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Is there still a role for cyclophosphamide in the treatment of systemic sclerosis?
Madelon C Vonk
doi : 10.1177/2397198320961673
Volume: 6 issue: 2, page(s): 117-122
Cyclophosphamide has been the cornerstone of treatment of systemic sclerosis for a long time and is the first-choice therapy for treating systemic sclerosis–associated interstitial lung disease according to the European League Against Rheumatism recommendations on treatment of systemic sclerosis. However, new therapeutic options are emerging, and treatment with cyclophosphamide is hampered by its toxicity and restricted possible treatment duration. This review has a focus on the evidence of efficacy of cyclophosphamide in different aspects of systemic sclerosis and its organ involvements, reviews its toxicity, and will answer the question whether there is still a role for cyclophosphamide in the treatment of systemic sclerosis, taking the evidence and current therapeutic options into account.
The role of ultrasound in systemic sclerosis: On the cutting edge to foster clinical and research advancement
Michael Hughes1, Cosimo Bruni2, Giovanna Cuomo3, Andrea Delle Sedie4, Luna Gargani5, Marwin Gutierrez6, 7, Gemma Lepri2, Barbara Ruaro8, Tania Santiago9, 10, Yossra Suliman11, Shinji Watanabe12, Annamaria Iagnocco13, Daniel Furst2, 14, 15, Silvia Bellando-Randone2, 16
doi : 10.1177/2397198320970394
Volume: 6 issue: 2, page(s): 123-132
Ultrasound has been widely explored in systemic sclerosis in the clinical and research settings. Ultrasound allows a non-invasive and ionising radiation-free ‘window’ into this complex disease and is well-suited to repeated examinations. Ultrasound provides novel insights into the pathogenesis and measurement of disease in systemic sclerosis, including early (preclinical) internal organ involvement. The purpose of this review is to describe the role of ultrasound to foster clinical and research advancements in systemic sclerosis relating to (1) musculoskeletal, (2) digital ulcer, (3) lung disease and (4) skin disease. We also highlight unmet needs which much be addressed for ultrasound to assume a central role in systemic sclerosis clinical care and research.
Impact of Covid-19 on clinical care and lived experience of systemic sclerosis: An international survey from EURORDIS-Rare Diseases Europe
Michael Hughes1, John D Pauling2, 3, Andrew Moore4, Jennifer Jones5
doi : 10.1177/2397198321999927
Volume: 6 issue: 2, page(s): 133-138
Outcomes related to Covid-19 in systemic sclerosis patients could be influenced by internal organ involvement and/or immunosuppressive treatment, leading to efforts to shield patients from Covid-19 transmission. We examined the impact of Covid-19 on the lived experience of systemic sclerosis with regards to other aspects of daily living including occupation and emotional well-being.
Geographic disparity in systemic sclerosis mortality in the United States: 1999–2017
Alicia Rodriguez-Pla1, 2, Robert W Simms3
doi : 10.1177/2397198319869566
Volume: 6 issue: 2, page(s): 139-145
Previous studies reported a progressive decrease in the systemic sclerosis mortality rates in the United States from 1959 to 2002. Identification of areas with clusters of higher mortality rates is important to implement targeted interventions. In this study, we aimed to estimate the mortality rates of scleroderma and to analyze its geographic variability at the state level in the United States.
Clinical trial protocol: PRednisolone in early diffuse cutaneous Systemic Sclerosis (PRedSS)
Ariane L Herrick1, Deborah J Griffiths-Jones1, W David Ryder2, Justin C Mason3, Christopher P Denton4
doi : 10.1177/2397198320957552
Volume: 6 issue: 2, page(s): 146-153
Many of the painful, disabling features of early diffuse cutaneous systemic sclerosis have an inflammatory component and are potentially treatable with corticosteroid therapy. These features include painful and itchy skin, fatigue and musculoskeletal involvement. Yet many clinicians are understandably reluctant to prescribe corticosteroids because of the concern that these are a risk factor for scleroderma renal crisis. The aim of PRedSS (PRednisolone in early diffuse cutaneous Systemic Sclerosis) is to evaluate the efficacy and safety of moderate dose prednisolone in patients with early diffuse cutaneous systemic sclerosis, specifically whether moderate dose prednisolone is (a) effective in terms of reducing pain and disability, and improving skin score and (b) safe, with particular reference to renal function.
Interstitial lung disease in systemic sclerosis quantification of disease classification and progression with high-resolution computed tomography: An observational study
Johan Clukers1, Maarten Lanclus2, Dennis Belmans3, Cedric Van Holsbeke3, Wilfried De Backer1, Dharshan Vummidi4, Paul Cronin4, Ben R Lavon3, Jan De Backer2, Dinesh Khanna5
doi : 10.1177/2397198320985377
Volume: 6 issue: 2, page(s): 154-164
Systemic sclerosis–associated interstitial lung disease accounts for up to 20% of mortality in these patients and has a highly variable prognosis. Functional respiratory imaging, a quantitative computed tomography imaging technique which allows mapping of regional information, can provide a detailed view of lung structures. It thereby shows potential to better characterize this disease.
