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dermatomyositis and rituximab

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https://www.readbyqxmd.com/read/29713969/rituximab-for-refractory-rapidly-progressive-interstitial-lung-disease-related-to-anti-mda5-antibody-positive-amyopathic-dermatomyositis
#1
Ho So, Victor Tak Lung Wong, Virginia Weng Nga Lao, Hin Ting Pang, Ronald Man Lung Yip
To report our experience in using rituximab (RTX) for treating refractory rapidly progressive interstitial lung disease (RP-ILD) complicating anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab)-positive amyopathic dermatomyositis (ADM). Medical records of four ADM patients with refractory RP-ILD treated with RTX therapy were reviewed retrospectively. All four patients were tested positive for anti-MDA5 Ab and failed to respond to high-dose systemic steroid and other intensive immunosuppressive therapies...
April 30, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29669460/current-diagnosis-and-treatment-of-polymyositis-and-dermatomyositis
#2
Hirokazu Sasaki, Hitoshi Kohsaka
Idiopathic inflammatory myopathies (IIMs) are heterogeneous disorders that affect the skeletal muscles. Polymyositis, dermatomyositis, and inclusion body myositis are major IIM subsets. Immune-mediated necrotizing myopathy became recognized as a potentially new IIM subset. Since the new classification criteria published by the International Myositis Classification Criteria Project have higher sensitivity and specificity for IIM classification and subclassification than the previous criteria, they should help precise diagnosis...
April 18, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29665800/mda-5-associated-rapidly-progressive-interstitial-lung-disease-with-recurrent-pneumothoraces-a-case-report
#3
Safi Alqatari, Peter Riddell, Sinead Harney, Michael Henry, Grainne Murphy
BACKGROUND: Clinically hypomyopathic dermatomyositis is a rare disease that is important to recognize, investigate and treat early as it is associated with poor prognosis. In a proportion of patients, myositis specific antibodies could be negative, but with high clinical suspicion, myositis associated antibodies should be ordered. Anti-MDA-5 antibodies was reported in literature to be associated with severe and rapidly progressive interstitial lung disease, with few case reports of pneumothorax and/or pneumomediastinum...
April 17, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29308288/microscopic-polyangiitis-with-dermatomyositis
#4
Erin M Bauer, Ernest Brahn
Dermatomyositis is a rare autoimmune disease with a heterogeneous presentation that often has multiple extramuscular manifestations, although it does not typically involve the renal function. A 62-year-old female presented with proximal muscle weakness and rashes, which are classic symptoms of dermatomyositis without creatine kinase (CK) elevation. Initial serologic evaluation revealed a positive p-ANCA, although she did not develop renal failure for several months, at which point renal biopsy findings were consistent with microscopic polyangiitis...
December 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/29225988/effective-administration-of-rituximab-in-anti-mda5-antibody-positive-dermatomyositis-with-rapidly-progressive-interstitial-lung-disease-and-refractory-cutaneous-involvement-a-case-report-and-literature-review
#5
Yuka Ogawa, Dai Kishida, Yasuhiro Shimojima, Koichi Hayashi, Yoshiki Sekijima
We describe the case of a 48-year-old man with dermatomyositis (DM) who demonstrated rapidly progressive interstitial lung disease (RP-ILD) and refractory cutaneous involvement together with high levels of anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab). Even after combination immunosuppressive therapy including a corticosteroid, cyclosporine A, and intravenous cyclophosphamide, his respiratory insufficiency and cutaneous involvement progressively worsened. However, the administration of rituximab (RTX) resulted in clinical remission as well as a visible reduction in anti-MDA5-Ab levels, suggesting that RTX could be a useful remedy in cases refractory to conventional immunosuppressive agents, especially those of RP-ILD related to anti-MDA5-Ab-positive DM...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29204355/cardiac-transplantation-in-dermatomyositis-a-case-report-and-literature-review
#6
Sangmee Bae, Negar Khanlou, Christina Charles-Schoeman
Background and objectives: Cardiac involvement has been well recognized in patients with dermatomyositis (DM) and polymyositis (PM) with a variable frequency between 9 and 72%. However, clinically significant heart involvement in DM/PM is relatively infrequent and there have been rare reports of cardiac transplantation in DM. Our aims were to describe a case of severe cardiac involvement in DM requiring heart transplantation and review the literature of cardiac disease in DM and PM. Methods: A patient with dermatomyositis who was referred to our institution with severe heart failure is described...
June 2017: Human Pathology (New York)
https://www.readbyqxmd.com/read/29160756/recent-developments-on-treatment-strategies-and-the-prognosis-of-dermatomyositis-a-review
#7
Verena Isak, Joseph L Jorizzo
Dermatomyositis is an autoimmune disease affecting both skin and muscle. Steroids are the first line treatment. However, no consensus regarding dosing, length of treatment, tapering speed, or when to add which immunosuppressant in case of steroid resistance, has been reached, as randomized-controlled trials are scarce. Especially data on long-term treatment is lacking in order to decide on the most effective treatment for long-term remission. Empiric evidence suggests that with an initially aggressive treatment with oral steroids followed by a slow taper, during which disease activity is adequately controlled, patients can be off therapy within typically 24-48 months, and remain disease-free without medication for over 20 years...
