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dermatomyositis and rituximab

Fabian Speth, Johannes-Peter Haas, Claas H Hinze
BACKGROUND: High-dose intravenous immune globulins (IVIg) are frequently used in refractory juvenile dermatomyositis (JDM) but are often poorly tolerated. High-dose recombinant human hyaluronidase-facilitated subcutaneous immune globulins (fSCIg) allow the administration of much higher doses of immune globulins than conventional subcutaneous immune globulin therapy and may be an alternative to IVIg. The safety and efficacy of fSCIg therapy in JDM is unknown. CASE PRESENTATION: In this retrospective case series, five patients with steroid-refractory severe JDM were treated with high-dose fSCIg due to IVIg adverse effects (severe headaches, nausea, vomiting, difficult venous access)...
2016: Pediatric Rheumatology Online Journal
Khaled Eissa, Jaime Palomino
Interstitial lung disease (ILD) is a major cause of morbidity and mortality in patients with idiopathic inflammatory myopathies (IIM). Glucocorticoids are the initial standard treatment. However, many patients fail to respond and continue to progress despite treatment with high dose glucocorticoids. The efficacy of rituximab has been suggested in case reports and case series of refractory antisynthetase (AS) syndrome, but data on patients without auto-antibodies or with rapidly progressive ILD are scarce. We report a case of rapidly progressive dermatomyositis (DM) associated ILD treated successfully with B-cell depletion therapy...
May 2016: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
Jutta Bauhammer, Norbert Blank, Regina Max, Hanns-Martin Lorenz, Ulrich Wagner, Dietmar Krause, Christoph Fiehn
OBJECTIVE: Rituximab (RTX) has been used successfully for the treatment of severe Jo1 antibody-associated antisynthetase syndrome. The aim of this retrospective study was to evaluate the effect of RTX in severe Jo1 antisynthetase syndrome and determine predictive factors for response. METHODS: There were 61 patients with Jo1 antisynthetase syndrome identified; 18 of these received RTX. One patient was lost to followup. The remaining 17 patients and 30 out of 43 patients who were treated with conventional immunosuppressive (IS) drugs were followed for a mean of 35 months and 84 months, respectively...
August 2016: Journal of Rheumatology
Maria Dubos, Kim Ly, Clothilde Martel, Anne Laure Fauchais
Calcinosis, the deposition of calcified material in soft tissues, is frequently seen in systemic sclerosis and dermatomyositis. Treatment options are limited, with disappointing results. Some recent case reports suggest that rituximab may be an attractive therapeutic option. In case 1, a 54-year-old woman who presented with rheumatoid arthritis in association with scleromyositis was treated with rituximab for rheumatoid arthritis. Despite this, she developed multiple progressive calcinosis, necessitating extracorporeal shock wave lithotripsy to limit calcinosis extension and pain...
2016: BMJ Case Reports
Shinsuke Yasuda, Michihito Kono, Sanae Shimamura, Takashi Kurita, Toshio Odani, Tatsuya Atsumi
  Treatment of organ involvements accompanied by systemic autoimmune diseases is still challenging for clinicians, reminding the existence of unmet needs. Among them, lupus nephritis (LN), neuropsychiatric lupus, interstitial lung diseases (ILD) complicated with polymyositis/dermatomyositis (PM/DM) or systemic sclerosis (SSc) are the most severe conditions with poor prognosis. Because of the rarity and severity of the disease status, and of variety in evaluation methods, randomized clinical trials tend to be difficult in recruiting patients, in designing protocols, and in meeting primary endpoints...
2016: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
Serena Fasano, Patrick Gordon, Raouf Hajji, Esthela Loyo, David A Isenberg
Several uncontrolled studies have encouraged the use of rituximab (RTX) in patients with myositis. Unfortunately, the first placebo-phase trial to assess the efficacy of RTX in refractory myositis did not show a significant difference between the two treatment groups, and doubts have been expressed about its study design. In this review we present an up-to-date overview of the reported experiences of RTX therapy in myositis. A PubMed search was performed to find all the available cases of refractory myositis patients treated with RTX up to July 2015...
