Clinical practice guidance for juvenile dermatomyositis (JDM) 2018-Update

Kobayashi, Ichiro; Akioka, Shinji; Kobayashi, N.; Iwata, Nakao; Takezaki, Shunichiro; Nakaseko, Haruna; Sato, Satoshi; Nishida, Yutaka; Nozawa, Tomo; Yamasaki, Yuichi; Yamazaki, Kazuko; Arai, Satoru; Nishino, Ichizo; Mori, Masaaki

doi:10.1080/14397595.2020.1718866

Cited by 37 publications

(52 citation statements)

References 180 publications

(230 reference statements)

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“…[10]. Based on these facts, recently developed clinical practice guidance for JDM 2018 in Japan, where anti-MDA5 JDM is prevalent, recommends screening for ILD by CT scan in all cases of JDM [11].…”

Section: Discussionmentioning

confidence: 99%

“…Recent report has suggested early intervention with multi-drug therapy should be started at the diagnosis of ILD associated with anti-MDA5 JDM [ 10 ]. Based on these facts, recently developed clinical practice guidance for JDM 2018 in Japan, where anti-MDA5 JDM is prevalent, recommends screening for ILD by CT scan in all cases of JDM [ 11 ].…”

Section: Discussionmentioning

confidence: 99%

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Fatal outcome of anti-MDA5 juvenile dermatomyositis in a paediatric COVID-19 patient: a case report

Quintana-Ortega

Remesal

Valbuena

et al. 2020

Modern Rheumatology Case Reports

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Anti-melanoma differentiation-associated gene 5 juvenile dermatomyositis (anti-MDA5 JDM) is associated with high risk of developing rapidly progressive interstitial lung disease (RP-ILD). Here we report an 11-year-old girl with anti-MDA5 JDM and RP-ILD which led to a fatal outcome, further aggravated by SARS-CoV-2 infection. She was referred to our hospital after being diagnosed with anti-MDA5 JDM and respiratory failure due to RP-ILD. On admission, fibrobronchoscopy with bronchoalveolar lavage (BAL) revealed Pneumocystis jirovecii infection so treatment with intravenous trimethoprim-sulfamethoxazole was initiated. Due to RP-ILD worsening, immunosuppressive therapy was intensified using methylprednisolone pulses, cyclophosphamide, tofacitinib and intravenous immunoglobulin without response. She developed severe hypoxemic respiratory failure, pneumomediastinum and pneumothorax, further complicated with severe RP-ILD and cervical subcutaneous emphysema. Three realtime RT-PCR for SARS-CoV-2 were made with a negative result. In addition, she was complicated with a secondary hemophagocytic lymphohistiocytosis and a fourth real-time PCR for SARS-CoV-2 performed in BAS sample was positive. Despite aggressive treatment of RP-ILD due to anti-MDA5 JDM, there was no improvement of respiratory failure in the following days and patient developed refractory septic shock and died. Anti-MDA5 JDM patients with RP-ILD have a poor prognosis with a high mortality rate. For this reason, intensive immunosuppressive therapy is essential including the use of promising drugs such as tofacitinib. COVID-19 in children with underlying health conditions like anti-MDA5 JDM may still be at risk for disease and severe complications.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Fatal outcome of anti-MDA5 juvenile dermatomyositis in a paediatric COVID-19 patient: a case report

Quintana-Ortega

Remesal

Valbuena

et al. 2020

Modern Rheumatology Case Reports

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“…Overall, the next most common MSA is melanoma differentiation-associated gene 5 (MDA-5). In Japanese children, the frequency of MDA-5 is increased to 28% [ 59 ] vs 6% in the western world [ 52 ]. In China, the frequency of MDA-5 is similar both in children and in adults—30%—and associated with an amyopathic disease course in DM, interstitial lung disease (ILD), and digital ulcers [ 60 ].…”

