Anti-MDA5 Antibodies in a Large Mediterranean Population of Adults with Dermatomyositis

Labrador‐Horrillo, Moisés; Martínez, María Ángeles; Selva-O’Callaghan, Albert; Trallero-Araguás, Ernesto; Balada, Eva; Vilardell‐Tarrés, Miquel; Júarez, Cándido

doi:10.1155/2014/290797

Cited by 157 publications

(162 citation statements)

References 26 publications

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“…The anti-MDA5 autoantibodies are specific for dermatomyositis, and may be found in 7-13% of adult dermatomyositis patients (table 2) [34][35][36]. Clinically significant myositis is present in only 20% of patients with anti-MDA5 autoantibodies [37].…”

Section: Classification Based On Autoantibodiesmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

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Idiopathic inflammatory myositis (IIM) is a group of rare connective tissue diseases (CTDs) characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD) is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD. @ERSpublications Interstitial lung disease is a leading cause of morbidity in dermatomyositis/polymyositis

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Section: Classification Based On Autoantibodiesmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

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“…43,[45][46][47][48] Interestingly, these characteristics of anti-MDA5-positive patients appear not to be the same in American and European cohorts. [49][50][51][52] The frequency of anti-MDA5 antibodies in American and European cohorts (5-10% in adult DM) is lower than in Asian cohorts. Furthermore, anti-MDA5 antibodies are associated with ILD (60-70%) but not with RP-ILD, in American and European cohorts.…”

Section: Anti-mda5 Antibodymentioning

confidence: 99%

Clinical significance and new detection system of autoantibodies in myositis with interstitial lung disease

Nakashima

Hosono

Mimori

2016

Lupus

103

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Anti-aminoacyl-tRNA synthetase (ARS) and anti-melanoma differentiation-associated gene 5 ( MDA5) antibodies are closely associated with interstitial lung disease in polymyositis and dermatomyositis. Anti-ARS-positive patients develop common clinical characteristics termed anti-synthetase syndrome and share a common clinical course, in which they respond well to initial treatment with glucocorticoids but in which disease tends to recur when glucocorticoids are tapered. Anti- MDA5 antibody is associated with rapidly progressive interstitial lung disease and poor prognosis, particularly in Asia. Therefore, intensive immunosuppressive therapy is required for anti- MDA5-positive patients from the early phase of the disease. New enzyme-linked immunosorbent assays to detect anti-ARS and anti- MDA5 antibodies have recently been established and are suggested to be efficient and useful. These assays are expected to be widely applied in daily practice.

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“…Zaobserwowano także zależność pomiędzy obecnością tych przeciwciał a zespołem objawów śluzówkowo-skórnych polegającym na tworzeniu się owrzodzeń i/lub bolesnych grudek na dłoniach oraz owrzodzeń błony śluzowej jamy ustnej [33]. Ze względu na silny związek obecności przeciwciał anty-MDA5 z ILD mogą one służyć jako przydatny biomarker oceny ryzyka wystąpienia zajęcia płuc u osób bez przeciwciał antysyntetazowych [34]. [35,36].…”

Section: Przeciwciała Anty-mda5 (Anty-cadm-140)unclassified

Special papers Diagnostic and therapeutic advances in dermatomyositis

Samotij¹,

Szczęch²,

Reich³

2015

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StreSzczenieZapalenie skórno-mięśniowe (dermatomyositis -DM) zalicza się do grupy tzw. idiopatycznych miopatii zapalnych (ang. idiopathic inflammatory myopathies -IIM). Schorzenie to ma podłoże autoimmunologiczne, cechuje się obecnością zmian skórnych i/lub objawami zapalenia mięśni. Wyróżnia się tzw. klasyczną postać DM, postać dziecięcą DM oraz paraneoplastyczną DM, DM indukowane lekami i DM bez zaję-cia mięśni. Ostatnio obserwuje się dynamiczny postęp w identyfikacji nowych autoprzeciwciał towarzyszących poszczególnym odmianom DM, w tym anty-TIF1, anty-NXP2, anty-SAE czy anty-MDA5. Prawdopodobnie w niedalekiej przyszłości przyczyni się on do zwiększenia częstości rozpoznawania DM i jego poszczególnych odmian, lepszej stratyfikacji pacjentów pod względem ryzyka zajęcia poszczególnych narządów wewnętrznych oraz wpłynie na całościową poprawę wyników leczenia. Obecnie glikokortykosteroidy są podstawą terapii DM, jednak wprowadzenie do lecznictwa szeregu nowych leków immunomodulujących i immunosupresyjnych, w tym leków biologicznych, wiąże się ze znaczącym zwiększeniem przeżywalności pacjentów z tą chorobą. W niniejszej pracy dokonano przeglądu piśmiennictwa na temat najnowszych trendów w diagnostyce i leczeniu DM. AbStrActDermatomyositis (DM) is one of the so-called idiopathic inflammatory myopathies (IIM). Dermatomyositis is an autoimmune disorder characterized by the presence of cutaneous lesions and/or symptoms of muscle involvement with the following variants: the "classic" variant of DM, juvenile DM, paraneoplastic DM, drug-induced DM and amyopathic DM. Dynamic discoveries of novel autoantibodies, including anti-TIF1, anti-NXP2, anti-SAE or anti-MDA5, related to certain DM variants, have been described in recent years. It seems that these antibodies will contribute to better recognition of DM and its particular variants, a better risk stratification for predicting internal organ involvement, and to a global improvement of treatment outcome. Corticosteroids remain the mainstay of DM therapy, but new immunomodulatory and immunosuppressive agents, including biologicals, resulted in a significant increase of the survival rate of DM patients. Here, we review the current literature data on DM with special emphasis on new trends in its diagnostics and treatment.

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Anti-MDA5 Antibodies in a Large Mediterranean Population of Adults with Dermatomyositis

Cited by 157 publications

References 26 publications

Idiopathic inflammatory myopathies and the lung

Idiopathic inflammatory myopathies and the lung

Clinical significance and new detection system of autoantibodies in myositis with interstitial lung disease

Special papers Diagnostic and therapeutic advances in dermatomyositis

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