April 27, 2016: Rheumatology
C V Oddis
The management of patients with idiopathic inflammatory myopathy (IIM) remains a challenge given the systemic features beyond active myositis. That is, recognizing the inflammatory arthropathy, varying dermatomyositis rashes, and overt and occult features of interstitial lung disease in addition to myositis adds to the complexity of diagnosis and treatment of IIM. However, clinicians now have available many more immunosuppressive drugs as well as biologic agents for use in patients with myositis and other autoimmune diseases...
July 2016: Journal of Internal Medicine
Cord Sunderkötter, Alexander Nast, Margitta Worm, Reinhard Dengler, Thomas Dörner, Horst Ganter, Reinhard Hohlfeld, Arthur Melms, Nico Melzer, Kai Rösler, Jens Schmidt, Michael Sinnreich, Maggi C Walter, Julia Wanschitz, Heinz Wiendl
Diese Leitlinie für Dermatomyositis (DM) ist ein Auszug aus der interdisziplinären S2k-Leitlinie der deutschen Gesellschaft für Neurologie zu Myositissyndromen. Schlüsselsymptom für die Myositis bei DM ist eine proximal-symmetrische Muskelschwäche. Weitere diagnostische Hinweise liefern Creatinkinase, CRP oder BSG, Elektromyographie und Muskelbiopsie. Schluck-, Atem-, Herz- und Nackenmuskulatur können beteiligt sein. Da ca. 30 % der Patienten eine interstitielle Lungenerkrankung haben, sollte auch ein Lungenfunktionstest erfolgen...
March 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Cord Sunderkötter, Alexander Nast, Margitta Worm, Reinhard Dengler, Thomas Dörner, Horst Ganter, Reinhard Hohlfeld, Arthur Melms, Nico Melzer, Kai Rösler, Jens Schmidt, Michael Sinnreich, Maggi C Walter, Julia Wanschitz, Heinz Wiendl
The present guidelines on dermatomyositis (DM) represent an excerpt from the interdisciplinary S2k guidelines on myositis syndromes of the German Society of Neurology (available at The cardinal symptom of myositis in DM is symmetrical proximal muscle weakness. Elevated creatine kinase, CRP or ESR as well as electromyography and muscle biopsy also provide important diagnostic clues. Pharyngeal, respiratory, cardiac, and neck muscles may also be affected. Given that approximately 30% of patients also develop interstitial lung disease, pulmonary function tests should be part of the diagnostic workup...
March 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Natalie A Wright, Ruth Ann Vleugels, Jeffrey P Callen
Dermatomyositis (DM) is a systemic inflammatory condition characterized by cutaneous and muscle findings, in addition to potential involvement of other organ systems. A distinct subtype of DM exists that is categorized by cutaneous findings with absent or minimal muscle involvement, referred to as clinically amyopathic dermatomyositis or dermatomyositis sine myositis. A variety of topical, immunosuppressive, and immunomodulatory therapies have been utilized to treat cutaneous DM. The advent of biological agents including tumor necrosis factor-α antagonists, intravenous immunoglobulin, rituximab, and others has allowed for the use of these agents with varying degrees of success for the treatment of cutaneous DM...
January 2016: Seminars in Immunopathology
Erin Vermaak, Sarah L Tansley, Neil J McHugh
Dermatomyositis and polymyositis are rare chronic inflammatory disorders with significant associated morbidity and mortality despite treatment. High-dose corticosteroids in addition to other interventions such as immunosuppressants, immunomodulators, and more recently, biologics are commonly used in clinical practice; however, there are no clear guidelines directing their use. Our objective was to systematically review the evidence for immunotherapy in the treatment of dermatomyositis and polymyositis. Relevant studies were identified through Embase and PubMed database searches...
December 2015: Clinical Rheumatology
Hidenaga Kawasumi, Takahisa Gono, Yasushi Kawaguchi, Hisashi Yamanaka
Interstitial lung disease (ILD) is a prognostic factor for poor outcome in polymyositis (PM)/dermatomyositis (DM). The appropriate management of ILD is very important to improve the prognosis of patients with PM/DM. ILD activity and severity depend on the disease subtype. Therefore, clinicians should determine therapeutic strategies according to the disease subtype in each patient with PM/DM. Anti-melanoma differentiation-associated gene 5 antibody and hyperferritinemia predict the development and severity of rapidly progressive (RP) ILD, particularly in East Asian patients...
