Brief Report: Antisynthetase Syndrome–Associated Myocarditis (original) (raw)
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Unusual presentation of antisynthetase syndrome: a case series and review of the literature
Journal of Medical Case Reports
Background Antisynthetase syndrome is an inflammatory myopathy that is characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies. Only 30% of those who suffer from the disease can be identified. We present three Hispanic cases of antisynthetase syndrome with unusual clinical pictures were extended myositis panel results enable disease diagnosis and treatment. Case presentation A 57-year-old Hispanic/Latino female with an erythematous scaly plaque, unresolved fever and non-immune haemolytic anaemia in whom inpatient work-up for fever of unknown origin was positive for anti-PL12 positive myositis extended panel. A 72-year-old Hispanic/Latino male with amyopathic weakness syndrome and mechanic hands in whom impatient work-up was relevant for proximal muscle uptake and anti-PM75 and AntiPL-12 myositis extended panel. And a 67-year-old Hispanic/Latino male with progressive interstitial lung disease and unresolved fever ended in myositis extended panel positive for antiPL...
Case reports in rheumatology, 2023
Antisynthetase syndrome is a rare autoimmune disease within the subset of idiopathic infammatory myopathies. Te diagnostic criteria include the presence of an aminoacyl-tRNA synthetase antibody, and typical clinical fndings, including myositis, mechanic's hands, Raynaud phenomenon, unexplained fever, and interstitial lung disease. We describe a case of a 59-year-old male who presented with a 1-month history of progressive purplish discoloration and pain of the fngertips, dyspnea, cough, weight loss, fatigue, and who developed progressive proximal muscle weakness and dysphagia. Investigations revealed pulmonic valve and mitral valve marantic endocarditis, pulmonary embolism, myositis, organizing pneumonia, and elevation of anti-OJ antibodies. He was diagnosed with antisynthetase syndrome and treated with high dose corticosteroids and mycophenolate mofetil with a fair response.
Antisynthetase Syndrome with Anti-Pl12 Antibodies and Cardiac Involvement
Iris Publishers LLC, 2019
Background: The antisynthetase syndrome (ASS) is a rare chronic autoimmune disorder associated with a various manifestation including myositis, interstitial lung disease, fever, Raynaud’s phenomenon, “mechanic’s hands, polyarthritis and antibody specificity. Cardiac involvement is relatively rare but possible. We report a case of ASS anti PL12 positive with cardiac manifestations. Case report: A 36 year old woman was admitted to the Department of Cardiology in our hospital for presyncope episodes, and exertional dyspnea. Physical examination revealed signs of congestive heart failure. Her heart rate was 40 bpm with a second-degree AV block Mobitz I on the electrocardiography. Transthoracic echocardiography showed cardiomyopathy with severe biventricular dysfunction and pulmonary hypertension. Further investigations were done to identify the cause of this cardiomyopathy especially as the patient presented arthralgias for last 2 years and history suggestive of Raynaud’s phenomenon and weakness of limbs. They revealed ASS syndrome with anti PL12 antibodies, diffuse interstitial lung involvement and cardiac involvement. The patient was treated with diuretic and corticosteroid therapy (methylprednisolone 1g pulse). On 4th day of admission, patient developed a complete AV block. Permanent dual chamber pacemaker implantation was performed. The patient was initiated on Cyclophosphamide and oral prednisolone which resulted in improved muscle strength and exercise tolerance. Conclusion: Antisynthetase syndrome is a rare inflammatory myopathy. The cardiac involvement is even rarer and it is associated with worse outcome. The cardiovascular manifestations are various. This is the first report of ASS associated with complete heart block, cardiomyopathy and pulmonary hypertension in the same patient. Given the possible severe consequences, the cardiac involvement should be considered in patients with antisynthetase syndrome and clinicians should be familiar with this entity.
A Curious Case of Acute Respiratory Failure: Is It Antisynthetase Syndrome?
Case reports in critical care, 2016
Antisynthetase (AS) syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. Although it is usually associated with elevated creatine phosphokinase level, some patients may have amyopathic dermatomyositis (ADM) like presentation with predominant skin involvement. Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis. It may rarely present with a very aggressive course resulting in acute respiratory distress syndrome (ARDS). We report a case of a 43-year-old male who presented with nonresolving pneumonia who was eventually diagnosed to have ADM through a skin biopsy without any muscle weakness. ADM may be associated with rapidly progressive course of interstitial lung disease (ADM-ILD) which is associated with high mortality. Differentiation between ADM-ILD and AS syndrome may be difficult in the absence of positive serology and clinical presentation ...
Interstitial lung disease in a patient with antisynthetase syndrome and no myositis
Clinical Rheumatology, 2007
Interstitial lung disease in patients with antisynthetase syndrome and no evidence of myositis is rare and may precede other disease manifestations. We report a patient who initially presented with symptoms primarily related to lung involvement. The diagnosis of the antisynthetase syndrome without myositis was made many months later when he developed a characteristic hand rash (mechanic's hands), which was confirmed by positive antibodies to Jo-1. With treatment, both the hand rash and the interstitial lung disease improved. Antisynthetase syndrome should be considered in patients presenting with interstitial lung disease with no evidence of myositis. Appropriate laboratory testing with measurement of specific autoantibodies may help in the early diagnosis and treatment of the syndrome.
