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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
#1
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/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#2
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/28606232/-a-clinical-analysis-of-15-children-with-systemic-lupus-erythematosus-accompanied-by-pulmonary-hypertension
#3
Ji Li, Jing-Ran Ma, Zhi-Xing Sun, Jing-Jing Jiang, Yan-Qing Dong, Qian Wang, Hong-Mei Song
OBJECTIVE: To evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus (SLE) accompanied by pulmonary hypertension (PH). METHODS: The clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed. RESULTS: Among the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0...
June 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28606036/scleroderma-like-disorders
#4
Amit Sharma
Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis. There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis. These are termed as "scleroderma variants" or "scleroderma like disorders"...
June 11, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28602360/the-role-of-platelets-in-autoimmunity-vasculopathy-and-fibrosis-implications-for-systemic-sclerosis
#5
REVIEW
Konstantinos Ntelis, Elena E Solomou, Lazaros Sakkas, Stamatis-Nick Liossis, Dimitrios Daoussis
INTRODUCTION: Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy, autoimmunity, and widespread dermal and visceral fibrosis. This article summarizes the current knowledge about the potential contribution of platelets in the disease process and the rationale of targeting platelets as an adjunct treatment for SSc. METHODS: We performed an electronic search (Medline) using the keywords platelets, systemic sclerosis, autoimmunity, fibrosis, Raynaud, and pulmonary arterial hypertension...
May 23, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28595537/capillaroscopic-findings-in-primary-fibromyalgia
#6
Sevdalina Lambova, Ulf Müller-Ladner
: Introduction: Although Raynaud&#039;s phenomenon (RP) is observed in a significant proportion of patients with primary fibromyalgia, the available data on capillaroscopic findings in primary fibromyalgia are scarce. OBJECTIVE: The aim of the study was to evaluate the capillaroscopic pattern in patients with primary fibromyalgia. PATIENTS AND METHODS: 26 patients with primary fibromyalgia (25 women and 1 man) were included in the study. Mean age was 55±10 years...
June 7, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28586844/skeletal-muscle-involvement-in-antisynthetase-syndrome
#7
Eri Noguchi, Akinori Uruha, Shigeaki Suzuki, Kohei Hamanaka, Yuko Ohnuki, Jun Tsugawa, Yurika Watanabe, Jin Nakahara, Takashi Shiina, Norihiro Suzuki, Ichizo Nishino
Importance: Antisynthetase syndrome, characterized by myositis, interstitial lung disease, skin rash, arthropathy, and Raynaud phenomenon, is a clinical entity based on the presence of aminoacyl transfer RNA synthetase (ARS) antibodies in patients' serum. However, antisynthetase syndrome is not included in the histological subsets of idiopathic inflammatory myopathies. Objective: To elucidate the clinical features of myositis in patients with antisynthetase syndrome...
June 5, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28580766/pharmacological-stress-rest-perfusion-and-delayed-enhancement-cardiac-magnetic-resonance-identifies-a-very-early-cardiac-involvement-in-systemic-sclerosis-patients-of-recent-onset
#8
Roberto Giacomelli, Ernesto Di Cesare, Paola Cipriani, Piero Ruscitti, Alessandra Di Sibio, Vasiliki Liakouli, Antonio Gennarelli, Francesco Carubbi, Alessandra Splendiani, Onorina Berardicurti, Paola Di Benedetto, Francesco Ciccia, Giuliana Guggino, Ganna Radchenko, Giovanni Triolo, Carlo Masciocchi
OBJECTIVE: To evaluate occult cardiac involvement in asymptomatic systemic sclerosis (SSc) patients by pharmacological stress, rest perfusion and delayed enhancement cardiac magnetic resonance (CMR), for a very early identification of patients at higher risk of cardiac-related mortality. METHODS: Sixteen consecutive patients with definite SSc, fulfilling the American College of Rheumatology/European League Against Rheumatism 2013 classification criteria in less than 1 year from the onset of Raynaud's phenomenon, underwent pharmacological stress, rest perfusion and delayed enhancement CMR...
