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Sclerodema

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https://www.readbyqxmd.com/read/27469583/mycophenolate-mofetil-versus-oral-cyclophosphamide-in-scleroderma-related-interstitial-lung-disease-sls-ii-a-randomised-controlled-double-blind-parallel-group-trial
#1
Donald P Tashkin, Michael D Roth, Philip J Clements, Daniel E Furst, Dinesh Khanna, Eric C Kleerup, Jonathan Goldin, Edgar Arriola, Elizabeth R Volkmann, Suzanne Kafaja, Richard Silver, Virginia Steen, Charlie Strange, Robert Wise, Fredrick Wigley, Maureen Mayes, David J Riley, Sabiha Hussain, Shervin Assassi, Vivien M Hsu, Bela Patel, Kristine Phillips, Fernando Martinez, Jeffrey Golden, M Kari Connolly, John Varga, Jane Dematte, Monique E Hinchcliff, Aryeh Fischer, Jeffrey Swigris, Richard Meehan, Arthur Theodore, Robert Simms, Suncica Volkov, Dean E Schraufnagel, Mary Beth Scholand, Tracy Frech, Jerry A Molitor, Kristin Highland, Charles A Read, Marvin J Fritzler, Grace Hyun J Kim, Chi-Hong Tseng, Robert M Elashoff
BACKGROUND: 12 months of oral cyclophosphamide has been shown to alter the progression of scleroderma-related interstitial lung disease when compared with placebo. However, toxicity was a concern and without continued treatment the efficacy disappeared by 24 months. We hypothesised that a 2 year course of mycophenolate mofetil would be safer, better tolerated, and produce longer lasting improvements than cyclophosphamide. METHODS: This randomised, double-blind, parallel group trial enrolled patients from 14 US medical centres with scleroderma-related interstitial lung disease meeting defined dyspnoea, pulmonary function, and high-resolution CT (HRCT) criteria...
September 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/25889583/proteasome-inhibitors-as-experimental-therapeutics-of-autoimmune-diseases
#2
REVIEW
Sue Ellen Verbrugge, Rik J Scheper, Willem F Lems, Tanja D de Gruijl, Gerrit Jansen
Current treatment strategies for rheumatoid arthritis (RA) consisting of disease-modifying anti-rheumatic drugs or biological agents are not always effective, hence driving the demand for new experimental therapeutics. The antiproliferative capacity of proteasome inhibitors (PIs) has received considerable attention given the success of their first prototypical representative, bortezomib (BTZ), in the treatment of B cell and plasma cell-related hematological malignancies. Therapeutic application of PIs in an autoimmune disease setting is much less explored, despite a clear rationale of (immuno) proteasome involvement in (auto)antigen presentation, and PIs harboring the capacity to inhibit the activation of nuclear factor-κB and suppress the release of pro-inflammatory cytokines such as tumor necrosis factor alpha and interleukin-6...
January 28, 2015: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/25776617/proteasome-inhibitors-as-experimental-therapeutics-of-autoimmune-diseases
#3
Sue Ellen Verbrugge, Rik J Scheper, Willem F Lems, Tanja D de Gruijl, Gerrit Jansen
Current treatment strategies for rheumatoid arthritis (RA) consisting of disease-modifying anti-rheumatic drugs or biological agents are not always effective, hence driving the demand for new experimental therapeutics. The antiproliferative capacity of proteasome inhibitors (PIs) has received considerable attention given the success of their first prototypical representative, bortezomib (BTZ), in the treatment of B cell and plasma cell-related hematological malignancies. Therapeutic application of PIs in an autoimmune disease setting is much less explored, despite a clear rationale of (immuno) proteasome involvement in (auto)antigen presentation, and PIs harboring the capacity to inhibit the activation of nuclear factor-κB and suppress the release of pro-inflammatory cytokines such as tumor necrosis factor alpha and interleukin-6...
December 2015: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/25493583/-squamous-cell-carcinoma-in-localized-scleroderma
#4
V Durčanská, H Jedličková, O Sláma, L Velecký, E Březinová, V Vašků
We present a case of a young 26-year-old woman, who has been suffering from localised scleroderma (morphea) for 15 years. Recently, a lesion on the dorsum of her right foot ulcerated. Based on a CT scan and X-ray a diagnosis of ulcerative osteomyellitis was established. The patient was treated with a combination of antibiotics. Subsequent histological examinations showed granulomatous tissue and chronic inflammatory changes on top of pseudoepiteliomatous hyperplasia. The patients status was deteriorating, which resulted in a limb amputation under the knee...
2014: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/23699595/abnormal-pulmonary-function-test-pft-patterns-in-patients-with-chronic-diffuse-fibrotic-lung-diseases
#5
Ameer Rasheed, Viswanath Vasudevan, Farhad Arjomand
SESSION TYPE: ILD PostersPRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PMPURPOSE: Pulmonary function tests help define severity of restrictive ventilator defect in diffuse fibrotic lung diseases like Idiopathic pulmonary fibrosis (IPF) and scleroderma. VC, TLC and DLCO are used interchangeably to define disease severity. VC is easily measured with office based spirometry, whereas measuring TLC and DLCO requires pulmonary physiology Lab. We sought to define the pattern of PFT abnormality and prevalence of reduced VC, TLC and diffusion capacity in patients with idiopathic pulmonary fibrosis (IPF) and Sclerodema...