Antisynthetase syndrome: An under-recognized cause of interstitial lung disease
Lung India, 2016
mechanic's hand and Raynaud's phenomenon (RP). [1] Though anti-ARS antibodies were initially considered to be myositis specific, subsequent studies have shown that they characterize their own clinical phenotype. [1] The clinical presentation of AS is variable and partly depends on the type of anti-ARS antibody. [2] Though typical Background: Antisynthetase syndrome (AS) is an uncommon and under-recognised connective tissue disease characterized by the presence of antibodies to anti-aminoacyl t-RNA synthetase along with features of interstitial lung disease (ILD), myositis and arthritis. The aim of the current study is to describe our experience with management of AS. Materials and Methods: This was a 2-year (2013-2014) retrospective analysis of patients diagnosed with anti-Jo-1-related AS. The presence of anti-Jo-1 antibody was tested by the immunoblot assay. All patients underwent high-resolution computed tomography of the chest, transthoracic echocardiography and evaluation for inflammatory myositis. Transbronchial lung biopsies and muscle biopsies were obtained when clinically indicated. Results: Nine patients (mean age: 43.8 years) were diagnosed with anti-Jo-1-related AS. The median duration of symptoms before diagnosis of AS was 6 months. All patients were negative for antinuclear antibodies by indirect immunofluorescence. The prevalence of ILD, myositis and arthritis at presentation was 100%, 77.8% and 55.6%, respectively. The most common ILD pattern was non-specific interstitial pneumonia (n = 6) followed by organizing pneumonia (n = 2) and usual interstitial pneumonia (n = 1). ILD was the sole manifestation in two patients and was subclinical in two patients. Six patients had pleuropericardial effusions, three patients had pulmonary artery hypertension and two patients had venous thromboembolism. Eight of the nine patients improved after treatment with steroids and other immunosuppressants. Conclusion: Antisynthetase syndrome is an important and a treatable cause of ILD. Strong clinical suspicion is needed to achieve an early diagnosis.
A multidisciplinary approach to the diagnosis of antisynthetase syndrome
Frontiers in Medicine
Antisynthetase syndrome is a subtype of idiopathic inflammatory myopathy, strongly associated with the presence of interstitial lung disease. Diagnosis is made by identifying myositis-specific antibodies directed against aminoacyl tRNA synthetase, and relevant clinical and radiologic features. Given the multisystem nature of the disease, diagnosis requires the careful synthesis of subtle clinical and radiological features with the interpretation of specialized autoimmune serological testing. This is provided in a multidisciplinary environment with input from rheumatologists, respiratory physicians, and radiologists. Differentiation from other idiopathic interstitial lung diseases is key; treatment and prognosis differ between patients with antisynthetase syndrome and idiopathic interstitial lung disease. In this review article, we look at the role of the multidisciplinary team and its individual members in the initial diagnosis of the antisynthetase syndrome, including the role of p...
Antisynthetase syndrome a rare and challenging diagnosis-case report and literature review
Rheumatology (Bulgaria)
This case describes a 46-years old woman who initially presented with myopathy and arthropathy (i.e. musculoskeletal manifestations), which delayed the recognition of antisynthetase syndrome (ASS) and contributed for considering the condition as seronegative rheumatoid arthritis for several years. During the next few years, the patient was progressively worsening, with a disability to stand up from a sitting position, gradual onset of exertional dyspnea, difficult-to-control dry cough and thick, hyperkeratotic skin of both hands (mechanic’s hands). This constellation of symptoms was highly suspicious for ASS and additional serological and radiological examinations were done which confirmed the diagnosis of ASS. The need for further detailed investigation when an interstitial lung disease overlaps with a known rheumatoid condition is obligatory, as shown in this case. A multidisciplinary evaluation is highly recommended to evaluate the clinical, serological and radiological findings ...
The clinical phenotype associated with antisynthetase autoantibodies
Reumatologia/Rheumatology, 2020
ObjectivesSpecific systemic autoimmune syndrome characterized by inflammatory myopathy, arthritis or arthralgias, interstitial lung disease (ILD), fever, Raynaud’s phenomenon, and mechanic’s hands is called antisynthetase syndrome (AS). The aim of this study was to assess the clinical spectrum associated with presence of aminoacyl-transfer RNA synthetase autoantibodies (ASA).Material and methodsA total of 305 patients with presence of myositis-specific autoantibodies were identified in the database of immunological tests performed in the Clinical Immunology and Transplantology Unit, Medical University of Gdansk between January 2011 and March 2016. In 110 patients (36%) ASA were detected. The detailed analysis included 50 patients with ASA for whom full clinical data were available.ResultsThe incidence of specific ASA in the analyzed group was: Jo-1 46% (23 patients), PL-12 32% (16 patients), PL-7 16% (8 patients), OJ 12% (6 patients), EJ 6% (3 patients). In 10% (5 patients) there was coexistence of at least one ASA, and in another 5 patients there was coexistence of ASA with other antibodies specific for myositis (MSA). In the analyzed group of patients 11 (22%) satisfied the Bohan and Peter criteria for dermatomyositis, 1 for polymyositis. In 5 patients (10%) based on clinical presentation and ASA presence the AS was recognized. Another 3 patients met the criteria of the overlap syndrome polymyositis respectively with systemic lupus, rheumatoid arthritis, and scleroderma. In 5 patients undifferentiated connective tissue disease was diagnosed, and 14 consecutive patients were diagnosed with other connective tissue diseases, while 12 patients did not receive a definitive diagnosis.ConclusionsThe clinical presentation of patients with the presence of ASA is varied. Their presence indicates not only idiopathic inflammatory myopathies, but also non-specifically other disease entities. These patients require observation for the development of idiopathic inflammatory myopathy, and ILD.