Fatal cardiac involvement complicating antisynthetase syndrome

Brady, Stefen; Melath, Sunil; Scalco, R.; Penn, Henry

doi:10.1136/bcr-2014-204409

Cited by 8 publications

(8 citation statements)

References 6 publications

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“…A registry of 352 ASS cases found 12 patients with ASS-associated myocarditis, of whom 75% had full recovery following appropriate therapy. Another case published in 2014 described fatal cardiac involvement 7. Myocarditis in ASS is rare and may be the first presenting feature of this syndrome.…”

Section: Discussionmentioning

confidence: 99%

When your immune system falls out with your heart: an important lesson on antisynthetase myocarditis

Gorecka¹,

Tummon

Smyth

et al. 2018

BMJ Case Reports

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A 57-year-old special Olympics athlete presented with subacute onset dyspnoea. Baseline investigations revealed troponin T rise and an abnormal ECG, which prompted coronary angiography. This was unremarkable, as was his transthoracic echocardiography (TTE). He re-presented 7 months later with progressive dyspnoea associated with significant weight loss, peripheral oedema and intermittent fevers. Examination revealed bilateral fine end-inspiratory crackles, peripheral oedema and fever. Investigations revealed elevated troponin T and raised inflammatory markers. ECG remained unchanged, whereas TTE revealed mild global impairment of left ventricular function. Chest radiography was suggestive of extensive interstitial lung disease, which was confirmed by high resolution CT. Presence of interstitial lung disease and myocarditis raised the suspicion of a systemic inflammatory condition. Subsequently, an autoimmune screen was positive for anti-Jo-1 antibody associated with antisynthetase syndrome. He was treated with high-dose steroids and rituximab with dramatic symptomatic improvement and immediate fall in troponin T level.

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Section: Discussionmentioning

confidence: 99%

When your immune system falls out with your heart: an important lesson on antisynthetase myocarditis

Gorecka¹,

Tummon

Smyth

et al. 2018

BMJ Case Reports

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“…This study showed a prevalence of myocarditis in aSS of 3.4%. However, the limitation of this study is inherent to its retrospective design, and these data could have been either underestimated due to severe undiagnosed cases 7 or overestimated due to recruitment bias (all centers being tertiary care centers). There have been no reports to date in the literature providing this specific figure for aSS, but it is comparable to what has been reported for other inflammatory myopathies.…”

Section: Discussionmentioning

confidence: 99%

“…This latter manifestation seems to be particularly rare in aSS, with only a few case reports published to date. 7 , 8 Although potentially severe, this condition could be underestimated.…”

Section: Introductionmentioning

confidence: 99%

“…Myocarditis can occur in contexts other than myopathies, including infectious or inflammatory diseases or hypereosinophilia. 7 , 8 Diagnosing myocarditis remains difficult and the clinical classification criteria are still a matter of debate. Based on the WHO classification, myocarditis is an inflammatory myocardial injury and is defined based on an endomyocardial biopsy (EB) with specific histological and immunological features.…”

Section: Introductionmentioning

confidence: 99%

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Myocarditis in Patients With Antisynthetase Syndrome

et al. 2015

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Antisynthetase syndrome (aSS) corresponds to an overlapping inflammatory myopathy identified by various myositis-specific autoantibodies (directed against tRNA-synthetases). Myocardial involvement in this condition is poorly described.From a registry of 352 aSS patients, 12 cases of myocarditis were retrospectively identified on the basis of an unexplained increase in troponin T/I levels associated with either suggestive cardiac magnetic resonance imaging (MRI) findings, nonsignificant coronary artery abnormalities or positive endomyocardial biopsy.The prevalence of myocarditis in aSS is 3.4% and was not linked to any autoantibody specificity: anti-Jo1 (n = 8), anti-PL7 (n = 3), and anti-PL12 (n = 1). Myocarditis was a part of the first aSS manifestations in 42% of the cases and was asymptomatic (n = 2) or revealed by an acute (n = 4) or a subacute (n = 6) cardiac failure. It should be noted that myocarditis was always associated with an active myositis. When performed (n = 11), cardiac MRI revealed a late hypersignal in the T1-images in 73% of the cases (n = 8). Half of the patients required intensive care. Ten patients (83%) received dedicated cardiotropic drugs. Steroids and at least 1 immunosuppressive drug were given in all cases. After a median follow-up of 11 months (range 0–84) 9 (75%) patients recovered whereas 3 (25%) developed a chronic cardiac insufficiency. No patient died.The prevalence of myocarditis in aSS is similar to that of other inflammatory myopathies. Although the prognosis is relatively good, myocarditis is a severe condition and should be carefully considered as a possible manifestation in active aSS patients.

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“…Anti-synthetase antibodies have also been detected in a case of cryptogenic organizing pneumonia (93) and in association with uncommon clinical manifestations such as aphthous-like ulcerations and xerostomia (94). As for their prognostic significance, anti-PL-12 and PL-7 antibodies are related to lower survival rates (81,82), and anti-EJ antibodies have been reported in a case of fatal myocarditis in anti-synthetase syndrome (95).…”

Section: Autoabs In Polymyositis/dermatomyositis (Pm/dm) and In The Amentioning

confidence: 99%

Clinical significance of rare serum autoantibodies in rheumatic diseases: a systematic literature review

Ceribelli¹,

Isailovic²,

Santis³

et al. 2018

J. Lab. Precis. Med

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The identification of serum autoantibodies is central in the diagnosis of systemic autoimmune rheumatic disease (SARD), and an increasing number of specificities have been detected in the past years.This allows an early diagnosis in the active phases of diseases, with the identification of specific disease subsets that may ultimately improve the disease outcomes. Thanks to the use of old and new laboratory techniques that are becoming increasingly available worldwide, the number of rheumatic patients with a specific autoantibody is increasing and this is improving also our knowledge of disease trigger mechanisms.The paradigmatic example is the plethora of serum autoantibodies described in polymyositis and dermatomyositis, coined myositis-specific antibodies (MSA) which include antibodies directed against tRNA synthetases, anti-SRP, anti-Mi-2, and anti-TIF-1γ and can discriminate disease subtypes, particularly when associated with the risk of cancer. As a further example, anti-HMGCR antibodies have been reported in several studies in association with necrotizing autoimmune myositis that may follow statin use. To clarify the current knowledge on these rare specificities, we performed a systematic literature review. We focused on the main features associated to specific autoantibodies that are rarely identified in rheumatic disease, to increase the awareness and scientific knowledge on these autoantibodies in different ethnic groups worldwide. Inclusion criteriaObservational studies, case reports and clinical trials were included. Exclusion criteriaArticles not concerning SARD, and reviews or editorials in languages different from English, if including children or animals, were excluded to limit the literature review to adults and because no funding was available for translation. The selection process was performed by two authors, based on titles, abstracts and subsequently full text papers. Figure 1 represents the flowchart of the selection process of this systematic literature review. Data extractionThe year of publication, study design, number of patients and demographic data were recorded. The outcome was defined by the identification of rare autoAbs and their prevalence and clinical significance in SARD. Articles were divided into categories depending on the disease.

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Fatal cardiac involvement complicating antisynthetase syndrome

Cited by 8 publications

References 6 publications

When your immune system falls out with your heart: an important lesson on antisynthetase myocarditis

When your immune system falls out with your heart: an important lesson on antisynthetase myocarditis

Myocarditis in Patients With Antisynthetase Syndrome

Clinical significance of rare serum autoantibodies in rheumatic diseases: a systematic literature review

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