November 22, 2017: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/29090371/cutaneous-manifestations-of-dermatomyositis-a-comprehensive-review
#8
REVIEW
Carlo Mainetti, Benedetta Terziroli Beretta-Piccoli, Carlo Selmi
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by the presence of skin lesions and inflammation of skeletal muscles; however, this feature may be absent in amyopathic DM. DM is a rare disease, occurring at any age, and has two peaks of incidence: one in childhood between 5 and 15 years of age and one in adulthood between 40 and 60 years, with a female preponderance. DM has been associated with malignancy; therefore, every newly diagnosed patient should undergo screening investigations, but evidence-based guidelines on their extension are lacking...
December 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29089059/effective-induction-therapy-for-anti-srp-associated-myositis-in-childhood-a-small-case-series-and-review-of-the-literature
#9
E L Binns, E Moraitis, S Maillard, S Tansley, N McHugh, T S Jacques, L R Wedderburn, C Pilkington, S A Yasin, K Nistala
BACKGROUND: Anti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to respond to conventional first line therapies. We present three cases where remission was successfully induced using combination therapy with intensive rehabilitation. CASE PRESENTATIONS: Three new patients are reported. All 3 cases presented with profound, rapid-onset, proximal myopathy and markedly raised CK, but no rash...
October 31, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28763333/recent-clinical-trials-in-idiopathic-inflammatory-myopathies
#10
Valérie Leclair, Ingrid E Lundberg
PURPOSE OF REVIEW: Idiopathic inflammatory myopathies (IIMs) are complex multisystemic autoimmune diseases. Glucocorticoids remain the cornerstone of treatment in IIM, and the benefit of additional immunosuppressors is still debated. A limited number of controlled clinical trials have been available to support treatment guidelines, but in the last year, several clinical trials have been published. In this review, the highlights of recently published and on-going clinical trials in IIM will be summarized and discussed...
November 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28685324/phenotypic-characteristics-and-outcome-of-juvenile-dermatomyositis-in-arab-children
#11
Sulaiman M Al-Mayouf, Nora AlMutiari, Mohammed Muzaffer, Rawiah Shehata, Adel Al-Wahadneh, Reem Abdwani, Safia Al-Abrawi, Mohammed Abu-Shukair, Zeyad El-Habahbeh, Abdullah Alsonbul
This study describes the disease characteristics and outcome of Arab children with juvenile dermatomyositis (JDM) and compares the findings with other ethnicities. We retrospectively reviewed the hospital registries of the participating hospitals for children with JDM seen between 1990 and 2016 in three Arab countries. All patients fulfilled Bohan and Peter criteria for JDM, diagnosed before 14 years of age and were of Arab ethnicity. Clinical and laboratory features as well as the long-term outcomes including accrual disease damage were collected at the last follow-up visit...
September 2017: Rheumatology International
https://www.readbyqxmd.com/read/28668260/-amyopathic-dermatomyositis-dm-with-anti-mda5-antibodies-associated-with-bullous-pemphigoid-sj%C3%A3-gren-syndrome-and-gastric-malt-lymphoma
#12
B Garcia, F Dabouz, L Pascal, M Gillard, P Modiano
BACKGROUND: The inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. hypomyopathic/amyopathic DM. Anti-MDA5 dermatomyositis (DM) is a rare form of dermatomyositis that is frequently amyopathic; the prognosis is linked mainly to pulmonary involvement. PATIENTS AND METHODS: A 69-year-old woman treated for mucosa-associated lymphoid tissue (MALT) gastric lymphoma was referred for a bullous eruption...
October 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28619061/rituximab-versus-cyclophosphamide-for-the-treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-recital-study-protocol-for-a-randomised-controlled-trial
#13
RANDOMIZED CONTROLLED TRIAL
Peter Saunders, Vicky Tsipouri, Gregory J Keir, Deborah Ashby, Marcus D Flather, Helen Parfrey, Daphne Babalis, Elisabetta A Renzoni, Christopher P Denton, Athol U Wells, Toby M Maher
BACKGROUND: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little...
