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Aikaterini Patsatsi, Miltiadis Kokolios, Olga Pikou, Vasilios Lambropoulos, Ioannis Efstratiou, Dimitrios Sotiriadis
Regression of congenital nevi is usually associated with loss of pigment or halo formation. In rare cases, regression is characterized by sclerosis and hair loss. We describe a rare case of a sclerotic hypopigmented large congenital melanocytic nevus in which a localized scleroderma-like reaction process of regression seemed to have started in utero and progressed throughout early childhood.
October 25, 2016: Pediatric Dermatology
Terek Wehbe, Majid Abi Saab, Nassim Abi Chahine, Talar Margossian
We treated 2 patients with progressive, refractory scleroderma with a combined immunotherapy approach using plasmapheresis followed by rituximab and then intravenous umbilical-cord-derived allogeneic mesenchymal stem cells (MSCs). Both patients improved subjectively and objectively for over a year. Upon recurrence of their symptoms, the patients were treated again with allogeneic MSCs alone with a very good response. The combination was well tolerated and effective suggesting a large scale study may be warranted in progressive, refractory Scleroderma...
2016: Stem Cell Investigation
Shantanu Dixit, Chaithra Kalkur, Atul P Sattur, Michael M Bornstein, Fred Melton
BACKGROUND: Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable...
October 24, 2016: Journal of Medical Case Reports
James Esposito, Zoe Brown, Wendy Stevens, Joanne Sahhar, Candice Rabusa, Jane Zochling, Janet Roddy, Jennifer Walker, Susanna M Proudman, Mandana Nikpour
BACKGROUND: In some rheumatic diseases such as systemic lupus erythematosus (SLE), low serum complement ('hypocomplementaemia') is a feature of active disease. However, the role of hypocomplementaemia in systemic sclerosis (SSc) is unknown. We sought to determine the frequency, clinical associations and relationship to disease activity of hypocomplementaemia in SSc. METHODS: The study included 1140 patients fulfilling the 2013 American College of Rheumatology criteria for SSc...
October 22, 2016: Arthritis Research & Therapy
Tien Tay, Nava Ferdowsi, Murray Baron, Wendy Stevens, Marie Hudson, Susanna M Proudman, Mandana Nikpour
OBJECTIVES: To identify and appraise measures of disease status in systemic sclerosis (SSc). METHODS: A systematic review of Medline (1966-2015), EMBASE (1974-2015), and Cochrane Library (inception-2015) was undertaken to identify indices of disease status in SSc. We focused on objective measures and excluded non-English articles. Measures were reviewed for content, whether they measured activity, damage and/or severity and whether they were validated according to the OMERACT filter...
July 29, 2016: Seminars in Arthritis and Rheumatism
Arsenio Spinillo, Fausta Beneventi, Elena Locatelli, Vèronique Ramoni, Roberto Caporali, Claudia Alpini, Giulia Albonico, Chiara Cavagnoli, Carlomaurizio Montecucco
BACKGROUND: The burden of pregnancy complications associated with well defined, already established systemic rheumatic diseases preexisting pregnancy such as rheumatoid arthritis, systemic lupus erythematosus or scleroderma is well known. Systemic rheumatic diseases are characterized by a long natural history with few symptoms, an undifferentiated picture or a remitting course making difficult a timely diagnosis. It has been suggested that screening measures for these diseases could be useful but the impact of unrecognized systemic rheumatic disorders on pregnancy outcome is unknown...
October 18, 2016: BMC Pregnancy and Childbirth
Lauren E Hock, Marinos Kontzialis, Aimee J Szewka
No abstract text is available yet for this article.
October 18, 2016: Neurology
Michael J Ryan
Connective tissue diseases including systemic lupus erythematosus (SLE), rheumatoid arthritis, and scleroderma, have a strong predilection for women and are associated with a marked increase in the prevalence of hypertension. The mechanisms leading to the increased risk of hypertension in these patients remain unclear; however, they are likely related to immune mediated changes in cardiovascular and renal function. Over the past several years, we have elucidated a number of factors that contribute to the development of hypertension during SLE using a widely established experimental model of SLE, the female NZBWF1 mouse...
