Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis

Gono, Takahisa; Sato, Shinji; Kawaguchi, Yasushi; Kuwana, Masataka; Hanaoka, Masanori; Katsumata, Yasuhiro; Takagi, Kae; Baba, Sayumi; Okamoto, Yuko; Ota, Yukiko; Yamanaka, Hisashi

doi:10.1093/rheumatology/kes102

Cited by 291 publications

(248 citation statements)

References 26 publications

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“…Anti-MDA5-positive-dermatomyositis (anti-MDA5-positive-DM) was first described in a group of patients who lacked clinical signs of muscle involvement (clinically amyopathic dermatomyositis); however, subsequent studies have also found patients with this antibody who have classic muscle involvement with weakness and elevated muscle enzymes. [6][7][8][9][10][11] Anti-MDA5-positive-DM patients typically present with prominent skin manifestations, including heliotrope rash, Gottron's papules as well as cutaneous ulceration and tender palmar papules, the latter of which are unique to anti-MDA5-positive-DM (Table 1). 6,[12][13][14] Oral manifestations-including oral ulceration, mucosal pain, and hoarseness-are common, as are arthritis, arthralgia, and fever.…”

Section: Discussionmentioning

confidence: 99%

“…6,7,12,14,16 In comparison to other patients with dermatomyositis and ILD, anti-MDA5-positive-DM patients are more likely to have rapidly progressive disease. 7,8 The development and rapid progression of ILD may account for the significantly higher mortality observed in anti-MDA5-positive-DM patients as compared to serologically negative cohorts. 6,14,18,19 Generally, skin manifestations precede ILD in these patients.…”

Section: Discussionmentioning

confidence: 99%

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Anti-MDA5-Positive Dermatomyositis Presenting as Fever of Unknown Origin

et al. 2016

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Dermatomyositis is a chronic systemic autoimmune disease characterized by inflammatory infiltrates in the skin and muscle. The wide variability in clinical and serologic presentation poses a diagnostic challenge for the internist. Appreciation of the clinical variants of dermatomyositis allows for expedient diagnosis and avoidance of diagnostic error. We illustrate these challenges with the case of a 51-year-old VietnameseAmerican man who initially presented with fever of unknown origin in the absence of overt skin and muscle manifestations. The diagnosis of dermatomyositis was not evident on several clinical encounters due to the absence of these hallmark symptoms. We review the variable clinical manifestations of a subtype of dermatomyositis associated with an autoantibody against melanoma differentiation-associated protein 5 (anti-MDA5) and suggest consideration of dermatomyositis as a diagnosis in patients presenting with systemic illness and markedly elevated ferritin, even in the absence of elevated muscle enzymes and classic autoantibodies. CASE PRESENTATIONA 51-year-old Vietnamese-American male industrial engineer was admitted to the medical ward for evaluation of fever of unknown origin (FUO). He reported 3 weeks of fever, fatigue, generalized weakness, and dyspnea. His past medical history included a remote 10-pack-year history of cigarette smoking and latent tuberculosis treated with 9 months of isoniazid treatment, completed 5 years prior. He had moved from Vietnam to the United States 30 years earlier and denied any recent travel, sick contacts, or insect bites. Physical examination revealed a fatigued man, with a temperature of 38.1°C, pulse of 108 beats/minute, respiratory rate of 28 breaths/minute, and room air oxygen saturation of 96 %. Oropharyngeal, neurologic, pulmonary, cardiac, musculoskeletal, and skin evaluation were otherwise normal.A complete blood count (CBC) revealed anemia (hemoglobin 12.3 g/dL, hematocrit 38.5 %). White blood cell counts, platelet counts, and the basic metabolic panel were normal. Erythrocyte sedimentation rate (ESR) was 97 mm/hr and C-reactive protein (CRP) was 5.7 mg/dL. Muscle enzymes, sent to evaluate his weakness in the setting of elevated inflammatory markers, showed normal creatine kinase (CK) at 44 units/liter and mildly elevated aldolase to 9.4 U/L (normal: < 7.7 U/L). Blood, urine, and sputum cultures had no growth. Chest x-ray (CXR) demonstrated low lung volumes with increased reticular markings, small bilateral pleural effusions, and bibasilar opacities. During the hospitalization, he continued to have intermittent fevers as high as 39.1°C, generalized weakness, and dyspnea. Additionally, he developed bibasilar crackles on pulmonary exam and flat, diffuse, slightly hyperpigmented patches on his arms and chest that faded over several days. Extensive workup for infectious, inflammatory, and malignant causes of fever was unrevealing, including negative viral hepatitis serologies, human immunodeficiency virus antibody, anti-nuclear antibody (ANA), and anti-...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Anti-MDA5-Positive Dermatomyositis Presenting as Fever of Unknown Origin

et al. 2016

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“…Gono et al (2) reported that a high ferritin level on admission was a poor prognostic factor in RPIP patients with DM, including anti-MDA5 antibody ADM. Regarding autoimmune disorders, the highest ferritin levels have been reported in patients with macrophage activation syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…Anti-MDA5 antibody is closely related to rapidly progressive interstitial pneumonitis (RPIP) associated with amyopathic dermatomyositis (ADM), particularly in Asian populations. RPIP secondary to ADM can be resistant to aggressive therapy and sometimes even fatal (2). Here, we report a case of anti-MDA5 antibody-positive ADM associated with RPIP and discuss high-dose intravenous immunoglobulin (IVIg) therapy as a possible therapeutic agent.…”