June 15, 2017: Trials
https://www.readbyqxmd.com/read/28610606/biologic-therapies-for-refractory-juvenile-dermatomyositis-five-years-of-experience-of-the-childhood-arthritis-and-rheumatology-research-alliance-in-north-america
#14
C H Spencer, K Rouster-Stevens, H Gewanter, G Syverson, R Modica, K Schmidt, H Emery, C Wallace, S Grevich, K Nanda, Y D Zhao, S Shenoi, S Tarvin, S Hong, C Lindsley, J E Weiss, M Passo, K Ede, A Brown, K Ardalan, W Bernal, M L Stoll, B Lang, R Carrasco, C Agaiar, L Feller, H Bukulmez, R Vehe, H Kim, H Schmeling, D Gerstbacher, M Hoeltzel, B Eberhard, R Sundel, S Kim, A M Huber, A Patwardhan
BACKGROUND: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011-2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA)...
June 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28566605/dermatomyositis-with-rapidly-progressive-interstitial-lung-disease-treated-with-rituximab-a-report-of-3-cases-in-japan
#15
Kenichiro Tokunaga, Noboru Hagino
We performed a retrospective chart review of three patients with hypomyopathic dermatomyositis and rapidly progressive interstitial lung disease. The patients were Japanese women of 71, 69, and 65 years of age. Two patients were anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and 1 was anti-aminoacyl-tRNA synthetase (anti-ARS) antibody-positive. Their respiratory statuses deteriorated despite the administration of glucocorticoid, calcineurin inhibitors, and intravenous cyclophosphamide therapy...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28553129/medical-resource-utilization-in-dermatomyositis-polymyositis-patients-treated-with-repository-corticotropin-injection-intravenous-immunoglobulin-and-or-rituximab
#16
Tyler Knight, T Christopher Bond, Breanna Popelar, Li Wang, John W Niewoehner, Kathryn Anastassopoulos, Michael Philbin
BACKGROUND: Dermatomyositis and polymyositis (DM/PM) are rare, incurable inflammatory diseases that cause progressive muscle weakness and can be associated with increased medical resource use (MRU). When corticosteroid treatment is unsuccessful, patients may receive intravenous immunoglobulin (IVIg), rituximab, or repository corticotropin injection (RCI). This study compared real-world, non-medication MRU between patients treated with RCI and those treated with IVIg and/or rituximab for DM/PM...
2017: ClinicoEconomics and Outcomes Research: CEOR
https://www.readbyqxmd.com/read/28538257/what-s-in-the-literature
#17
Mark Bromberg, Nicholas J Silvestri, David Lacomis
In this edition, we provide a detailed summary of an informative book, "GBS100: Celebrating a Century of Progress in Guillain-Barré Syndrome" developed by the Peripheral Nerve Society to honor the centenary of the original paper on Guillain-Barré Syndrome. We also review various studies in myasthenia gravis including: management with rituximab; the efficacy of early fast-acting treatment with corticosteroids; and various dosing strategies for tacrolimus. Finally, we review new studies including: the potential pathogenesis, risk factors, and functional decline of patients with inclusion body myositis; MxA immunoreactivity in dermatomyositis; diagnostic approaches for evaluating patients with myalgia, fatigue, and exercise intolerance; MRI patterns in genetic muscle disease; and MRI as an outcome measure in facioscapulohumeral muscular dystrophy...
June 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28536935/-rituximab-for-the-treatment-of-poly-and-dermatomyositis-results-from-the-graid-2-registry
#18
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...
February 2018: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28524083/idiopathic-inflammatory-myopathies-a-review-of-the-classification-and-impact-of-pathogenesis
#19
REVIEW
Dana E Mandel, Charles J Malemud, Ali D Askari
Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality. This review details and updates the pathogenesis and emerging importance of myositis-specific antibodies in the development of IIMs. An increase in the understanding of how these myositis-specific antibodies play a role in IIMs has led to the further categorization of IIMs from the traditional polymyositis versus dermatomyositis, to additional subcategories of IIMs such as necrotizing autoimmune myositis (NAM)...
May 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28385804/2016-american-college-of-rheumatology-european-league-against-rheumatism-criteria-for-minimal-moderate-and-major-clinical-response-in-juvenile-dermatomyositis-an-international-myositis-assessment-and-clinical-studies-group-paediatric-rheumatology-international
#20
Lisa G Rider, Rohit Aggarwal, Angela Pistorio, Nastaran Bayat, Brian Erman, Brian M Feldman, Adam M Huber, Rolando Cimaz, Rubén J Cuttica, Sheila Knupp de Oliveira, Carol B Lindsley, Clarissa A Pilkington, Marilynn Punaro, Angelo Ravelli, Ann M Reed, Kelly Rouster-Stevens, Annet van Royen-Kerkhof, Frank Dressler, Claudia Saad Magalhaes, Tamás Constantin, Joyce E Davidson, Bo Magnusson, Ricardo Russo, Luca Villa, Mariangela Rinaldi, Howard Rockette, Peter A Lachenbruch, Frederick W Miller, Jiri Vencovsky, Nicolino Ruperto
To develop response criteria for juvenile dermatomyositis (DM). We analysed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial...
May 2017: Annals of the Rheumatic Diseases
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