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Juvenile dermatomyositis

Alessandro Consolaro, Esi M Morgan, Gabriella Giancane, Silvia Rosina, Stefano Lanni, Angelo Ravelli
Information technology in paediatric rheumatology has seen several exciting developments in recent years. The new multidimensional questionnaires for juvenile idiopathic arthritis, juvenile dermatomyositis, and juvenile autoinflammatory diseases integrate all major parent- and child-reported outcomes (PCROs) used in these diseases into a single tool, and provide an effective guide to manage, document change in health, assess effectiveness of therapeutic interventions, and verify the parent and child satisfaction with illness outcome...
September 2016: Clinical and Experimental Rheumatology
Thomas Schwartz, Louise Pyndt Diederichsen, Ingrid E Lundberg, Ivar Sjaastad, Helga Sanner
Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention...
2016: RMD Open
Ana Luiza M Amorim, Nadia C Cabral, Fabiane M Osaku, Claudio A Len, Enedina M L Oliveira, Maria Teresa Terreri
INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients...
September 28, 2016: Revista Brasileira de Reumatologia
Hiroyuki Wakiguchi, Syuji Takei, Yoshifumi Kawano
No abstract text is available yet for this article.
September 20, 2016: Pediatrics and Neonatology
Muhammed Razmi T, Keshavamurthy Vinay, Davinder Parsad
No abstract text is available yet for this article.
September 27, 2016: Rheumatology
E D Owen, E H Choy, V Piguet
No abstract text is available yet for this article.
October 2016: British Journal of Dermatology
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
H Rachadi, K Bouayad, S Chiheb
: Juvenile dermatomyositis (JDM) is a rare disease, with a mean age of onset of 7 years. We report a case of JDM in a 13-month-old infant. OBSERVATION: A 13-month-old infant presented with an edema of the upper lip, 4 days after receiving amoxicillin-clavulanate. The patient was treated with betamethasone and an antihistamine. Progression was marked by the appearance of a white edema of the periorbital area and the upper lip, with purpuric lesions, aphthoid ulcerations, and a drooping head...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
S Almeida, M Braga, M J Santos
Serologic investigation has been explored in inflammatory myopathies in order to define subgroups that can help us predict clinical course, treatment and prognosis. The level of similarity between juvenile and adult myopathies regarding the presence of myositis-specific autoantibodies has not been fully elucidated. We report the case of a 8-year-old girl who presented with a rapid progression of muscle weakness and cutaneous signs consistent with the diagnosis of juvenile dermatomyositis and whose serologic testing revealed the presence of anti-signal recognition particle (SRP) antibodies...
April 2016: Acta Reumatológica Portuguesa
Satoshi Sato, Yoji Uejima, Meika Nanbu, Eisuke Suganuma, Tadamasa Takano, Risa Tanaka, Tomoyuki Kabuki, Eiji Oguma, Tsutomu Oh-Ishi, Yutaka Kawano
OBJECTIVES: The aim of this study was to determine the clinical phenotype and outcome of interstitial lung disease (ILD) complicated with juvenile dermatomyositis (JDM) or juvenile polymyositis (JPM). METHODS: This was a single-center retrospective study. From 1984 to 2015, we retrospectively reviewed 29 patients who were diagnosed with JDM/JPM, among whom eight cases were ILD and 21 were non-ILD. The clinical features and laboratory findings included chest computed tomography (CT) images that were compared between the patients with ILD and non-ILD...
September 20, 2016: Modern Rheumatology
Rajendra P Nagar, Joyita Bharati, Abraar Sheriff, Praytusha Priyadarshini, Sunil Chumber, S K Kabra
We report a case of dystrophic calcification presenting as soft cystic swelling in a patient with juvenile dermatomyositis. A 15-year-old boy with lumbosacral cystic swelling, which was considered a cold abscess clinically, was evaluated for nonresponse to antitubercular therapy. The cystic swelling had liquefied calcium with a well circumscribed calcified wall on imaging, which was subsequently excised.
March 2016: National Medical Journal of India
Michael C Kwa, Kaveh Ardalan, Anne E Laumann, Beatrice Nardone, Dennis P West, Jonathan I Silverberg
OBJECTIVE: To assess the validity of using ICD-9-CM code 710.3 to identify adult patients with dermatomyositis in outpatient and inpatient settings. METHODS: Electronic medical records were examined for adult patients with ICD-9 code 710.3 between January 2001 and November 2014 (n=511). Physician diagnosis, clinical findings and diagnostic testing results were recorded. A dermatomyositis rating scale was assigned based on classic cutaneous findings and at least 2 additional clinical and diagnostic findings from the Bohan criteria...