Section: Serologic and Other Blood Assaysmentioning

confidence: 99%

Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy

Pachman

Nolan

DeRanieri

et al. 2021

Curr Treat Options in Rheum

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Purpose of review To identify clues to disease activity and discuss therapy options. Recent findings The diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine phosphokinase, LDH, and SGOT—which often normalize with a longer duration of untreated disease. Ultrasound identifies persistent, occult muscle inflammation. The myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are associated with specific disease course variations. Anti-NXP-2 is found in younger children and is associated with calcinosis; anti-TIF-1γ+ juvenile dermatomyositis has a longer disease course. The diagnostic rash—involving the eyelids, hands, knees, face, and upper chest—is the most persistent symptom and is associated with microvascular compromise, reflected by loss of nailfold (periungual) end row capillaries. This loss is associated with decreased bioavailability of oral prednisone; the bioavailability of other orally administered medications should also be considered. At diagnosis, at least 3 days of intravenous methyl prednisolone may help control the HLA-restricted and type 1/2 interferon–driven inflammatory process. The requirement for avoidance of ultraviolet light exposure mandates vitamin D supplementation. Summary This often chronic illness targets the cardiovascular system; mortality has decreased from 30 to 1–2% with corticosteroids. New serological biomarkers indicate occult inflammation: ↑CXCL-10 predicts a longer disease course. Some biologic therapies appear promising.

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“…U dzieci częściej niż u pacjentów dorosłych dochodzi także do uogólnienia procesu chorobowego i zajęcia przewodu pokarmowego (zaburzenia motoryki, owrzodzenia) zarówno wskutek waskulopatii, jak i zajęcia mięśni [50]. Do wskaźników prognostycznych ciężkiego przebiegu DM u dzieci należą: zmiany rumieniowe w okolicy mostka, objaw szala i zapalenie stawów [1,51].…”

Section: Inhibitory Czynnika Martwicy Nowotworów (Tnf)unclassified

“…In children, more often than in adults, the disease becomes generalized and the gastrointestinal tract is affected (motor disorders, ulcerations), both due to vasculopathy and muscle involvement [50]. Prognostic indicators of a severe course of DM in children include erythematous changes around the neckline, "scarf" symptom and arthritis [1,51].…”

Section: Prognosismentioning

confidence: 99%

Dermatomyositis. Diagnostic and therapeutic recommendations of the Polish Dermatological Society

Reich¹,

Lis‐Święty²,

Krasowska³

et al. 2021

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Dermatomyositis is an autoimmune disease characterized by skin lesions and/or symptoms of myositis. In addition to the so-called classic dermatomyositis the following forms of dermatomyositis are distinguished based on the clinical presentation: pediatric, paraneoplastic, drug-induced and amyopathic. A number of disease-specific autoantibodies are identified in dermatomyositis (including anti-Mi2, anti-TIF1, anti-NXP2, anti-SAE or anti-MDA5), presence of which may be associated with a specific clinical phenotype. The diagnosis of the severity of muscle involvement is currently based mainly on physical examination, deviations in results of laboratory investigations, electromyographic examination and imaging examination, mainly with the use of magnetic resonance imaging. Systemic glucocorticosteroids administered as monotherapy or in combination with other immunosuppressants remain the mainstay of dermatomyositis treatment, and in the absence of satisfactory improvement, intravenous immunoglobulins are used. In addition, in case of interstitial lung disease, the use of cyclophosphamide may be necessary. The choice of a therapy, as well as the rate of dose reduction, depend on the dynamics of the disease, symptoms, diagnosed immunological disorders, as well as comorbidities and the drugs used. Each diagnostic and therapeutic decision must take into account the individual clinical data of the patient and current scientific reports.

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Clinical practice guidance for juvenile dermatomyositis (JDM) 2018-Update

Cited by 37 publications

References 180 publications

Fatal outcome of anti-MDA5 juvenile dermatomyositis in a paediatric COVID-19 patient: a case report

Fatal outcome of anti-MDA5 juvenile dermatomyositis in a paediatric COVID-19 patient: a case report

Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy

Dermatomyositis. Diagnostic and therapeutic recommendations of the Polish Dermatological Society

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