Antisynthetase syndrome: Analysis of 11 cases

Zamarrón, Ester; Carrera, Luis Gómez; Bonilla, Gema; Petit, D.; Mangas, Alberto; Álvarez-Sala, R

doi:10.1016/j.medcle.2017.02.018

Cited by 3 publications

(3 citation statements)

References 11 publications

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“…ILD is the first manifestation in many patients and is easily missed and misdiagnosed (13). Previous data show that patients with ASS have a high incidence of ILD, which is the main cause of morbidity and death (14)(15)(16). However, the pathogenesis of ASS-ILD remains unclear.…”

Section: Discussionmentioning

confidence: 99%

Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

Zhao

Jiang

et al. 2022

Front. Immunol.

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ObjectiveThis study aimed to analyze the clinical features and prognostic factors of imaging progression and survival in patients with antisynthetase syndrome (ASS) complicated by interstitial lung disease (ILD) in a large Chinese cohort.MethodsMedical records, imaging, and serological data of 111 patients with ASS-ILD (positive for at least one of the following autoantibodies: anti-Jo1, anti-PL7, anti-PL12, and anti-EJ) from the Affiliated Yantai Yuhuangding Hospital of Qingdao University database were retrospectively investigated. According to the changes in high-resolution computed tomography (HRCT) outcomes at 1 year follow-up, Patients were categorized into three groups: the regression, stability, and deterioration groups. Univariate analysis was performed to evaluate the possible prognostic factors of ILD outcome and death, and multivariate analysis was performed to determine the independent predictors of ASS-ILD outcome and death by logistic regression.ResultsThe number of CD3-CD19+ cells and initial glucocorticoid dosage were correlated with imaging progression, and may be independent risk factors for ILD deterioration. Dyspnea as the first symptom, hypohemoglobinemia, the serum ferritin level, oxygen partial pressure at diagnosis, and different treatment types were important factors affecting survival, and the initial serum ferritin level may be an independent risk factor for survival.ConclusionsThe clinical characteristics of patients with ASS-ILD with different antisynthetase antibody subtypes are different. An increase in the CD3-CD19+ cell level is an independent risk factor for the deterioration of HRCT imaging. Early intensive treatment with high-dose glucocorticoids can effectively improve imaging prognosis of ILD. Patients with significantly elevated serum ferritin levels should be treated intensively.

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Section: Discussionmentioning

confidence: 99%

Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

Zhao

Jiang

et al. 2022

Front. Immunol.

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“…The exception is in those patients with the PM that are positive for anti-Jo, anti-Mi, and anti-SRP antibodies (201). The anti-Jo-1 is the primary antibody that defines the antisynthetase syndrome (202,203), and the other antibodies support the diagnosis. The most specific antibody for DM is the anti-Mi-2 (204), where the anti-SRP is present in the immune-mediated necrotizing myopathy variant (205).…”

Section: Clinical Manifestations Of CV Involvement and Pathogenesis O...mentioning

confidence: 99%

“…Patients with cardiac involvement have a poor prognosis and are responsible for 50% of the deaths of this disease (208)(209)(210)(211)(212). Therefore, all asthmatic patients with dyspnea, vasculitis, and hypereosinophilia suspected of EGPA cardiac involvement should be considered (207) since early identification and diagnosis of the cardiac involvement and proper treatment may prevent the progression of the cardiac disease (203).…”

Section: Clinical Manifestations Of CV Involvement and Pathogenesis O...mentioning

confidence: 99%

Cardiac Magnetic Resonance in Rheumatology to Detect Cardiac Involvement Since Early and Pre-clinical Stages of the Autoimmune Diseases: A Narrative Review

Sierra-Galan

Bhatia

Alberto-Delgado

et al. 2022

Front. Cardiovasc. Med.

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Autoimmune diseases (ADs) encompass multisystem disorders, and cardiovascular involvement is a well-known feature of autoimmune and inflammatory rheumatic conditions. Unfortunately, subclinical and early cardiovascular involvement remains clinically silent and often undetected, despite its well-documented impact on patient management and prognostication with an even more significant effect on severe and future MACE events as the disease progresses. Cardiac magnetic resonance imaging (MRI), today, commands a unique position of supremacy versus its competition in cardiac assessment and is the gold standard for the non-invasive evaluation of cardiac function, structure, morphology, tissue characterization, and flow with the capability of evaluating biventricular function; myocardium for edema, ischemia, fibrosis, infarction; valves for thickening, large masses; pericardial inflammation, pericardial effusions, and tamponade; cardiac cavities for thrombosis; conduction related abnormalities and features of microvascular and large vessel involvement. As precise and early detection of cardiovascular involvement plays a critical role in improving the outcome of rheumatic and autoimmune conditions, our review aims to highlight the evolving role of CMR in systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), rheumatoid arthritis (RA), systemic sclerosis (SSc), limited sclerosis (LSc), adult-onset Still's disease (AOSD), polymyositis (PM), dermatomyositis (DM), eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome), and DRESS syndrome (DS). It draws attention to the need for concerted, systematic global interdisciplinary research to improve future outcomes in autoimmune-related rheumatic conditions with multiorgan, multisystem, and cardiovascular involvement.

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Defining anti-synthetase syndrome: a systematic literature review

Zanframundo

Faghihi-Kashani

Scirè

et al. 2022

Clinical and Experimental Rheumatology

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Anti-synthetase syndrome (ASSD) is a heterogeneous autoimmune disease characterised by multi-system involvement with a wide variety of manifestations. Validated classification criteria are necessary to improve recognition and prevent misclassification, especially given the lack of reliable and standardised autoantibody testing. We systematically reviewed the literature to analyse proposed ASSD criteria, characteristics, and diagnostic performance. PubMed and Embase databases (01/01/1984 to 06/11/2018) and the ACR and EULAR meeting abstracts (2017-2018. Sensitivities, specificities, positive, negative likelihood ratios and risk of bias were calculated for ASSD criteria and key variables reported in the literature. We performed meta-analysis when appropriate. Methods We searched ResultsWe retrieved 4,358 studies. We found 85 proposed ASSD criteria from a total of 82 studies. All but one study included anti-synthetase autoantibody (ARS) positivity in the ASSD criteria. Most studies required only one ASSD feature plus anti-ARS to define ASSD (n=64, 78%), whereas 16 studies required more than one ASSD variable plus anti-ARS. The only criteria not including anti-ARS positivity required 5 ASSD clinical features. We found limited data and wide variability in the diagnostic performance of each variable and definition proposed in the literature. Given these limitations we only meta-analysed the performance of individual muscle biopsy and clinical variables in diagnosing ASSD, which performed poorly. ConclusionThe current ASSD criteria include a variety of serological, clinical, and histological features with wide variability amongst proposed definitions and the performance of these definitions has not been tested. This systematic literature review suggests the need for additional data and consensus-driven classification criteria for ASSD.

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Antisynthetase syndrome: Analysis of 11 cases

Cited by 3 publications

References 11 publications

Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

Cardiac Magnetic Resonance in Rheumatology to Detect Cardiac Involvement Since Early and Pre-clinical Stages of the Autoimmune Diseases: A Narrative Review

Defining anti-synthetase syndrome: a systematic literature review

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