Patient preferences for systemic sclerosis treatment: A descriptive study within an Italian cohort
Khadija El Aoufy1, Cosimo Bruni1, Laura Rasero2, Marco Matucci Cerinic1, Daniel Eric Furst1, 3, 4
doi : 10.1177/2397198320971984
Volume: 6 issue: 2, page(s): 165-169
The aim of this study is to ascertain systemic sclerosis patients’ preferences regarding the formulations of the medications they use.
Development of the optimal touchscreen interface for patients with scleroderma
Gianluca Moroncini1, 2, Agnese Brunzini3*, Alessandra Papetti3*, Emanuele Filippini4*, Valentina Maurizi4*, Giulia Cesaretti1*, Rosaria Gesuita5, Armando Gabrielli1, 2, Michele Germani3
doi : 10.1177/2397198320965382
Volume: 6 issue: 2, page(s): 170-177
Impaired hand function is a major contributor to overall disability and reduced health-related quality of life in scleroderma patients. A relevant issue concerns interaction of scleroderma subjects with touchscreen interfaces. This study aims at investigating this problem assessing scleroderma patients’ performance with a novel, aptly designed, touchscreen application in order to identify critical items of touchscreen technology which may impair or facilitate the use by scleroderma subjects. Eighty scleroderma patients performed this novel application including three games, each of which tested a different gesture: tapping, dragging/dropping, and pinching-to-zoom. Eighty healthy subjects without hand impairments were recruited as controls. Scleroderma patients performed worse than healthy users in each game, and statistically significant negatively impacting items were identified. In the second phase of the study, the 10 worst touchscreen performers within the scleroderma cohort were recruited for a physio-rehabilitation trial based on the daily use at home of a modified version of the software application downloaded into the personal devices of patients. The results of this study allow introduction of guidelines to design accessible touchscreen interfaces for subjects with scleroderma and suggest that touchscreen technology may be included in self-administered physio-rehabilitation programs for scleroderma hand.
High D-dimer plasma concentration in systemic sclerosis patients: Prevalence and association with vascular complications
Sofia Furtado1, 2, Bertrand Dunogué1, Georges Jourdi3, 4, Benjamin Chaigne1, Aziza Chibah1, Paul Legendre1, Luc Mouthon1
doi : 10.1177/2397198320957558
Volume: 6 issue: 2, page(s): 178-186
To determine the frequency of elevated D-dimer plasma concentration (>500 ng/mL) in patients with systemic sclerosis and evaluate its association with systemic sclerosis–specific microvascular and macrovascular complications.
Fatigue predicts future reduced social participation, not reduced physical function or quality of life in people with systemic sclerosis
Susan L Murphy1, 2, Daniel Whibley3, 4, Anna L Kratz1, Janet L Poole5, Dinesh Khanna6
doi : 10.1177/2397198320965383
Volume: 6 issue: 2, page(s): 187-193
Although fatigue is one of the most problematic symptoms for people with systemic sclerosis, little is known about how fatigue impacts daily life over time. Such information is important when developing fatigue management interventions. This study was conducted to examine (1) if fatigue severity predicted outcomes of worse functioning (social participation, physical function), and quality of life and (2) if level of self-efficacy moderated significant relationships between fatigue and these outcomes.
A case of recalcitrant linear morphea responding to subcutaneous abatacept
Jordan Talia1, Carole Bitar2, Yue Wang3, Michael L Whitfield3, Dinesh Khanna4
doi : 10.1177/2397198320983240
Volume: 6 issue: 2, page(s): 194-198
Linear morphea is an inflammatory condition that is often treated with systemic glucocorticoids and methotrexate, with mycophenolate mofetil being used as an alternative agent. However, there are few published reports on beneficial effect of abatacept for refractory disease. We present a case of a woman in her 30s who presented with linear morphea on her scalp, with a notable response following the addition of subcutaneous abatacept.
Re-evaluating inclusion criteria for autologous hematopoietic stem cell transplantation in advanced systemic sclerosis: Three successful cases and review of the literature
Ankoor Shah1, J Spierings2, JM van Laar2, Keith M Sullivan3
doi : 10.1177/2397198320985766
Volume: 6 issue: 2, page(s): 199-205
Systemic sclerosis is a chronic autoimmune disease with a poor prognosis, particularly when a patient has rapidly progressive skin or pulmonary involvement. Autologous hematopoietic stem cell transplant is an emerging treatment for this condition, that has been demonstrated to be more effective than immunosuppressants. Careful selection of patients has reduced the transplant-related mortality and maximized the likelihood of benefit. In this report, we present three cases of successful autologous hematopoietic stem cell transplant in patients who would not have met inclusion criteria for entrance into the completed hematopoietic stem cell transplant. After >18 months of follow-up, three patients had clinically significant benefit in terms of skin tightening and pulmonary function tests. Future studies of hematopoietic stem cell transplant in systemic sclerosis may aim to carefully liberalize inclusion criteria to include patients who may not have otherwise been treated while still maintaining an acceptable safety profile.
