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Raynaud syndrome

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https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#1
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...
September 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28901690/hypothenar-hammer-syndrome-in-an-office-worker
#2
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/28864092/facts-and-controversies-in-mixed-connective-tissue-disease
#3
REVIEW
Julia Martínez-Barrio, Lara Valor, F Javier López-Longo
Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterised by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titers of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility. Although widely discussed, most authors today accept MCTD as an independent entity...
August 29, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28831927/-necrotic-toes-in-a-female-patient-using-interferon
#4
T J R M Auping, P Maat, K van der Waal, R H H Bemelmans
BACKGROUND: Interferon has an important role in treatment of viral hepatitis, multiple sclerosis and solid and non-solid tumours. Ischaemia and necrosis of the extremities are relatively little-known adverse effects of treatment with interferon. CASE DESCRIPTION: A 44-year-old woman was treated with interferon-beta for relapsing-remitting multiple sclerosis. She developed ischaemia and necrosis of the right lower extremity. Extensive laboratory and imaging investigations offered no clear diagnosis, leading to suspicion of a connexion with interferon-beta treatment...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28831595/juvenile-systemic-sclerosis-experience-from-a-tertiary-care-center-from-india
#5
Narendra Kumar Bagri, Dinesh Raj, Jasmeet Kaur, Harish Punia, Isha Saini, Rakesh Lodha, S K Kabra
Juvenile systemic sclerosis (JSSc) is a rare disorder with paucity of information on its treatment and longterm  outcome. Herein, we are sharing our experience with this rare entity. Case records of children, diagnosed to have systemic sclerosis attending Pediatric Rheumatology Clinic at All India Institute of Medical Sciences, New Delhi from January 1998 to June 2016 were reviewed. The demographic, clinical, laboratory, treatment and outcome details were recorded. Disease outcome was classified arbitrarily as controlled, partly controlled or non-responsive/progressive based on: (A) ability to perform activities of daily life (ADL) and (B) presence or absence of musculoskeletal symptoms, skin changes (ulceration/progressive digital pitting/gangrene), and visceral organ involvement (dyspahgia, cardiopulmonary symptoms)...
August 22, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28811188/familial-1p36-3-microduplication-resulting-from-a-1p-9q-non-reciprocal-translocation
#6
Valentine Marquet, Sylvie Bourthoumieu, Amelia Dobrescu, Cécile Laroche-Raynaud, Catherine Yardin
Unlike the 1p36 microdeletion syndrome, which has been extensively described, 1p36 microduplications have rarely been reported. We describe a three years old boy presenting with a severe global developmental delay and a few dysmorphic features. Cytogenetic analyses revealed a maternally inherited 3.35 Mb microduplication of chromosomal band 1p36.3. The maternal grandfather is also carrier of the same chromosomal rearrangement. Interestingly, the duplicated 1p36.3 segment was found to be localized at the telomeric end of the long arms of a chromosome 9, probably deriving from a 1p36...
August 12, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28807722/coronary-microvascular-dysfunction-is-not-associated-with-a-history-of-reproductive-risk-factors-in-women-with-angina-pectoris-an-ipower-substudy
#7
Hannah Elena Suhrs, Anna Meta Kristensen, Anna Bay Rask, Marie Mide Michelsen, Daria Frestad, Naja Dam Mygind, Kira Bové, Eva Prescott
BACKGROUND: Reproductive risk factors such as preeclampsia and recurrent miscarriages have been associated with adverse cardiovascular (CV) events. Underlying coronary microvascular dysfunction (CMD) may be a common denominator. PURPOSE: We investigated whether a history of reproductive risk factors was associated with CMD in women with angina pectoris and no obstructive coronary artery disease (CAD). METHODS: Participants from the iPOWER study, including women with angina pectoris and no obstructive CAD (<50% stenosis), were invited to complete an electronic survey regarding reproductive risk factors: recurrent miscarriages, gestational diabetes, preeclampsia, rhesus immunity, polycystic ovary syndrome and menopausal status as well as migraine and Raynaud phenomenon...
August 11, 2017: Maturitas
https://www.readbyqxmd.com/read/28796005/clinical-spectrum-of-anti-jo-1-associated-disease
#8
Sara Monti, Carlomaurizio Montecucco, Lorenzo Cavagna
PURPOSE OF REVIEW: To provide the most recent evidence on anti-Jo-1 syndrome. RECENT FINDINGS: Several new evidences on anti-Jo-1 syndrome have recently emerged. It has been clearly established that, at disease onset, the classic clinical triad (arthritis, myositis and interstitial lung disease - ILD) is only rarely observed. Indeed, disease onset with an isolated arthritis is common. Patients presenting with an isolated manifestation are at high risk for the subsequent occurrence of initially lacking triad findings...
