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https://www.readbyqxmd.com/read/29145709/defining-a-unified-vascular-phenotype-in-systemic-sclerosis
#1
REVIEW
Yannick Allanore, Oliver Distler, Marco Matucci-Cerinic, Christopher P Denton
Microcirculation impairment and related vasculopathy is a hallmark of systemic sclerosis. Digital ulcers are second only to Raynaud's phenomenon as a vascular complication occurring in scleroderma patients. Digital ulcers are painful and generate disability. Furthermore, patients may develop recurrent digital ulcers and one may ask whether the outcomes of such patients might be different to those of unaffected ones. Recently several registries have provided relevant information about digital ulcers. Male sex and severe skin disease seem to be the main associated factors observed in several registries...
November 16, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29123575/systemic-sclerosis-and-the-gastrointestinal-tract
#2
REVIEW
Irena Walecka
Systemic sclerosis (SSc) is an autoimmunological disease of unknown origin with complex pathogenesis and multiple organ involvement. It is characterised by vascular and immunological abnormalities leading to fibrosis of the skin and internal organs. It is a rather rare disease with a prevalence of around 20 per 100,000. The disease results in heterogeneous clinical findings and different courses. Systemic sclerosis usually begins with the onset of Raynaud's phenomenon (RP), followed by skin sclerosis and internal organ involvement, although it may appear synchronously with RP...
2017: Przegla̜d Gastroenterologiczny
https://www.readbyqxmd.com/read/29118999/associates-and-predictors-of-pleurisy-or-pericarditis-in-sle
#3
Seungwon Ryu, Wei Fu, Michelle A Petri
Background/Purpose: Serositis is one of both ACR and SLICC classification criteria for systemic lupus erythematosus (SLE) and a common type of extra renal flare. However, little is known about clinical or immunological associations of pleurisy or pericarditis. The aim of this study is to analyze associates and predictors of pleurisy versus pericarditis in Caucasians and African Americans with SLE. Methods: 2,390 SLE patients in the Hopkins Lupus Cohort were analyzed for demographic, clinical and serologic associates of pleurisy or pericarditis, defined using the SELENA revision of the SLE Disease Activity Index (SLEDAI)...
2017: Lupus Science & Medicine
https://www.readbyqxmd.com/read/29104809/mycobacterium-intracellulare-infection-mimicking-progression-of-scleroderma
#4
Simon Krabbe, Merete Engelhart, Sören Thybo, Søren Jacobsen
This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud's phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29072596/environmental-pollution-by-benzene-and-pm10-and-clinical-manifestations-of-systemic-sclerosis-a-correlation-study
#5
Alice Borghini, Andrea Poscia, Silvia Bosello, Adele Anna Teleman, Mario Bocci, Lanfranco Iodice, Gianfranco Ferraccioli, Daniele Ignazio La Milìa, Umberto Moscato
Atmospheric air pollution has been associated with a range of adverse health effects. The environment plays a causative role in the development of Systemic Sclerosis (SSc). The aim of the present study is to explore the association between particulate (PM10) and benzene (B) exposure in Italian patients with systemic sclerosis and their clinical characteristics of the disease. A correlation study was conducted by enrolling 88 patients who suffer from SSc at the Fondazione Policlinico "A. Gemelli" in Rome (Italy) in the period from January 2013 to January 2014...
October 26, 2017: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/29068146/chronic-inflammation-predicts-long-term-mortality-in-patients-with-raynaud-s-phenomenon
#6
M Mueller, M E Gschwandtner, J Gamper, G-A Giurgea, H P Kiener, T Perkmann, R Koppensteiner, O Schlager
BACKGROUND: Subclinical chronic inflammation could be the driving force behind the recently revealed association between abnormal nailfold capillaries as well as autoantibodies and long-term mortality in patients with incipient Raynaud's phenomenon. Whether laboratory markers that reflect a chronic inflammatory process are directly related to mortality in Raynaud's phenomenon is not known. METHODS: In total, 2958 patients with incipient Raynaud's phenomenon without previously known connective tissue disease (CTD) were enrolled...
October 25, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/29052562/clinical-profile-and-significance-of-mucocutaneous-lesions-of-primary-sj%C3%A3-gren-s-syndrome-a-large-cross-sectional-study-with-874-patients
#7
Lei Xuan, Yi-Dong Zhang, Li Li, Yue-Ping Zeng, Hao-Ze Zhang, Jing Wang, Zhen-Hua Dong
BACKGROUND: Mucocutaneous lesions are common features of primary Sjögren's syndrome (pSS), but only a few studies have focused on them. To demonstrate the profile of mucocutaneous lesions of pSS and further explore their potential clinical significance, we performed a cross-sectional study on 874 patients. METHODS: Demographic data, clinical manifestations, and laboratory results of 874 pSS patients were collected. Patients were divided into two groups according to the presence of mucocutaneous lesions...