June 4, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28578493/nailfold-videocapillaroscopy-results-in-patients-with-rheumatoid-arthritis
#9
Sinem Sag, Mustafa Serdar Sag, Ibrahim Tekeoglu, Ayhan Kamanli, Kemal Nas, Yıldıray Aydın
We aimed to analyse the nailfold capillaryscopy findings morphologically and examine their relationship with disease activity and demographic characteristics in patients with rheumatoid arthritis. In accordance with the 2010 ACR/EULAR classification criteria, 201 patients diagnosed with Romatoiad artrit (RA) and 50 healthy controls were included. We analysed capillaroscopic abnormalities such asmegacapillaries, haemorrhages, ramifications and avascular areas in patients affected with rheumatoid arthritis. The findings in our study are as follows: in 45...
June 4, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28564618/very-early-and-early-systemic-sclerosis-in-the-spanish-scleroderma-registry-rescle-cohort
#10
REVIEW
Luis Trapiella-Martínez, José Bernardino Díaz-López, Luis Caminal-Montero, Carles Tolosa-Vilella, Alfredo Guilléndel Castillo, Dolores Colunga-Argüelles, Manuel Rubio-Rivas, Nerea Iniesta-Arandia, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, Jose Antonio Vargas-Hitos, Juan José Ríos-Blanco, Jose Antonio Todolí-Parra, Mónica Rodríguez-Carballeira, Adela Marín-Ballvé, Antonio Javier Chamorro-Fernández, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñóz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar
OBJECTIVES: According to the existence of subclinical organ involvement pre-scleroderma should be divided into two subsets: very early and early disease. Pre-scleroderma patients included in the Spanish Scleroderma Registry (RESCLE) Cohort were reclassified into subsets. Differences were evaluated and the risk of progression to definite systemic sclerosis was estimated. METHODS: The characteristics of very early and early SSc patients were compared. A logistic regression model was used to determine the risk factors of progression...
May 28, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28539684/coexistence-of-diabetes-mellitus-type-1-with-diffuse-systemic-sclerosis-case-report-and-literature-review
#11
Ewa Wielosz, Maria Kurowska, Dorota Suszek, Maria Majdan
Diabetic sclerodactyly is a frequently recognized skin finding that may occur in patients with diabetes mellitus but coexistence of diabetes and systemic sclerosis is rare. We describe a case of coexistence of type 1 diabetes mellitus and systemic sclerosis in 42-year-old man with the history of Raynaud's phenomenon, progressive diffuse hardening of the skin and sclerodactyly, slowly worsening with time. The medical history included type 1 diabetes since childhood with microvascular complications. The patient presented a typical capillaroscopic scleroderma-like pattern, antinuclear antibodies and sclerotic lesions in gastrointestinal system...
2017: Reumatologia
https://www.readbyqxmd.com/read/28536935/-rituximab-for-the-treatment-of-poly-and-dermatomyositis-results-from-the-graid-2-registry
#12
C Fiehn, L Unger, H Schulze-Koops, F Proft, J C Henes, A Jacobi, T Dörner
INTRODUCTION: In the treatment of poly- and dermatomyositis, only a limited number of treatment modalities are established. OBJECTIVE: The goal of the GRAID-2 registry was to study off-label use of biologic drugs for this indication in Germany. PATIENTS AND METHODS: Analysis of the data of the GRAID-2 registry for poly- and dermatomyositis. RESULTS: In 22 of the 23 patients in the GRAID-2 registry, rituximab (RIX) was administered, while 1 patient was given tocilizumab as off-label therapy...
