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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
Dileep Unnikrishnan, Nasreen Shaikh, Ahmad Sharayah, Chandler Patton
A 47-year-old woman with a medical history of Raynaud's phenomenon presented with fever, cough and shortness of breath. She was found to have left lower lobe consolidation and pleural effusion and was treated as a case of pneumonia. During the hospital course, her respiratory status worsened, and she was intubated on the third hospital day. To investigate the high A-a gradient, a Computerized Tomographic Pulmonary Embolism (CTPE) study was done which identified a large left lower pulmonary artery embolism. She was also found to have a new murmur, and an echocardiogram demonstrated a large lesion on tricuspid valve...
March 13, 2018: BMJ Case Reports
Steven M Dean
In the contemporary era of medical diagnosis via sophisticated radiographic imaging and/or comprehensive serological testing, a focused physical examination remains paramount in recognizing the cutaneous manifestations of chronic vascular disease. Recognition of the unique cutaneous signs of lymphatic and venous hypertension assists in the diagnosis as well as the staging and classification of both lymphedema and chronic venous insufficiency. Awareness of explicit dermatologic vasomotor manifestations aids not only in the identification of acrocyanosis, Raynaud phenomenon, pernio, and erythromelalgia but also mitigates confusion related to their clinical overlap...
March 10, 2018: Progress in Cardiovascular Diseases
Joseph M Kheir, Carla J Guthridge, Jonathon R Johnston, Lucas J Adams, Astrid Rasmussen, Timothy F Gross, Melissa E Munroe, Rebecka L Bourn, Kathy L Sivils, Joel M Guthridge, Michael H Weisman, Daniel J Wallace, Juan-Manuel Anaya, Adriana Rojas Villarraga, James N Jarvis, John B Harley, Judith A James
Objective: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with varied morbidity and mortality. We assessed clinical presentations, autoantibody specificities and therapeutic interventions in Native American (NA) patients with SLE. Methods: Patients with SLE meeting 1997 American College of Rheumatology classification criteria (n=3148) were enrolled between 1992 and 2010 in the multiethnic, cross-sectional Lupus Family Registry and Repository...
2018: Lupus Science & Medicine
Maurizio Cutolo, Amelia C Trombetta, Karin Melsens, Carmen Pizzorni, Alberto Sulli, Barbara Ruaro, Sabrina Paolino, Ellen Deschepper, Vanessa Smith
BACKGROUND: Absolute nailfold capillary number should be a putative biomarker in selected rheumatic diseases but could be time consuming and not highly repeatable. OBJECTIVE: To validate an automated software for absolute nailfold capillary number and density evaluation, on nailfold videocapillaroscopy (NVC) images in systemic sclerosis (SSc). METHODS: An automated software to count nailfold capillary number (AUTOCAPI) had been constructed, through an exploratory image set...
March 12, 2018: Microcirculation: the Official Journal of the Microcirculatory Society, Inc
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
Kiran Dhaliwal, Michelle Griffin, Christopher P Denton, Peter E M Butler
Raynaud's phenomenon is a vasospastic disorder of the digital vessels triggered by exposure to cold or stress. It is most commonly observed in the hands, but also frequently affects the toes. We present three cases of patients with severe Raynaud's phenomenon in the toes, secondary to scleroderma. The diagnosis of Raynaud's syndrome and scleroderma was established according to the 2010 American College of Rheumatology and European League Against Rheumatism criteria. Patients were treated with 10 units of botulinum toxin injected into each foot...
March 9, 2018: BMJ Case Reports
Shintaro Watanuki, Taku Kikuchi, Takaaki Toyama, Ryohei Abe, Hitomi Nakayama, Daiki Karigane, Takayuki Shimizu, Jun Kikuchi, Kotaro Matsumoto, Hidetaka Yasuoka, Masaharu Kataoka, Shinichiro Okamoto, Takehiko Mori
A 37-year-old woman was diagnosed with chronic phase chronic myeloid leukemia. Nilotinib treatment was initiated; however, it had to be discontinued due to an allergic reaction one month later, and dasatinib treatment was provided. Although favorable response was obtained, she started complaining of shortness of breath 7 months after initiating dasatinib treatment. Chest X-ray and echocardiography indicated pulmonary congestion and hypertension. Further, she was diagnosed with mixed connective tissue disease (MCTD) based on Raynaud phenomenon, swollen fingers, sclerodactyly, pancytopenia, hypocomplementemia, and positive anti-U1-RNP antibody...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Christopher P Denton, Ariane L Herrick
Pauling et al highlight some of the limitations of using the Raynaud's Condition Score (RCS) in clinical trials of Raynaud's phenomenon and the need for better patient reported outcome measures. They have explored this in a survey of patients with systemic sclerosis who completed the RCS and although the number of cases in the survey is small, and possibly not representative of a clinical trial population, the points raised are interesting.We acknowledge the difficulties of undertaking clinical trials in Raynaud's phenomenon...
March 7, 2018: Arthritis & Rheumatology
Anna Tjärnlund, Matteo Bottai, Ingrid E Lundberg
We have with great interest read the letter titled "Comments on the "2017 EULAR/ACR Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups". Points of concern", by Dr Castañeda et al published in your journal [1]. The authors discuss the antisynthetase syndrome (ASSD), a condition characterized by myositis, arthritis, interstitial lung disease (ILD), Raynaud's phenomenon and the presence of autoantibodies targeting aminoacyl transfer RNA synthetases, and the fact that this group was not included in the 2017 EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups [2]...
