connective tissue diseases are chronic inflammatory diseases that can affect multiple organs and, thus, have a broad spectrum of clinical presentations. Various autoantibodies are detected in patients with connective tissue diseases, represented by anti-nuclear antibody for systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), Sjögren's syndrome, and mixed connective tissue disease. Assessment of the autoantibody profile is fundamental for the clinical management of patients with connective tissue diseases, providing important data for the diagnosis, clinical characterization, and disease activity evaluation...
May 2015: Rinsho Byori. the Japanese Journal of Clinical Pathology
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