May 23, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28523890/handy-hints-about-raynaud-s-phenomenon-in-children-a-critical-review
#13
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/28514385/-the-effects-of-diosmin-and-hesperidin-on-capillary-blood-flow-of-upper-limbs-in-patients-with-secondary-raynaud-s-syndrome
#14
A M Zudin, A G Gritsenko, I T Hadzhishvili
The article discusses the effects of diosmin and hesperidin on capillary blood flow in patients with secondary Raynaud's syndrome (RS). Raynaud's syndrome a difficult problem of modern angiology, since in its development there is a large range of disorders of the capillary blood flow of the limbs. Currently, the main way of therapy in patients with secondary Raynaud's syndrome is the use of either calcium channel blockers (if angiospastic stage of the disease), or prostaglandins of group E1 (with tropho-paralytic stage of secondary RS)...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28507447/managing-refractory-cryoglobulinemic-vasculitis-challenges-and-solutions
#15
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/28503297/cardiovascular-involvement-and-manifestations-of-systemic-chikungunya-virus-infection-a-systematic-review
#16
María Fernanda Alvarez, Adrián Bolívar-Mejía, Alfonso J Rodriguez-Morales, Eduardo Ramirez-Vallejo
BACKGROUND: In the last three years, chikungunya virus disease has been spreading, affecting particularly the Americas, producing more than two million cases. In this setting, not only new disease-related epidemiological patterns have been found, but also new clinical findings have been reported by different research groups. These include findings on the cardiovascular system, including clinical, electrocardiographic and echocardiographic alterations. METHODS: We performed a systematic review looking for reports about cardiovascular compromise during chikungunya disease...
2017: F1000Research
https://www.readbyqxmd.com/read/28502960/circulating-anti-nuclear-antibodies-in-systemic-sclerosis-utility-in-diagnosis-and-disease-subsetting
#17
Masataka Kuwana
The presence of circulating anti-nuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). Currently, a variety of SSc-specific ANAs, including anticentromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies, have been well characterized, and their commercial kits are available worldwide. Since these autoantibodies are specifically detected in SSc patients and are associated with unique sets of disease manifestations, they are widely used in routine clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvements and prognosis...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/28479486/integrated-diagnosis-project-for-inflammatory-myopathies-an-association-between-autoantibodies-and-muscle-pathology
#18
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...
May 4, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28476103/intracranial-lesion-as-onset-symptom-in-a-patient-with-early-undifferentiated-connective-tissue-disease-a-case-report
#19
Ying Du, Chuan Li, Dai-di Zhao, Jia-Rui Lu, Wei Zhang, Zhu-Yi Li
BACKGROUND: Undifferentiated connective tissue disease (UCTD) is widely considered to be a distinct clinical entity, and now divided into two subgroups: stable UCTD and early UCTD. The most frequent onset symptoms of UCTD include arthralgias, arthritis, Raynaud's phenomenon, mucocutaneous involvement, and sicca symptoms. However, Neurologic involvement is rare, and intracranial lesion as onset symptom in a patient with early UCTD has not yet been reported. CASE PRESENTATION: A 51-year-old Chinese female experienced progressive left leg weakness for 14 days before hospitalizing in our department...
May 5, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28466365/women-with-silicone-breast-implants-and-autoimmune-inflammatory-syndrome-induced-by-adjuvants-description-of-three-patients-and-a-critical-review-of-the-literature
#20
REVIEW
Slavica Pavlov-Dolijanovic, Nada Vujasinovic Stupar
Silicone has been widely used in the manufacture of medical implants. It is well tolerated in most cases. However, in this paper we report the cases of three women who developed autoimmune/inflammatory syndrome induced by adjuvants (ASIA syndrome), namely with silicone breast implants. The symptoms in these cases include arthralgia, arthritis, myalgia, sleep disturbances, the appearance of autoantibodies, miscarriage, Raynaud's phenomenon, and involvement of autoimmune diseases (scleroderma and undifferentiated connective tissue diseases)...
May 2, 2017: Rheumatology International
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