March 7, 2018: Arthritis & Rheumatology
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
Lea Estermann, Pascal Ducommun, Isabelle Steurer-Dober, Urs Hug
The hypothenar hammer syndrome (HHS) is a rare entity of secondary Raynaud's phenomenon. The blunt hypothenar trauma causes a lesion of the vessel wall with a consecutive thrombosis or aneurysm of the ulnar artery at the Guyon's canal. Different risk factors are discussed such as nicotine abuse, or a muscle anomaly in the Guyon's canal. To date, there are five case reports published about muscle anomalies and HHS. We present a case of a 51-year-old shipbuilder with a unilateral HHS on his right dominant hand with a bilateral muscle anomaly...
March 6, 2018: Archives of Orthopaedic and Trauma Surgery
Rita de Sousa Gameiro, Ana Isabel Alves Reis, Ana Cristina Grilo, Carla Noronha
Mixed connective tissue disease (MCDT) is a rare condition characterised by the presence of high titres of anti-U1 ribonucleoprotein antibodies and selected clinical features of systemic lupus erythematosus, systemic sclerosis and polymyositis/dermatomyositis. Early symptoms are non-specific, including easy fatigability, myalgia, arthralgia and Raynaud's phenomenon. Some reports emphasised the favourable outcome and excellent response to glucocorticoids, but there are contradictory studies reporting worse prognosis...
March 5, 2018: BMJ Case Reports
Olivier Bouillanne, Jean-Claude Melchior, Cécile Faure, Muriel Paul, Florence Canouï-Poitrine, Yves Boirie, Didier Chevenne, Christine Forasassi, Ester Guery, Stéphane Herbaud, Philippe Le Corvoisier, Nathalie Neveux, Valérie Nivet-Antoine, Alain Astier, Agathe Raynaud-Simon, Stéphane Walrand, Luc Cynober, Christian Aussel
BACKGROUND: Citrulline (CIT), is not extracted by the splanchnic area, can stimulate muscle protein synthesis and could potentially find clinical applications in conditions involving low amino acid (AA) intake, such as in malnourished older subjects. OBJECTIVE: Our purpose was to research the effects of CIT supplementation on protein metabolism in particular on non-oxidative leucine disposal (NOLD, primary endpoint), and splanchnic extraction of amino acids in malnourished older patients...
February 21, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
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
Robert Benamouzig, Mourad Benallaoua, Jean-Christophe Saurin, Marouane Boubaya, Christophe Cellier, René Laugier, Magalie Vincent, Christian Boustière, Rodica Gincul, Elia Samaha, Philippe Grandval, Thomas Aparicio, Gheorghe Airinei, Bakhtiar Bejou, Cyriaque Bon, Jean-Jacques Raynaud, Vincent Levy, Denis Sautereau
Background: Gastrointestinal angiodysplasias (GIADs) could be responsible for recurrent bleeding and severe anemia. Somatostatin analogs could reduce transfusion requirements in these patients but no randomized controlled study is available. The main objective of the ANGIOPAS phase II double-blinded randomized, noncomparative study was to assess the effectiveness of pasireotide-LAR in reducing transfusion requirements in patients with refractory GIADs bleeding. Methods: A total of 22 patients with transfusion requirements ⩾6 units of packed red blood cells (pRBCs) during the 6 months prior to inclusion were randomized to receive pasireotide-LAR 60 mg ( n = 10) or placebo ( n = 12) every 28 days for 6 months...
2018: Therapeutic Advances in Gastroenterology
Marie-France Mamzer, Sophie Dubois, Christian Saout, Nicolas Albin, Jehan-Michel Béhier, Anne Buisson, Vincent Diebolt, Olivier Delaitre, Christophe Duguet, Jean-Yves Fagon, Ségolène Gaillard, Claire Le Jeunne, René Mazars, Joëlle Micallef, Hervé Nabarette, Laurent Piazza, Catherine Raynaud, Nathalie Varoqueaux
The constant development of health technologies, combined with the increase in the cost of treatment, means that States must continually make choices about the introduction of new technologies into their healthcare system and how they are to be funded. In France, the systematic participation of patients in these processes is one of the targets to be met in terms of healthcare democracy. Although, on an international level, patient involvement in these assessments is constantly growing, it is difficult to define due to the presence of unstabilised elements in terms of both terminology and assessment methods...
December 21, 2017: Thérapie
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
S Bellando-Randone, C Bruni, G Lepri, G Fiori, F Bartoli, M L Conforti, A Moggi-Pignone, S Guiducci, D Giuggioli, M Colaci, A Spinella, C Ferri, M Matucci-Cerinic
Iloprost (ILO) is employed intravenously for the treatment of severe Raynaud phenomenon (RP) and digital ulcers (DU) in systemic sclerosis (SSc). The aim of this study was to evaluate the safety and tolerability of the intravenous treatment with ILO in different phases of SSc. Eighty-one consecutive non-selected SSc patients, all on nifedipine, with moderate RP, treated with ILO infusion, were retrospectively evaluated. Patients were sub classified according to the edematous or fibrotic/atrophic cutaneous phase of the disease...
February 22, 2018: Clinical Rheumatology
Jasmine Rana, Andrea Primiani Moy, Adriano Piris, Gideon P Smith
A 28-year-old man with clinically and laboratory diagnosed anti-PL-12 anti-synthetase syndrome (AS) in 2009 developed cutaneous lupus lesions, discoid lupus lesions, and sclerodacytly with finger-tip ulcerations four years following his AS diagnosis. Laboratory tests including +ANA, +anti-dsDNA antibody, +anti-Smith antibody, and +anti-RNP antibody in 2014 confirmed the diagnosis of progression to an overlap syndrome including systemic lupus erythematosus. The patient now also has clinical findings (sclerodacytly, Raynaud phenomenon, finger-tip ulcerations) consistent with scleroderma overlap...
September 15, 2017: Dermatology Online Journal
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