A systematic review of juvenile-onset clinically amyopathic dermatomyositis

Gerami, Pedram; Walling, Hobart W.; Lewis, Jennifer; Doughty, Lesley; Sontheimer, Richard D.

doi:10.1111/j.1365-2133.2007.08055.x

Cited by 97 publications

(98 citation statements)

References 45 publications

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“…However, over the past decade the clinical picture of CADM has started to come into sharper focus as a result of additional descriptive case reports, case series, summative systematic literature reviews and one population-based study (40)(41)(42)(43)(44).…”

Section: Prognosis Of Patients Presenting With Cadmmentioning

confidence: 99%

MDA5 autoantibody—another indicator of clinical diversity in dermatomyositis

Sontheimer¹

2017

Annals of Translational Medicine

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Allenbach and colleagues have recently reported for the first time the results of an intriguing study of the histopathologic, immunopathologic and gene expression differences in muscle biopsy tissue from adult dermatomyositis (DM) patients who do and do not have circulating MDA5 autoantibodies (anti-MDA5). Anti-MDA5 were originally identified in a clinically-defined subset of DM patients whose disease was expressed predominately in the skin for unusually long periods of time without accompanying muscle weakness [i.e., "clinically-amyopathic DM" (CADM)] and were at risk for acute, rapidly-progressive form of interstitial lung disease (ILD). As an academic dermatologist in the United States of America (USA) having a career-long interest in the CADM subset, I would like to share my perspective on the results of the work by Allenbach and colleagues and offer some suggestions for additional study in this area. But to do so most effectively, I first would like to review the clinical concept of CADM and its association with anti-MDA5 antibody production and a potentially-fatal form of (ILD).

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Section: Prognosis Of Patients Presenting With Cadmmentioning

confidence: 99%

MDA5 autoantibody—another indicator of clinical diversity in dermatomyositis

Sontheimer¹

2017

Annals of Translational Medicine

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“…ADM is a clinical subtype of DM characterized by elevated muscle enzymes with the absence of motor weakness (16)(17)(18)(19)(20)(21)(22). Physicians should be watchful for accompanying ILD or malignant disease, as both can strongly affect the prognosis.…”

Section: Discussionmentioning

confidence: 99%

“…ILD was detected in 15% of the ADM patients reported by Gerami, with a mortality rate of 39%. Another 14% of the ADM patients reported by Gerami suffered from accompanying malignant diseases (17).…”

Section: Discussionmentioning

confidence: 99%

Amyopathic Dermatomyositis Complicated with Eosinophilic Pneumonia

et al. 2014

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A 67-year-old woman was admitted to our hospital due to dyspnea on exertion with lung abnormal shadows. A transbronchial lung biopsy specimen demonstrated eosinophilic pneumonia (EP). The patient also exhibited heliotrope eyelids without muscle weakness, which led to a diagnosis of amyopathic dermatomyositis (ADM). As we were unable to find any other causes of EP, we diagnosed her as having EP associated with ADM. Dermatomyositis (DM) has been reported to be associated with various interstitial lung diseases; however, only one case of EP associated with DM has been reported. We herein report the first case of EP complicated with ADM.

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“…Among amyopathic DM patients, calcinosis is uncommon, while an association with interstitial lung disease has been rarely identified [6]. Notably, almost 26 % of these patients subsequently develop a classic JDM over several years [14]. For this reason, a close clinical monitoring aimed at identifying progression toward muscle involvement is mandatory beyond treatment of cutaneous manifestations.…”

Section: Clinical Feature and Classificationmentioning

confidence: 99%

Idiopathic Inflammatory Myopathies: an Update on Classification and Treatment with Special Focus on Juvenile Forms

Pagnini

Vitale

Selmi

et al. 2015

Clinic Rev Allerg Immunol

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Juvenile inflammatory myopathies represent a heterogeneous group of rare and potentially fatal disorders of unknown aetiology, characterised by inflammation and proximal and symmetric muscle weakness. Beyond many similarities, specific clinical, laboratoristic and histopathologic features underlie different subsets with distinguishing demographic, prognostic and therapeutic peculiarities. Over time, several forms of inflammatory idiopathic myopathies have been described, including macrophagic myofascitis, immune-mediated necrozing myopathy and the spectrum of amyopathic dermatomyositis that include hypomyopathic dermatomyositis, inclusion body myositis and cancer-associated myositis occurring almost exclusively in adults. However, juvenile dermatomyositis is the most frequent in childhood, whereas polymyositis is relatively more frequent in adults. The aetiology is nowadays widely unclear; however, current theories contemplate a combination of environmental triggers, immune dysfunction and specific tissue responses involving muscle, skin and small vessels endothelium in genetically susceptible individuals. Myositis-specific autoantibodies, found almost exclusively in patients with myositis and myositis-associated autoantibodies, detectable both among patients with myositis and in subjects suffering from other autoimmune diseases, have an important clinical role because of their relation to specific clinical features, response to therapy and prognosis. The gold standard treatment for juvenile dermatomyositis is represented by corticosteroids, along with adjunctive steroid-sparing immunosuppressive therapies, which are used to counteract disease activity, prevent mortality, and reduce long-term disability. Further treatment approach such as biologic agents and autologous stem cell transplantation are emerging during the last years, in particular in patients difficult to treat and with poor prognosis. Therefore, a highly medical specialised approach is required for diagnosis and management of these conditions. This review comprehensively examines juvenile inflammatory myopathies focusing on clinical and laboratory classifications as well as on the current treatment approaches, referring in particular on biologic agents and latest therapeutic opportunities.

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A systematic review of juvenile-onset clinically amyopathic dermatomyositis

Cited by 97 publications

References 45 publications

MDA5 autoantibody—another indicator of clinical diversity in dermatomyositis

MDA5 autoantibody—another indicator of clinical diversity in dermatomyositis

Amyopathic Dermatomyositis Complicated with Eosinophilic Pneumonia

Idiopathic Inflammatory Myopathies: an Update on Classification and Treatment with Special Focus on Juvenile Forms

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