Anakinra for the Treatment of Antisynthetase Syndrome: A Monocentric Case Series and a Systematic Literature Review

Campochiaro, Corrado; Farina, Nicola; Luca, Giacomo De; Trignani, Giorgia; Tomelleri, Alessandro; Matucci‐Cerinic, M.; Dagna, Lorenzo

doi:10.3899/jrheum.220213

Cited by 4 publications

(2 citation statements)

References 10 publications

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“…Of note, in our cohort, two patients were fully responsive to the IL-1 blocking agent anakinra. Consistently, the efficacy of anakinra in treating both patients with refractory ASS and those with different types of myocarditis has been recently reported [ 31–35 ]. This evidence may indicate a possible pathogenic role of IL-1–mediated inflammation in ASS with myocarditis.…”

Section: Discussionmentioning

confidence: 60%

Myocarditis in anti-synthetase syndrome: clinical features and diagnostic modalities

De Luca,

Campochiaro,

Palmisano

et al. 2023

Rheumatology

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Objectives Myocarditis is an overlooked manifestation of anti-synthetase syndrome (ASS). Our study describes the clinical and instrumental features of ASS-myocarditis and evaluates the diagnostic performance of cardiac magnetic resonance (CMR) with mapping techniques. Methods Data from ASS-patients were retrospectively analyzed. CMR data of patients diagnosed with myocarditis, including late gadolinium enhancement (LGE), T2-ratio, T1-mapping, extra-cellular volume (ECV) and T2-mapping, were reviewed. Myocarditis was defined by the presence of symptoms of heart involvement with increased high-sensitive troponin T (hs-TnT) and/or NT-proBNP and at least an instrumental abnormality. Clinical features of ASS patients with and without myocarditis were compared. A p value<0.05 was considered. Results Among a cohort of 43 ASS-patients (median age 58[48.0-66.0] years; females 74.4%; anti-Jo1 53.5%), 13(30%) were diagnosed with myocarditis. In 54% of patients, myocarditis was diagnosed at clinical onset. All ASS-myocarditis patients had at least one CMR abnormality: increased ECV in all cases, presence of LGE, increased T1 and T2-mapping in 91%. The 2009-Lake Louis criteria (LLC) were satisfied by 6 patients, the 2018-LLC by 10. With the updated LLC, the sensitivity for myocarditis improved from 54.6% to 91.0%. ASS-patients with myocarditis were more frequently males(53% vs 13%;p=0.009) with fever(69% vs 17%;p=0.001), and had higher hs-TnT (88.0[23.55–311.5] vs 9.80[5.0–23.0]ng/L; p < 0.001), NT-proBNP(525.5[243.5–1575.25] vs 59.0[32.0–165.5;p=0.013]pg/ml;p=0.013) and C-reactive protein(CRP)(7.0[1.7–15.75] vs 1.85[0.5–2.86]mg/L;p=0.011) compared to those without myocarditis. Conclusion In ASS, myocarditis is frequent, even at clinical onset. ASS-patients with myocarditis frequently presented with fever and increased CRP, suggesting the existence of an inflammatory phenotype. The use of novel CMR mapping techniques may increase the diagnostic sensitivity for myocarditis in ASS.

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Section: Discussionmentioning

confidence: 60%

Myocarditis in anti-synthetase syndrome: clinical features and diagnostic modalities

De Luca,

Campochiaro,

Palmisano

et al. 2023

Rheumatology

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“…A systematic literature review and case series demonstrated a beneficial role of IL-1 inhibition with anakinra in seven patients with difficult to treat manifestations of ASyS, despite the use of other immunosuppressive agents [134]. Similarly, IL-6 blockade with tocilizumab in two patients with treatment-refractory ASyS-ILD showed an improvement in and stabilization of lung function over time [135].…”

Section: Other Biological Agentsmentioning

confidence: 98%

A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease

Patel,

Marinock,

Ajmeri

et al. 2024

IJMS

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Our objective in this review article is to present a clinical case of a patient with antisynthetase syndrome (ASyS) and provide an overview of the pathogenesis, classification criteria, antibody profiles, clinical features, and current knowledge of treatment options, focusing on interstitial lung disease (ILD). ASyS is an uncommon autoimmune disease with a heterogenous clinical presentation characterized by the presence of autoantibodies against an aminoacyl-tRNA synthetase and manifested by myositis, fever, inflammatory arthritis, Raynaud’s phenomenon, mechanics hands, and ILD. ASyS-associated ILD (ASyS-ILD) is the most serious complication of ASyS, which may evolve to rapidly progressive ILD; therefore, it often requires thorough clinical and radiologic evaluation including recognition of a specific clinical phenotype associated with the antisynthetase antibodies (ASAbs) to guide therapeutic interventions.

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Where are we now in biologic drugs for myositis?

Neves,

Viveiros,

Venturelli

et al. 2024

Rheumatology

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Idiopathic inflammatory myopathies (IIM) are a rare and heterogeneous group of chronic autoimmune disorders. Up to 40% of IIM patients have long-term sequelae and significant functional disability. Its management can be challenging. New therapies are badly needed. The small number of cases with diverse presentations, and different diagnostic criteria interfere significantly with clinical trial results. Only intravenous immunoglobulin has been internationally approved for IIM patients. Most clinical trials of new biological therapies have failed to meet their primary endpoints in IIM, with only one biological drug recommended for refractory IIM treatment (rituximab), although not approved. We review several new emerging biological drugs including B cell depletion therapies, abatacept, janus-kinase inhibitors, and aldesleukin. Encouragingly, some phase II randomized controlled trials have evaluated the efficacy and safety of new biologics in IIM, demonstrating an improvement in clinical and laboratory measures.

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Anakinra for the Treatment of Antisynthetase Syndrome: A Monocentric Case Series and a Systematic Literature Review

Abstract: Antisynthetase syndrome (AS) is a rare autoimmune disorder whose treatment remains a challenge.1 In refractory cases, treatment escalation from glucocorticoids (GCs) and immunosuppressants to rituximab (RTX) is recommended.1

Cited by 4 publications

References 10 publications

Myocarditis in anti-synthetase syndrome: clinical features and diagnostic modalities

Myocarditis in anti-synthetase syndrome: clinical features and diagnostic modalities

A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease

Where are we now in biologic drugs for myositis?

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