October 1, 2012: Chest
https://www.readbyqxmd.com/read/21794601/-clinical-characteristics-of-children-with-scleroderma-in-a-referral-hospital
#6
Zoilo Morel, Eduardo Benadón, Enrique Faugier, María Del Rocío Maldonado
INTRODUCTION: Scleroderma is an autoimmune disease that involves the connective tissue characterized by skin fibrosis, classified as localized and systemic (participation of one or more internal organs). The primary objective of this study is to describe and analyze the clinical and laboratory findings in a group of children diagnosed with scleroderma at a referral hospital. MATERIAL AND METHODS: Extraction of data from clinical charts of children with scleroderma in the rheumatology department at the Hospital Infantil de México Federico Gómez, between January 2000 and December 2007...
July 2009: Reumatología Clinica
https://www.readbyqxmd.com/read/19208687/male-microchimerism-and-hla-compatibility-in-french-women-with-sclerodema-a-different-profile-in-limited-and-diffuse-subset
#7
COMPARATIVE STUDY
Justyna M Rak, Philippe P Pagni, Kiet Tiev, Yannick Allanore, Dominique Farge, Jean-Robert Harlé, David Launay, Eric Hachulla, Rémi Didelot, Jean Cabane, André Kahan, Marielle Martin, Brigitte Granel, Jean Roudier, Nathalie C Lambert
OBJECTIVES: Male microchimerism (Mc) persisting from pregnancy has been found at greater frequencies and/or higher quantities in women with scleroderma (SSc) compared with controls, suggesting a possible role in disease development. Moreover, women with an HLA-compatible child have a higher risk to develop SSc. We tested the hypothesis, on our French SSc cohort, that women with lcSSc and dcSSc, two distinct clinical subsets, have a different profile in terms of Mc and HLA compatibility in families...
April 2009: Rheumatology
https://www.readbyqxmd.com/read/16643177/laparoscopic-fundoplication-in-patients-with-an-aperistaltic-esophagus-and-gastroesophageal-reflux
#8
D I Watson, G G Jamieson, J R Bessell, P G Devitt
A minority of patients with severe gastroesophageal reflux who present to surgeons for antireflux surgery have absent esophageal peristalsis when investigated before surgery with esophageal manometry. Some of these patients also have systemic sclerodema. While conventional wisdom suggests that these patients are at risk of a poor outcome if they proceed to fundoplication, some will have severe reflux symptoms, which are poorly controlled by medical therapy, and surgery will therefore offer the only chance of 'cure'...
2006: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
https://www.readbyqxmd.com/read/15575905/kidney-transplantation-for-systemic-sclerosis-improves-survival-and-may-modulate-disease-activity
#9
Eric M Gibney, Chirag R Parikh, Alkesh Jani, Michael J Fischer, David Collier, Alexander C Wiseman
Systemic sclerosis (SS) may lead to sclerodema renal crisis, an unusual cause of end-stage renal disease (ESRD) with historically poor hemodialysis outcomes. Little information is available on outcomes after kidney transplantation. Information from the UNOS registry was obtained on SS patients in the United States, listed for kidney transplants between 1985-2002. We compared survival at 1 and 3 years in patients who received cadaveric transplants with patients who remained on the waiting list. Graft survival, cause of graft loss, frequency of early graft loss and pre- and post-transplant skin scores were analyzed...
December 2004: American Journal of Transplantation
https://www.readbyqxmd.com/read/11988681/-obesity-persistent-scleredema-study-of-49-cases
#10
V Ray, A M Boisseau-Garsaud, P Ray, F Pont, L Lin, R Hélénon, D Calès-Quist
BACKGROUND: Buschke sclerodema is a very rare disease. Our objective was to show that persistent scleredema is frequent in certain group of patients at risk. PATIENTS AND METHODS: We studied 49 patients, diagnosed between 1995 and 1999 in dermatology, pneumology and endocrinology departments in Martinique. Diagnosis was performed on classical clinical and histopathological aspects of sclerodema. Data studied were age, sex, mode of occurrence, clinical and histopathological aspects and associated diseases...
March 2002: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/11039266/-progressive-systemic-sclerosis-associated-with-anti-myeloperoxidase-anca-vasculitis-with-renal-and-cutaneous-involvement
#11
J Martínez Ara, M L Picazo, A Torre, D Pascual, C Díaz Rodríguez, C Riñón
Sclerodema renal crisis is the usual form of presentation of renal disease in systemic sclerosis. We report a woman who at age 63 was given a diagnosis of scleroderma with Raynaud's phenomenon and cutaneous, oesophageal and lung involvement but no evidence of renal disease and no treatment with D-penicillamine. Two years later she developed progressive renal failure, nephrotic range proteinuria, haematuria and the presence of serum MPO-ANCA; she was normotensive. Renal biopsy revealed extracapillary and necrotizing glomerulonephritis and skin biopsy showed leucocytoclastic vasculitis...
July 2000: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
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