August 8, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28770709/timing-of-onset-affects-arthritis-presentation-pattern-in-antisyntethase-syndrome
#9
Miguel A González-Gay, Carlomaurizio Montecucco, Albert Selva-O'Callaghan, Ernesto Trallero-Araguas, Ovynd Molberg, Helena Andersson, Jorge Rojas-Serrano, Diana Isabel Perez-Roman, Jutta Bauhammer, Christoph Fiehn, Rossella Neri, Simone Barsotti, Hannes M Lorenz, Andrea Doria, Anna Ghirardello, Florenzo Iannone, Margherita Giannini, Franco Franceschini, Ilaria Cavazzana, Konstantinos Triantafyllias, Maurizio Benucci, Maria Infantino, Mariangela Manfredi, Fabrizio Conti, Andreas Schwarting, Giandomenico Sebastiani, Annamaria Iuliano, Giacomo Emmi, Elena Silvestri, Marcello Govoni, Carlo Alberto Scirè, Federica Furini, Francisco Javier Lopez-Longo, Julia Martínez-Barrio, Marco Sebastiani, Andreina Manfredi, Javier Bachiller-Corral, Walter Alberto Sifuentes Giraldo, Marco A Cimmino, Claudio Cosso, Alessandro Belotti Masserini, Giovanni Cagnotto, Veronica Codullo, Mariaeva Romano, Giuseppe Paolazzi, Raffaele Pellerito, Lesley Ann Saketkoo, Norberto Ortego-Centeno, Luca Quartuccio, Alberto Batticciotto, Elena Bartoloni Bocci, Roberto Gerli, Christof Specker, Elena Bravi, Carlo Selmi, Simone Parisi, Fausto Salaffi, Federica Meloni, Enrico Marchioni, Alberto Pesci, Giulia Dei, Marco Confalonieri, Paola Tomietto, Laura Nuno, Francesco Bonella, Nicolò Pipitone, Antonio Mera-Valera, Nair Perez-Gomez, Simone Gerzeli, Raquel Lopez-Mejias, Carlo Jorge Matos-Costa, Jose Antonio Pereira da Silva, José Cifrian, Claudia Alpini, Ignazio Olivieri, María Ángeles Blázquez Cañamero, Ana Belén Rodriguez Cambrón, Santos Castañeda, Lorenzo Cavagna
OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2)...
July 26, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28752373/skin-manifestations-of-rheumatoid-arthritis-juvenile-idiopathic-arthritis-and-spondyloarthritides
#10
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...
July 27, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28712039/cutaneous-manifestations-of-scleroderma-and-scleroderma-like-disorders-a-comprehensive-review
#11
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...
July 16, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28705597/-systemic-sclerosis-and-occupational-difficulties-results-of-a-prospective-study
#12
N Peres, S Morell-Dubois, E Hachulla, P Y Hatron, A Duhamel, D Godard, A S Tellart-Mamert, M Lambert, D Launay, S Fantoni-Quinton
Between 9000 and 14000 people have systemic sclerosis (ScS) in France. The work is often affected. Our study aims to assess the frequency of professional difficulties (DP) of scleroderma patients, identify these DP, the symptoms involved and the solutions used. A heteroquestionnaire was offered to scleroderma patients the Lille University Hospital. Data were cross-checked with the clinical database of the National Observatory of scleroderma patients. We used 104 questionnaires of which 84% are women. A total of 62...
July 10, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28704466/hand-arm-vibration-and-the-risk-of-vascular-and-neurological-diseases-a-systematic-review-and-meta-analysis
#13
Tohr Nilsson, Jens Wahlström, Lage Burström
BACKGROUND: Increased occurrence of Raynaud's phenomenon, neurosensory injury and carpal tunnel syndrome has been reported for more than 100 years in association with work with vibrating machines. The current risk prediction modelling (ISO-5349) for "Raynaud's phenomenon" is based on a few studies published 70 to 40 years ago. There are no corresponding risk prediction models for neurosensory injury or carpal tunnel syndrome, nor any systematic reviews comprising a statistical synthesis (meta-analysis) of the evidence...