August 26, 2016: Arthritis Care & Research
Manabu Fujimoto, Rei Watanabe, Yosuke Ishitsuka, Naoko Okiyama
PURPOSE OF REVIEW: In dermatomyositis, disease-specific autoantibodies now cover more than 70% of patients. These autoantibodies closely correlate with distinct clinical manifestations. In the past few years, extensive evidence has been accumulated on clinical significance of dermatomyositis-specific autoantibodies including autoantibodies against melanoma differentiation antigen 5 (MDA5), transcriptional intermediary factor 1 (TIF1), nuclear matrix protein 2 (NXP2), and small ubiquitin-like modifier activating enzyme (SAE)...
November 2016: Current Opinion in Rheumatology
Cecilia Beatrice Chighizola, Voon H Ong, Pier Luigi Meroni
Cyclosporine A, an inhibitor of calcineurin, exerts an immunomodulator action interfering with T cell activation. Even though novel therapeutic tools have emerged, CyA still represents a suitable option in several clinical rheumatology settings. This is the case of refractory nephritis and cytopenias associated with systemic lupus erythematosus. Furthermore, CyA is a valued therapeutic tool in the management of uveitis and thrombophlebitis in course of Behçet's disease. Topical CyA has been proven to be beneficial in the dry eye of Sjogren's syndrome, whereas oral treatment with CyA can be considered for the severe complications of adult onset Still's disease...
August 11, 2016: Clinical Reviews in Allergy & Immunology
Felicitas Bellutti Enders, Brigitte Bader-Meunier, Eileen Baildam, Tamas Constantin, Pavla Dolezalova, Brian M Feldman, Pekka Lahdenne, Bo Magnusson, Kiran Nistala, Seza Ozen, Clarissa Pilkington, Angelo Ravelli, Ricardo Russo, Yosef Uziel, Marco van Brussel, Janjaap van der Net, Sebastiaan Vastert, Lucy R Wedderburn, Nicolaas Wulffraat, Liza J McCann, Annet van Royen-Kerkhof
BACKGROUND: In 2012, a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile dermatomyositis (JDM) is a rare disease within the group of paediatric rheumatic diseases (PRDs) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience...
August 11, 2016: Annals of the Rheumatic Diseases
Benito Lourenço, Katia T Kozu, Gabriela N Leal, Marco F Silva, Elisabeth G C Fernandes, Camila M P França, Fernando H C Souza, Clovis A Silva
Contraception is an important issue and should be a matter of concern in every medical visit of adolescent and young patients with chronic rheumatic diseases. This narrative review discusses contraception methods in adolescents with juvenile systemic lupus erythematosus (JSLE), antiphospholipid syndrome (APS), juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM). Barrier methods are safe and their use should be encouraged for all adolescents with chronic rheumatic diseases. Combined oral contraceptives (COC) are strictly prohibited for JSLE and APS patients with positive antiphospholipid antibodies...
July 16, 2016: Revista Brasileira de Reumatologia
Satoru Teshigawara, Yoshinori Katada, Yuichi Maeda, Maiko Yoshimura, Eriko Kudo-Tanaka, Soichiro Tsuji, Yoshinori Harada, Masato Matsushita, Shiro Ohshima, Kotaro Watanabe, Takahiro Kumode, Yoshihiko Hoshida, Yukihiko Saeki
BACKGROUND: Hemophagocytic lymphohistiocytosis associated with autoimmune diseases is seen in patients with systemic juvenile idiopathic arthritis, adult-onset Still's disease, and systemic lupus erythematosus, whereas it is rarely seen in patients with dermatomyositis. In addition, central nervous system involvement with dermatomyositis is rare. To the best of our knowledge, this is the first case of hemophagocytic lymphohistiocytosis complicated by leukoencephalopathy in a patient with dermatomyositis accompanied with peripheral T-cell lymphoma...
2016: Journal of Medical Case Reports
Shin Ohta, Ki-Ichi Unoda, Hideto Nakajima, Soichiro Ikeda, Yasuhito Hamaguchi, Fumiharu Kimura
Myositis-specific autoantibodies (MSAs) are associated with myositis. Anti-nuclear matrix protein 2 (NXP-2) antibody was recently identified as a major MSA and was observed mostly in juvenile dermatomyositis. We report the case of a 44-year-old man who presented with myopathy with anti-NXP-2 antibody and large cell carcinoma of the lung. He was hospitalized because of myalgia and edema of limbs. Neurological examination revealed mild proximal-dominant weakness in all four extremities, and laboratory studies showed elevated creatine kinase level (6,432 IU/l)...
August 31, 2016: Rinshō Shinkeigaku, Clinical Neurology
Andrea Váncsa, Katalin Dankó
Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans...
July 2016: Orvosi Hetilap
K O Schneider, C Braeuninger, F Bergmann, B Kammer
No abstract text is available yet for this article.
July 2016: Klinische Pädiatrie
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