October 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/29044769/human-papillomavirus-vaccination-of-adult-women-and-risk-of-autoimmune-and-neurological-diseases
#8
A Hviid, H Svanström, N M Scheller, O Grönlund, B Pasternak, L Arnheim-Dahlström
BACKGROUND: Since 2006, human papillomavirus (HPV) vaccines have been introduced in many countries worldwide. Whilst safety studies have been reassuring, focus has been on the primary target group, the young adolescent girls. However, it is also important to evaluate safety in adult women where background disease rates and safety issues could differ significantly. OBJECTIVE: We took advantage of the unique Danish and Swedish nationwide healthcare registers to conduct a cohort study comparing incidence rate ratios (RRs) of 45 preselected serious chronic diseases in quadrivalent HPV (qHPV)-vaccinated and qHPV-unvaccinated adult women 18-44 years of age...
October 18, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/29044628/possible-pro-inflammatory-role-of-heparin-binding-epidermal-growth-factor-like-growth-factor-in-the-active-phase-of-systemic-sclerosis
#9
Megumi Hirabayashi, Yoshihide Asano, Takashi Yamashita, Shunsuke Miura, Kouki Nakamura, Takashi Taniguchi, Ryosuke Saigusa, Takehiro Takahashi, Yohei Ichimura, Takuya Miyagawa, Ayumi Yoshizaki, Tomomitsu Miyagaki, Makoto Sugaya, Shinichi Sato
Heparin-binding epidermal growth factor (EGF)-like growth factor (HB-EGF) is a member of the EGF family growth factors, which affects multiple aspects of the wound healing process such as epithelialization, wound contraction and angiogenesis. In our study, we measured the serum HB-EGF levels of 51 systemic sclerosis (SSc) patients, which showed a significant increase compared with those of 20 normal subjects. Further analysis revealed a positive correlation between the HB-EGF level and pulmonary ground-glass score but no correlation between the former and pulmonary fibrosis score...
October 17, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28993902/cardiac-involvement-in-undifferentiated-connective-tissue-disease-at-risk-for-systemic-sclerosis-otherwise-referred-to-as-very-early-early-systemic-sclerosis-a-tdi-study
#10
Michele D'Alto, Antonella Riccardi, Paola Argiento, Ilaria Di Stefano, Emanuele Romeo, Agostino Mattera Iacono, Antonello D'Andrea, Serena Fasano, Alessandro Sanduzzi, Marialuisa Bocchino, Ludovico Docimo, Salvatore Tolone, Maria Giovanna Russo, Gabriele Valentini
Undifferentiated connective tissue disease at risk for systemic sclerosis (UCTD-risk-SSc), otherwise referred to as very early-early SSc, is a condition characterized by Raynaud's phenomenon with serum SSc marker autoantibodies and/or typical capillaroscopic findings and unsatisfying classification criteria for the disease. The aim of the present study was to assess the prevalence of right (RV) or left ventricular (LV) systolic and/or diastolic dysfunction by standard echocardiography and tissue Doppler imaging (TDI)...
October 9, 2017: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/28975961/outcomes-rates-and-predictors-of-transition-of-isolated-raynaud-s-phenomenon-a-systematic-review-and-meta-analysis
#11
Francesca Ingegnoli, Nicola Ughi, Chiara Crotti, Marta Mosca, Chiara Tani
QUESTIONS: Published studies lack clear indicators of risk and predictors of transition from Raynaud's phenomenon (Rp) to connective tissue diseases (CTDs). Therefore, we aimed to study the outcomes, rates and predictors of transition to CTDs in patients with Rp. METHODS: A sensitive search was developed in Medline and Embase. Observational studies reporting incidence and risk factors of transition from Rp to a CTD were analysed by two independent reviewers. The main outcome was the rate of transition to a CTD; the secondary outcome was the evaluation of predictors...
October 4, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28967806/pulmonary-pathologic-manifestations-of-anti-alanyl-trna-synthetase-anti-pl-12-related-inflammatory-myopathy
#12
Frank Schneider, Samuel A Yousem, Chester V Oddis, Rohit Aggarwal
CONTEXT: - Patients with anti-aminoacyl-tRNA synthetase syndrome (ARS), a subset of idiopathic inflammatory myopathy, have a high prevalence of lung involvement. Autoantibodies directed against alanyl-tRNA synthetase (anti-PL-12 Abs) represent 1 of the 8 autoantibodies currently described under the rubric of ARS. OBJECTIVE: - To describe the clinical, radiographic, and pulmonary histopathologic findings in patients possessing anti-PL-12 autoantibodies. DESIGN: - Patients with anti-PL-12 ARS were identified in the University of Pittsburgh Idiopathic Inflammatory Myopathy registry...
