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Shubhra Jain, Vinod Joshi, Yogendra S Rathore, Narendra Khippal
Several occupational hazards, especially exposure to silica, have been implicated as causal factors for the development of scleroderma-like disorders. Compared to other connective tissue disorders, silica-associated systemic sclerosis (SA-SS) is relatively rare. Silica-induced scleroderma is indistinguishable from idiopathic systemic sclerosis. However, the former expresses a high predisposition of pulmonary involvement and anti-Scl-70 antibody. We report the case of a 42-year-old male, stone cutter by occupation, who was diagnosed as simple chronic silicosis and developed systemic sclerosis...
May 2017: Indian Journal of Occupational and Environmental Medicine
Arti Nanda, Maitham A A Husain, Waleed Al-Herz, Adla Almekaimi, Humoud Al-Sabah, Mohammad Al-Otaibi
BACKGROUND/OBJECTIVES: Allogeneic hematopoietic stem cell transplantation (HSCT) is a treatment option for many life-threatening disorders in children. Chronic graft-versus-host disease (cGVHD) is a significant complication of HSCT, and its treatment is challenging. Skin is the most common organ affected in cGVHD, with protean manifestations posing a challenge in diagnosis and management. The objective was to have a better understanding of the spectrum of chronic cutaneous GVHD (cc-GVHD) in children...
March 14, 2018: Pediatric Dermatology
Seynabou Séne, Marc-André Selosse, Mathieu Forget, Josie Lambourdière, Khoudia Cissé, Abdala Gamby Diédhiou, Elsie Rivera-Ocasio, Hippolyte Kodja, Norikazu Kameyama, Kazuhide Nara, Lucie Vincenot, Jean-Louis Mansot, Jean Weber, Mélanie Roy, Samba Ndao Sylla, Amadou Bâ
Global trade increases plant introductions, but joint introduction of associated microbes is overlooked. We analyzed the ectomycorrhizal fungi of a Caribbean beach tree, seagrape (Coccoloba uvifera, Polygonacaeae), introduced pantropically to stabilize coastal soils and produce edible fruits. Seagrape displays a limited symbiont diversity in the Caribbean. In five regions of introduction (Brazil, Japan, Malaysia, Réunion and Senegal), molecular barcoding showed that seagrape mostly or exclusively associates with Scleroderma species (Basidiomycota) that were hitherto only known from Caribbean seagrape stands...
March 13, 2018: ISME Journal
Sakeen W Kashem, Colleen K Correll, Richard K Vehe, Patricia M Hobday, Bryce A Binstadt, Sheilagh M Maguiness
BACKGROUND: Morphea, or 'localized scleroderma', is an inflammatory disorder resulting in fibrosis of the skin and subcutaneous tissues. Joint contractures, arthralgias and functional compromise are recognized associations of pediatric morphea. The co-existence of inflammatory arthritis and morphea is not well-described in the literature. OBJECTIVE: To investigate the relationship between pediatric morphea and inflammatory arthritis with regards to cutaneous, musculoskeletal and laboratory findings and treatment regimens...
March 10, 2018: Journal of the American Academy of Dermatology
Divya Singh, Anita Ganger, Noopur Gupta, Murugesan Vanathi, Rajesh Khadgawat, Radhika Tandon
Here, we present, to the best of our knowledge, the first case of Werner syndrome with corneal blindness due to bilateral primary bullous keratopathy. Werner syndrome is a rare autosomal recessive disorder characterized by features of premature aging, insulin-dependent diabetes mellitus, osteoporosis, atherosclerosis, hypergonadotrophic hypogonadism, hypertriglyceridemia, scleroderma-like skin changes, and sarcomas. Among ocular manifestations, cataracts, cystoid macular edema, and retinal detachment have been reported...
March 9, 2018: Experimental and Clinical Transplantation
Yongxia Wu, Steven Schutt, Katelyn Paz, Mengmeng Zhang, Ryan P Flynn, David Bastian, M Hanief Sofi, Hung Nguyen, Min Dai, Chen Liu, Ying-Jun Chang, Bruce R Blazar, Xue-Zhong Yu
Chronic graft-versus-host disease (cGVHD) is characterized as autoimmune-like fibrosis and antibody production mediated by pathogenic T-cells and B-cells. MicroRNA (miR)-17-92 influences the survival, differentiation and function of lymphocytes in cancer, infections and autoimmunity. To determine whether miR-17-92 regulates T- and B-cell responses in cGVHD, we generated mice conditionally deficient for miR-17-92 in T-cells, B-cells, or both. Using murine models of allogeneic bone marrow transplantation (allo-BMT), we demonstrate that expression of miR-17-92 in donor T- and B-cells is essential for the induction of both scleroderma and bronchiolitis obliterans in cGVHD...
March 12, 2018: Blood
Aina Venkatasamy, Quentin Minault, Benoit Romain, Francis Veillon
No abstract text is available yet for this article.
March 10, 2018: Abdominal Radiology
Yossra A Suliman, Suzanne Kafaja, John Fitzgerald, Ximena Wortsman, Jonathan Grotts, Marco Matucci-Cerrinic, Veena K Ranganath, Daniel E Furst
Skin ulcers in scleroderma (SSc) patients are considered a major challenge, both in clinical assessment and treatment decisions. The objective of our study is to assess ultrasonographic (US) morphology of skin ulcers in SSc patients and evaluate if US will be of value in enhancing our clinical information and influence our management plans. We examined a convenience sample of 21 skin ulcers reported in 10 SSc patients by US. We used a previously published US definition of normal skin and developed a preliminary US definition of skin ulcer...