2015: Clinical Medicine Insights. Circulatory, Respiratory and Pulmonary Medicine
E G Healy, R Phadke, M Kidd, M M Reilly, M P Lunn
We report a rare case of myofasciitis and meningitis with deafness caused by systemic enterovirus infection in the setting of hypogammaglobulinaemia induced by rituximab. Whilst effective and generally safe, anti- CD 20 antibody therapy is increasingly recognised to result in unusual infectious complications to be considered in a treated patient presenting with neurological symptoms. These cases may pose diagnostic difficulties and can have atypical presentations. We present this rare complication of rituximab therapy, with histopathological confirmation of myofasciitis...
July 2015: Neuromuscular Disorders: NMD
Moncef Belhassen-Garcia, Alberto Rábano-Gutiérrez, Virginia Velasco-Tirado, Angela Romero-Alegria, Maria-Luisa Pérez-Garcia, Jose Angel Martin-Oterino
Antisynthetase syndrome is a disorder belonging to the dermatomyositis/polymyositis group, with high rates of morbidity and mortality. We herein present the case of a 71-year-old man who was diagnosed with antisynthetase syndrome and treated with rituximab. Almost three years later, the patient showed right-sided hemiparesis that ultimately progressed to complete hemiplegia and advancing cognitive deterioration with a poor clinical outcome. The neuropathological diagnosis was progressive multifocal leukoencephalopathy...
2015: Internal Medicine
Ryu Watanabe, Tomonori Ishii, Kyoko Araki, Mariko Ishizuka, Yukiko Kamogawa, Yoko Fujita, Yuko Shirota, Hiroshi Fujii, Hideo Harigae
No abstract text is available yet for this article.
2016: Modern Rheumatology
E Laffitte
This article focuses on current trends in various autoimmune diseases of interest for the dermatologist. In the antiphospholipid syndrome, many news: better characterization of the severe disease, involvement of the mTOR pathway in the vasculopathy-induced renal disease, and diversification of the therapeutic approaches: use of mTOR inhibitors and several biologics, new various antiplatelet and anticoagulants. In dermatomyositis, new autoantibodies are better characterized with a good correlation with clinical disease; the results of a large study on genetic predisposition to the disease are available...
December 2014: Annales de Dermatologie et de Vénéréologie
Guixiu Shi, Yuan Liu
No abstract text is available yet for this article.
2014: Current Pharmaceutical Biotechnology
Ingrid E Lundberg, Jiri Vencovsky, Helene Alexanderson
PURPOSE OF REVIEW: To give an update on reported use and effects of biological and physical therapies in patients with myositis. RECENT FINDINGS: The most promising biological treatment in polymyositis, dermatomyositis and juvenile dermatomyositis is B-cell blockade by rituximab. Anti-Jo or anti-Mi-2 antibodies were predictors of response suggesting different molecular pathways in different subsets of myositis. T-cell blockade with abatacept is a new possibility, as is blockade of interleukin-1, interleukin-6 or type I interferon, but controlled studies are needed...
November 2014: Current Opinion in Rheumatology
Lisa G Rider, Adrienne L Yip, Iren Horkayne-Szakaly, Rita Volochayev, Joseph A Shrader, Maria L Turner, Heidi H Kong, Minal S Jain, Anna V Jansen, Chester V Oddis, Thomas A Fleisher, Frederick W Miller
OBJECTIVES: We aimed to assess changes in myositis core set measures and ancillary clinical and laboratory data from the National Institutes of Health's subset of patients enrolled in the Rituximab in Myositis trial. METHODS: Eighteen patients (5 dermatomyositis, 8 polymyositis, 5 juvenile dermatomyositis) completed more in-depth testing of muscle strength and cutaneous assessments, patient-reported outcomes, and laboratory tests before and after administration of rituximab...
September 2014: Clinical and Experimental Rheumatology
Leonore Unger, Susanne Kampf, Kirsten Lüthke, Martin Aringer
OBJECTIVES: While a double-blind trial has not met its endpoint, rituximab (RTX) is still seen as useful in refractory DM and PM. In this study we analysed the charts of all patients receiving RTX for myositis in our institutions for objective outcome parameters. METHODS: In a retrospective way, the charts of all patients with PM or DM who received RTX were analysed for glucocorticoid dose, creatine phosphokinase (CPK) and lung function tests, as well as for serious adverse events...
September 2014: Rheumatology
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