September 2016: Journal of Hypertension
Bernadette M Lynch, Edward P Stern, Voon Ong, Mark Harber, Aine Burns, Christopher P Denton
The UK Scleroderma Study Group developed guidelines on the diagnosis and management of scleroderma renal crisis (SRC) based on best available evidence and clinical experience. SRC is characterised by the acute onset of severe hypertension and acute kidney injury. Current strategies to reduce the associated morbidity and mortality include identifying at risk patients to aid early diagnosis. ACE inhibitor therapy should be lifelong in all patients, regardless of whether they require renal replacement therapy...
September 2016: Clinical and Experimental Rheumatology
Wakana Nakayama, Masatoshi Jinnin, Yukiko Tomizawa, Kayo Nakamura, Hideo Kudo, Kuniko Inoue, Katsunari Makino, Noritoshi Honda, Ikko Kajihara, Satoshi Fukushima, Hironobu Ihn
OBJECTIVES: The overexpression of IL-12 family cytokines is implicated in the pathogenesis of SSc, but their exact role is still unclear. The aim of this study was to investigate the regulation of extracellular matrix expression by IL-23 and its contribution to the phenotype of SSc. METHODS: The mRNA expression was determined by PCR array and real-time PCR. The expression levels of proteins were determined by immunoblotting and immunohistochemical staining. The effect of IL-23 on dermal fibrosis in vivo was examined in a mouse model of SSc induced by bleomycin injection...
October 15, 2016: Rheumatology
Aseem Sharma, Rahul Ray, Jandhyala Sridhar, Arti Trehan, Manish Khandare
Eosinophilic fasciitis, also known as Shulman's syndrome, is a fibrosing scleroderma-like syndrome, which is a distinct entity. A 55-year-old man, presented with progressive skin darkening, thickening, and tightening over the left lower limb since 6 months. Dermatological examination revealed a hyperpigmented indurated area on the left thigh, extending to the anterior aspect of the left leg. A well-defined hypopigmented indurated plaque was present over the left iliac region. Histopathology and imaging studies confirmed the diagnosis of eosinophilic fasciitis and lichen sclerosus...
September 2016: Indian Dermatology Online Journal
Samaneh Davoudi, Tina Ahmadi, Evangelia Papavasilieou, Ilya Leskov, Lucia Sobrin
PURPOSE: To identify autoantigens in autoimmune retinopathy patients by phage immunoprecipitation sequencing (PhIP-Seq), a new technique for autoantigen discovery. METHODS: PhIP-Seq was used to sequence putative autoantibodies in plasma from 11 patients with autoimmune retinopathy and eight controls. We compared the autoantibodies' molecular weights with those of proteins detected by Western blot. RESULTS: Several autoantigens were found in cases and not detected in the controls...
October 11, 2016: Ocular Immunology and Inflammation
Jirakrit Leelarungrayub, Decha Pinkaew, Khanittha Wonglangka, Wichai Eungpinichpong, Jakkrit Klaphajone
Although previously proposed that chronic scleroderma should be cared for clinically and early rehabilitation should be performed in hospital by a chest physical therapist, little evidence is currently available on its benefits. Therefore, this study demonstrated the benefits of short-term pulmonary rehabilitation during hospitalization in a female patient with chronic scleroderma. The aim of rehabilitation was to improve ventilation and gas exchange by using airway clearance, chest mobilization, and breathing-relearning techniques, including strengthening the respiratory system and the muscles of the limbs by using the BreathMax(®) device and elastic bands...
2016: Clinical Medicine Insights. Circulatory, Respiratory and Pulmonary Medicine
Jennifer J Chia, Tong Zhu, Susan Chyou, Dragos C Dasoveanu, Camila Carballo, Sha Tian, Cynthia M Magro, Scott Rodeo, Robert F Spiera, Nancy H Ruddle, Timothy E McGraw, Jeffrey L Browning, Robert Lafyatis, Jessica K Gordon, Theresa T Lu
Scleroderma is a group of skin-fibrosing diseases for which there are no effective treatments. A feature of the skin fibrosis typical of scleroderma is atrophy of the dermal white adipose tissue (DWAT). Adipose tissue contains adipose-derived mesenchymal stromal cells (ADSCs) that have regenerative and reparative functions; however, whether DWAT atrophy in fibrosis is accompanied by ADSC loss is poorly understood, as are the mechanisms that might maintain ADSC survival in fibrotic skin. Here, we have shown that DWAT ADSC numbers were reduced, likely because of cell death, in 2 murine models of scleroderma skin fibrosis...