March 10, 2018: Clinical Rheumatology
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
Emelina Preis, Kristina Franz, Elise Siegert, Alexander Makowka, Christine March, Gabriela Riemekasten, Emanuele Cereda, Kristina Norman
BACKGROUND/OBJECTIVES: Our objective was to investigate the prevalence of malnutrition in patients with systemic sclerosis (SSc) and its impact on their quality of life (QoL). SUBJECTS/METHODS: One hundred and twenty-nine patients with SSc underwent clinical evaluation and were screened with the Malnutrition Universal Screening Tool (MUST). Malnutrition was defined as MUST score ≥2.To investigate QoL, all patients completed the Short Form 36 Questionnaire and the Scleroderma Health Assessment Questionnaire...
March 8, 2018: European Journal of Clinical Nutrition
Erin L Merz, Linda Kwakkenbos, Marie-Eve Carrier, Shadi Gholizadeh, Sarah D Mills, Rina S Fox, Lisa R Jewett, Heidi Williamson, Diana Harcourt, Shervin Assassi, Daniel E Furst, Karen Gottesman, Maureen D Mayes, Tim P Moss, Brett D Thombs, Vanessa L Malcarne
OBJECTIVE: Valid measures of appearance concern are needed in systemic sclerosis (SSc), a rare, disfiguring autoimmune disease. The Derriford Appearance Scale-24 (DAS-24) assesses appearance-related distress related to visible differences. There is uncertainty regarding its factor structure, possibly due to its scoring method. DESIGN: Cross-sectional survey. SETTING: Participants with SSc were recruited from 27 centres in Canada, the USA and the UK...
March 6, 2018: BMJ Open
Alexander Kreuter, Georg Mitrakos, Silke C Hofmann, Percy Lehmann, Michael Sticherling, Thomas Krieg, Nina Lahner, Christian Tigges, Nicolas Hunzelmann, Pia Moinzadeh
No abstract text is available yet for this article.
March 2, 2018: Acta Dermato-venereologica
Konstantinos Denaxas, Spyros D Ladas, George P Karamanolis
Systemic sclerosis (SSc) is a multisystemic autoimmune connective tissue disorder; in the gastrointestinal tract, the esophagus is the most commonly affected organ. Symptoms of esophageal disease are due to gastroesophageal reflux disease (GERD) and esophageal motor dysfunction. Since the development of high-resolution manometry (HRM), this method has been preferred for the study of SSc patients with esophageal involvement. Using HRM, classic scleroderma esophagus, defined as absent or ineffective peristalsis of the distal esophagus in combination with a hypotensive lower esophageal sphincter, was found in as many as 55% of SSc patients...
March 2018: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
Danielle B Rice, Mara Cañedo-Ayala, Kimberly A Turner, Stephanie T Gumuchian, Vanessa L Malcarne, Mariët Hagedoorn, Brett D Thombs
OBJECTIVES: The nominal group technique (NGT) allows stakeholders to directly generate items for needs assessment surveys. The objective was to demonstrate the use of NGT discussions to develop survey items on (1) challenges experienced by informal caregivers of people living with systemic sclerosis (SSc) and (2) preferences for support services. DESIGN: Three NGT groups were conducted. In each group, participants generated lists of challenges and preferred formats for support services...
March 2, 2018: BMJ Open
Rosemarie A Curley, Alycia Markowski
A 50-year-old woman with an 8-year history of diffuse systemic sclerosis, a form of scleroderma, was referred by her rheumatologist to physical therapy for decreased finger range of motion (ROM) and pain that adversely affected her dexterity and ability to perform activities of daily living. To determine whether joint mobilization would be appropriate for this patient, posterior-to-anterior and modified lateral radiographs of both hands were requested by the physical therapist. Images showed significant bone loss in the distal phalanges of both thumbs and in the left third and fourth digits, and calcinosis in the tips of both thumbs...
March 2018: Journal of Orthopaedic and Sports Physical Therapy
Anas Alzahrani, Yoon Chi, Kenneth W Finnson, Meryem Blati, Bertrand Lussier, Mohit Kapoor, Stephane Roy, Anie Philip
Transforming growth factor (TGF)-β is a multifunctional growth factor with potent pro-fibrotic effects. Endoglin is a TGF-β co-receptor that strongly regulates TGF-β signaling in a variety of cell types. Although aberrant regulation of TGF-β signaling is known to play a key role in fibrotic diseases such as scleroderma and impaired cartilage repair, the significance of endoglin function in regulating these processes is poorly understood. Here we examined whether endoglin haploinsufficiency regulates extracellular (ECM) protein expression and fibrotic responses during bleomycin induced skin fibrosis and surgically induced osteoarthritis, using endoglin-heterozygous (Eng+/-) mice and wild-type (Eng+/+) littermates...
March 2018: Journal of Cell Communication and Signaling
Anita Slomski
No abstract text is available yet for this article.
February 27, 2018: JAMA: the Journal of the American Medical Association
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
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
David Saceda-Corralo, Antonella Tosti
Linear scleroderma en coup de sabre (LSCS) is the most common form of morphea on the scalp and its trichoscopic features have not been described yet. We report 2 adult women with LSCS with distinct dermoscopic findings. They both had an atrophic well-defined linear patch of alopecia on the frontotemporal scalp. Dermoscopy showed loss of follicular openings on a whitish skin surface; scattered black dots, broken hairs, and pili torti; and short thick linear and branching tortuous vessels on the periphery of the lesion...
January 2018: Skin Appendage Disorders
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