Parry Romberg disease with En Coup de Sabre Scleroderma: Effect of tocilizumab on periodontal bone inflammation
ThaÃs Cugler Meneghetti1, Juliana Yassue Barbosa da Silva2, Lúcio Eduardo Kluppel3, Vânia Oliveira de Carvalho4
doi : 10.1177/2397198320975872
Volume: 6 issue: 2, page(s): 206-210
Parry Romberg disease and En Coupe de Sabre Scleroderma are frequently associated disorders that affect the face and can cause severe aesthetic and functional impairment. Systemic immunosuppression is the gold standard of first-line treatment in the pediatric rheumatology standpoint although it is often delayed in the pediatric dermatology clinics and more often used in cases of refractory neurological impairment. We report on a case with dental root resorption and severe periodontal bone inflammation detected on magnetic resonance imaging, which was successfully treated with the anti-IL-6 agent tocilizumab.
Refractory eosinophilic fasciitis successfully treated with infliximab: A case report
Paulina Åšmigielska1, Justyna Czarny1, Jacek Kowalski2, Aleksandra Wilkowska1, Roman J. Nowicki1
doi : 10.1177/23971983211004399
Volume: 6 issue: 2, page(s): 211-213
Eosinophilic fasciitis is a rare connective tissue disease of unknown etiology. Therapeutic options include high-dose corticosteroids and other immunosuppressive drugs. We present a typical eosinophilic fasciitis case, which did not respond to first-line treatment, but improved remarkably after infliximab administration. This report demonstrates that in case of initial treatment failure, infliximab might be a relatively safe and effective way of eosinophilic fasciitis management.
Primary central nervous system lymphoma in scleroderma: A case series
Danielle M Robinett1, Laura K Hummers1, Meaghan Morris2, Amy S Duffield2, Ami A Shah1, 3
doi : 10.1177/2397198320970395
Volume: 6 issue: 2, page(s): 214-219
Many studies have demonstrated an increased risk of cancer in patients with rheumatologic diseases, including systemic sclerosis. Less explored is the role of immunosuppressive therapy as a contributing factor in cancer emergence or detection. This series introduces two cases of patients with systemic sclerosis who demonstrated clinical improvement in their rheumatic disease process with immunosuppression, but both of whom developed neurologic symptoms in the setting of decreasing or discontinuing immunosuppressive therapy, leading to the ultimate diagnosis of Epstein–Barr virus positive diffuse large B-cell lymphoma of the central nervous system. To our knowledge, primary central nervous system lymphoma has not been previously described in systemic sclerosis patients. Immunosuppressive therapies could promote the development of virus-associated malignancies due to decreased viral clearance. We hypothesize that removing immunosuppression could allow the immune system to generate an inflammatory response to an underlying tumor or viral antigen, contributing to development of neurologic symptoms and detection of underlying disease.
Unusual type of scleromyxedema with multiple subcutaneous nodules, IgM-? paraproteinemia, and scleroderma-like microangiopathy
Kentaro Awaji, Yoshihide Asano, Mariko Takaoka, Tomonori Oka, Tomomi Miyake, Hiraku Suga, Shinichi Sato
doi : 10.1177/2397198320974190
Volume: 6 issue: 2, page(s): 220-224
Scleromyxedema is a mysterious cutaneous mucinosis of unknown etiology. Various types of scleromyxedema variant have been reported, which often give us a clue to understand the key aspects of this disease. Here, we describe a woman with highly unusual type of scleromyxedema. In addition to the rare manifestations of multiple subcutaneous nodules and IgM-λ paraproteinemia, our patient showed several characteristic symptoms of scleroderma such as shortened nails and fingertips, sclerodactyly, and bone resorption of fingertips and mandibles as a result of peripheral circulatory insufficiency, although this disease is known to be pathophysiologically different from scleroderma. A skin biopsy revealed cutaneous microvascular stenosis and occlusion due to intravascular mucin deposition and fibrotic changes, suggesting that scleromyxedema potentially develops peripheral circulatory disorders and other vascular involvement. The subcutaneous nodules were responsive to high-dose intravenous immunoglobulin. Scleromyxedema can represent a wide variety of systemic involvement, and therefore, we should pay attention to those symptoms as well as skin lesions.