2017: PloS One
https://www.readbyqxmd.com/read/28664836/the-use-of-hand-perfusion-scintigraphy-to-assess-raynaud-s-phenomenon-associated-with-hand-arm-vibration-syndrome
#14
Kyung-Ann Lee, Hyung Woo Chung, Sang-Heon Lee, Hae-Rim Kim
OBJECTIVES: This study aimed to evaluate the hand perfusion scintigraphic features of hand-arm vibration syndrome (HAVS) and to compare these with the features of primary and secondary Raynaud's phenomenon (RP) associated with systemic sclerosis (SSc). METHODS: Hand perfusion scintigraphy was performed in 57 patients with primary RP, 71 patients with HAVS-related RP, and 15 patients with SSc-related RP. We calculated 6 ratios: chilled to ambient hand and wrist ratios of the first peak height, initial slope, and blood pool uptake...
June 30, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28658830/blue-finger-syndrome-an-unusual-presentation-of-rheumatoid-arthritis
#15
Robin George Manappallil, Jishnu Jayaraj
Blue Finger Syndrome (BFS) is a benign and rare condition with an idiopathic aetiology. It is characterised by an acute bluish discoloration of fingers which may be accompanied by pain. This is a case of a middle aged female who presented with painless bluish discoloration of right hand and was diagnosed to have BFS. Though BFS is idiopathic, our patient on evaluation was found to have an underlying Rheumatoid Arthritis (RA). Patients with RA are subject to Raynaud's phenomenon; but BFS presenting in a patient with RA is a rare scenario...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28649388/systemic-sclerosis-due-to-crystalline-silica-exposure-among-jewelry-workers-in-korea-two-case-reports
#16
Jae Yoon Kim, Sang Yoon Do, Young Hoon Moon, Chul Gab Lee, Yun Sung Kim, Byung Soon Choi, Eun-A Kim, Han Soo Song
BACKGROUND: Occupational exposure to crystalline silica is a potential risk factor for various systemic autoimmune diseases including systemic sclerosis. The etiology of systemic sclerosis is not conclusively known, but there are epidemiological studies that show the relationship between exposure to crystalline silica and risk of systemic sclerosis. Here we report, for the first time, two cases of crystalline silica-related systemic sclerosis in patients who worked in crystal processing in the jewelry-manufacturing field...
2017: Annals of Occupational and Environmental Medicine
https://www.readbyqxmd.com/read/28645243/extended-periarterial-sympathectomy-evaluation-of-long-term-outcomes
#17
Collier S Pace, Wyndell H Merritt
BACKGROUND: Periarterial sympathectomy is a proposed surgical treatment for patients with refractory Raynaud syndrome; however, there is debate regarding the indications and extent of dissection. Due to the segmental arterial sympathetic innervation, we favor an extended sympathectomy in concert with vein graft reconstruction of occluded vessels when necessary. The purpose of this study is to examine outcomes of extended periarterial sympathectomy in our patients. METHODS: A retrospective chart review was performed on 46 patients who underwent 58 periarterial sympathectomies (12 bilateral) since 1981...
June 1, 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28633610/popliteal-artery-entrapment-syndrome-presenting-as-raynaud-phenomenon
#18
Braden Passias, Sean Lyden, Amanda Frederick, Michael Jolly, Mitchell J Silver
No abstract text is available yet for this article.
June 1, 2017: Vascular Medicine
https://www.readbyqxmd.com/read/28628467/juvenile-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study-in-a-large-cohort-from-the-spanish-society-of-rheumatology-lupus-registry-relesser
#19
Vicenç Torrente-Segarra, Tarek Carlos Salman Monte, Iñigo Rúa-Figueroa, Fernando Sánchez-Alonso, Francisco Javier López-Longo, María Galindo-Izquierdo, Jaime Calvo-Alén, Alejandro Olivé-Marqués, Jesús Ibañez-Ruán, Loreto Horcada, Ana Sánchez-Atrio, Carlos Montilla, Rafael Benito Melero González, Elvira Díez-Álvarez, Victor Martinez-Taboada, José Luis Andreu, Olaia Fernández-Berrizbeitia, José Ángel Hernández-Beriain, Marian Gantes, Blanca Hernández-Cruz, Ángela Pecondón-Español, Carlos Marras, Gema Bonilla, José M Pego-Reigosa
OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years...
June 12, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28624897/corrosion-of-harrington-rod-in-idiopathic-scoliosis-long-term-effects
#20
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
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