October 2, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28966213/elevated-serum-anti-gm-csf-antibodies-before-the-onset-of-autoimmune-pulmonary-alveolar-proteinosis-in-a-patient-with-sarcoidosis-and-systemic-sclerosis
#13
Mari Yamasue, Shin-Ichi Nureki, Yuko Usagawa, Tomoko Ono, Hiroyuki Matsumoto, Takamasa Kan, Jun-Ichi Kadota
Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-schiff stain-positive lipoproteinaceous materials in the alveolar space due to impaired surfactant clearance by alveolar macrophage. Autoimmune PAP is the most common form of PAP, but rarely accompanies collagen disease or sarcoidosis. We report here a rare case of autoimmune PAP preceded by systemic sclerosis and sarcoidosis. A 64-year-old woman was admitted to our hospital for blurred vision, muscle weakness of extremities, Raynaud's phenomenon, and exertional dyspnea...
September 2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28960445/vascular-branches-from-cutaneous-nerve-of-the-forearm-and-hand-application-to-better-understanding-raynaud-s-disease
#14
K Umemoto, M Ohmichi, Y Ohmichi, T Yakura, N Hammer, D Mizuno, M Naito, T Nakano
INTRODUCTION: Cutaneous nerves have branches called vascular branches (VBs) that reach arteries. VBs are thought to be involved in arterial constriction, and this is the rationale for periarterial sympathectomy as a treatment option for Raynaud's disease. However, the branching patterns and distribution areas of the VBs remain largely unclear. The aim of the present study was to investigate the anatomical structures of the VBs of the cutaneous nerves. MATERIALS AND METHODS: Forty hands and forearms were examined to assess the branching patterns and distribution areas of the VBs of the superficial branch of the radial nerve (SBRN), the lateral antebrachial cutaneous nerve (LACN), the medial antebrachial cutaneous nerve (MACN), and the palmar cutaneous branch of the ulnar nerve (PCUN)...
September 29, 2017: Clinical Anatomy
https://www.readbyqxmd.com/read/28954092/anti-neutrophil-cytoplasmic-antibodies-in-new-onset-systemic-lupus-erythematosus
#15
Fang Su, Weiguo Xiao, Pingting Yang, Qingyan Chen, Xiaojie Sun, Tienan Li
Background: The clinical significance of anti-neutrophil cytoplasmic antibodies in patients with new-onset systemic lupus erythematosus, especially in systemic disease accompanied by interstitial lung disease remains to be elucidated. Objectives: This study was designed to investigate the role of anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus patients. Methods: A hundred and seven patients with new-onset SLE were enrolled...
July 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28940121/anti-cenp-b-and-anti-topo-1-containing-sera-from-systemic-sclerosis-related-diseases-with-raynaud-s-phenomenon-induce-vascular-endothelial-cell-senescence-not-via-classical-p53-p21-pathway
#16
Chieh-Yu Shen, Ko-Jen Li, Pei-Hsuan Lai, Chia-Li Yu, Song-Chou Hsieh
Raynaud's phenomenon (RP) is the earliest and most common clinical manifestation in patients with systemic sclerosis (SSc) and its related diseases containing anti-TOPO-1 and/or anti-CENP-B autoantibodies in the sera. However, the cause-effect relationship between the two autoantibodies and RP remains elucidation. Sera containing anti-CENP-B and anti-TOPO-1 autoantibodies were obtained from SSc-related diseases manifesting RP. The polyclonal auto-antibodies were purified from pooled sera by affinity chromatography...
September 23, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28937580/diagnosis-of-systemic-inflammatory-diseases-among-patients-admitted-for-acute-pericarditis-with-pericardial-effusion
#17
Maureen Assayag, Rachid Abbas, Noémie Chanson, Anne Perozziello, Gregory Ducrocq, Jean-François Alexandra, Antoine Dossier, Thomas Papo, Karim Sacre
AIMS: Acute pericarditis may be the heralding manifestation of various systemic inflammatory diseases (SIDs). The aim of this study was to identify clinical indicators for SIDs in patients admitted for acute pericarditis with pericardial effusion. METHODS: All consecutive adult patients hospitalized in a Department of Internal Medicine over a 10-year period for acute pericarditis with pericardial effusion were retrospectively reviewed. Patients with cancer and tuberculosis were excluded...
November 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28920529/surgical-interventions-for-organ-and-limb-ischemia-associated-with-primary-and-secondary-antiphospholipid-antibody-syndrome-with-arterial-involvement
#18
Carlos A Hinojosa, Javier E Anaya-Ayala, Karla Bermudez-Serrato, Ramón García-Alva, Hugo Laparra-Escareno, Adriana Torres-Machorro, Rene Lizola
OBJECTIVE: The association of antiphospholipid antibody syndrome (APS) and hypercoagulability is well known. Arterial compromise leading to ischemia of organs and/or limbs in patients with APS is uncommon, frequently unrecognized, and rarely described. We evaluated our institutional experience. METHODS: Retrospective review was conducted. From August 2007 to September 2016, 807 patients with diagnosis of APS were managed in our Institution. Patients with primary and secondary APS who required interventions were examined...
November 2017: Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#19
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
November 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913687/non-atherosclerotic-vascular-disease-in-women
#20
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
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