October 10, 2016: Journal of Clinical Investigation
Jitesh Ahuja, Deepika Arora, Jeffrey P Kanne, Travis S Henry, J David Godwin
Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia...
November 2016: Radiologic Clinics of North America
Laura Groseanu, Violeta Bojinca, Tania Gudu, Ioana Saulescu, Denisa Predeteanu, Andra Balanescu, Florian Berghea, Daniela Opris, Andreea Borangiu, Cosmin Constantinescu, Magda Negru, Ruxandra Ionescu
OBJECTIVE: Vitamin D has pleiotropic effects including immunomodulatory, cardioprotective, and antifibrotic properties and is thus able to modulate the three main links in scleroderma pathogenesis. The aim of the study was to evaluate the level of vitamin D in patients with systemic sclerosis and to analyze the associations between the concentration of vitamin D and the features of systemic sclerosis. MATERIAL AND METHODS: Fifty-one consecutive patients were evaluated for visceral involvement, immunological profile, activity, severity scores, and quality of life...
June 2016: Eur J Rheumatol
Yusuf Savran, Sevgi Akarsu
Scleromyxedema is an uncommon connective tissue disease characterized by mucin deposits, fibrosis, and proliferation of fibroblasts in the dermis. Although it shares similar sclerodermoid features, it is a different clinical entity than scleroderma. A monoclonal gammopathy is almost always present; however, progression to multiple myeloma is rare. It may have many systemic manifestations, of which the most notable being the dermato-neuro syndrome because of its rarity and potential fatal outcome. We present a case of a 50-year-old woman with scleromyxedema in whom the dermato-neuro syndrome developed...
December 2015: Eur J Rheumatol
Saleh Saleh Elessawy, Eman Muhammad Abdelsalam, Eman Abdel Razek, Samar Tharwat
BACKGROUND: Conventional magnetic resonance imaging (MRI) is a highly valuable tool for full assessment of the extent of bilateral symmetrical diffuse inflammatory myopathy, owing to its high sensitivity in the detection of edema which correlates with, and sometimes precedes, clinical findings. PURPOSE: To evaluate the use of whole-body (WB)-MRI in characterization and full assessment of the extent and distribution of diffuse inflammatory myopathy. MATERIAL AND METHODS: A prospective study on 15 patients presenting with clinical evidence of inflammatory myopathy...
September 2016: Acta Radiologica Open
Mariachiara Arisi, Ilaria Cavazzana, Maria E Cerutti, Fabio Ferrari, Nice Carabellese, Maria T Rossi, Angela Tincani, Pier G Calzavara-Pinton, Franco Franceschini
BACKGROUND: Morphea is a rare fibrosing skin disorder. Antinuclear antibodies (ANA), antihistone and rheumatoid factor are detected in high rate of morphea cases. Scleroderma-related antibodies are usually absent. METHODS: 21 adult patients affected by morphea were examined at the time of enrollment and after 6 months with the assessment of clinical outcome measures of disease severity and damage. Healthy subjects were considered as normal controls while patients affected by systemic sclerosis and other connective tissue diseases (CTD) were considered as pathological controls...
October 5, 2016: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Eliza R Pelrine, Marie-Dominique Ah-Kioon, Meng Zhang, Franck J Barrat, Robert F Spiera, Jessica K Gordon
BACKGROUND: Systemic sclerosis (SSc) is characterized by a wide variety of symptoms and disease manifestations including joint pain, gastrointestinal dysfunction, interstitial lung disease, and cardiomyopathy. QUESTIONS/PURPOSES: Using the Scleroderma Health Assessment Questionnaire (SHAQ) and Short Form-36 (SF-36) we explored how patient-reported physical health, mental health, and functional status related to these clinical characteristics and to cytokine levels utilizing the Hospital for Special Surgery Scleroderma Registry...
October 2016: HSS Journal: the Musculoskeletal Journal of Hospital for Special Surgery
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