Section: Introductionmentioning

confidence: 99%

High-dose intravenous immunoglobulin therapy for rapidly progressive interstitial pneumonitis accompanied by anti-melanoma differentiation-associated gene 5 antibody-positive amyopathic dermatomyositis

Hamada‐Ode

Taniguchi

Kimata³

et al. 2015

Eur J Rheumatol

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Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive amyopathic dermatomyositis (ADM) associated with rapidly progressive interstitial pneumonitis (RPIP) frequently has a poor prognosis and optimal treatment is not well defined. Here, we report a 62-year-old Japanese man with anti-MDA5 antibody-positive ADM associated with RPIP presented with progressive shortness of breath, Heliotrope rash, Gottron's papules, arthralgia, and fatigue but no sign of muscle weakness. Laboratory investigation revealed serum levels of the following biomarkers: ferritin, 1393 ng/mL; Krebs von der Lungen-6, 1880 U/mL; and creatine kinase, 85 U/L. Computed tomography (CT) images showed diffuse ground-glass opacity in both lung fields. Because anti-MDA5 was positive, we made a diagnosis of ADM associated with RPIP and initiated treatment. Following five courses of combination therapy with prednisolone, cyclosporine A, and intravenous cyclophosphamide (IVCY), IVCY treatment was switched to high-dose intravenous immunoglobulin therapy (IVIg) because of the reactivation of interstitial pneumonia with an increased serum ferritin level. Additional treatment with IVIg improved RPIP, with normalization of anti-ADM antibody levels. Therefore, IVIg mayt be a new candidate treatment for anti-MDA5 antibody-positive ADM associated with RPIP.

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“…В случае гипомиопатического варианта ДМ предполагается возможность субклинического поражения мышечной тка-ни, выявляемого при целенаправленном обследовании маркеров миозита, включая игольчатую электромиогра-фию (и-ЭМГ), биопсию и магнитно-резонансную томо-графию (МРТ) мышц. CADM чаще встречается у азиатско-го населения [18,19] и ассоциирован с высокой частотой быстропрогрессирующего ИПЛ (13-14%). Позитивность по анти-CADM-140 (MDA5) антителам рассматривается как фактор риска ИПЛ [20].…”

Section: классификацияunclassified

Polymyositis/Dermatomiositis: Differential Diagnosis

Антелава¹

2016

rsp

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Идиопатические воспалительные миопа-тии (ИВМ) -группа редких аутоиммунных ге-терогенных заболеваний неизвестной этиоло-гии, характеризующихся воспалительным по-ражением поперечно-полосатой мускулатуры и кожи, а также специфической органной па-тологией [1,2].Клиническая картина ИВМ характеризу-ется прогрессирующей симметричной мы-шечной слабостью проксимальных отделов конечностей (что ведет к обездвиженности пациентов), мышц шеи, дисфагией, измене-нием голоса (назолалией), параорбитальным отеком, формированием сгибательных сухо-жильно-мышечных контрактур.Помимо перечисленных клинических проявлений, общих для ИВМ, наблюдается фенотипическая неоднородность их подти-пов, связанная с различными морфологи-ческими и серологическими характеристи-ками [3], определяющими особенности ма-нифестации, течения и прогноза [4,5]. Фармакотерапия базируется на примене-нии высоких доз глюкокортикоидов (ГК; ≥1 мг/кг/сут) в сочетании с иммуносупрес-сивными препаратами. Терапевтическая та- 191П р о г р а м м а н е п р е р ы в н о г о п о с л е д и п л о м н о г о о б р а з о в а н и я в р а ч е й В лекции рассматривается проблема редких системных заболеваний соединительной ткани -идиопатиче-ских воспалительных миопатий (ИВМ). Подчеркивается клинико-иммунологическая неоднородность их подтипов, определяющая терапевтическую тактику и прогноз. Представлены диагностические критерии ИВМ. Предлагается алгоритм дифференциальной диагностики, который базируется на исключении феноти-пически сходных форм миопатий различного генеза. Ключевые слова: идиопатические воспалительные миопатии; полимиозит; дерматомиозит; антисинтетазный синдром; некротизирующая миопатия; миозит с включениями; дифференциальная диагностика миопатий. Для ссылки: Антелава ОА. Полимиозит/дерматомиозит: дифференциальная диагностика. Научно-практиче-ская ревматология. 2016;54(2):191-198. POLYMYOSITIS/DERMATOMIOSITIS: DIFFERENTIAL DIAGNOSISAntelava O.A.The lecture considers the problem of rare systemic connective tissue diseases, such as idiopathic inflammatory myopathies (IIMs). It underlines the clinical and immunological heterogeneity of their subtypes, which defines therapeutic tactics and prognosis. The diagnostic criteria for IIMs are given. A differential diagnostic algorithm based on the exclusion of phenotypically similar forms of myopathies of different genesis is proposed.

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Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis

Abstract: Anti-MDA5ab titre and ferritin and IL-18 concentrations are useful for the evaluation of the response to treatment and the status of ILD in patients with anti-MAD5ab-positive DM.

Cited by 291 publications

References 26 publications

Anti-MDA5-Positive Dermatomyositis Presenting as Fever of Unknown Origin

Anti-MDA5-Positive Dermatomyositis Presenting as Fever of Unknown Origin

High-dose intravenous immunoglobulin therapy for rapidly progressive interstitial pneumonitis accompanied by anti-melanoma differentiation-associated gene 5 antibody-positive amyopathic dermatomyositis

Polymyositis/Dermatomiositis: Differential Diagnosis

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