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Objective: We aimed to investigate the remission rate and disease duration in idiopathic or post-cardiac injury pericarditis and risk factors for disease duration and anti-interleukin-1 (IL-1) agent discontinuation.

Methods: This was a multicenter, longitudinal, observational study including 370 patients (51.4% female).

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Patients with A20 haploinsufficiency (HA20) presenting with central nervous system (CNS) symptoms are rare, and available reports are limited. Here, we describe a patient with HA20, previously followed up as Behçet disease, who presented with CNS symptoms in adulthood. A 38-year-old Japanese male who had been followed up for incomplete Behçet disease at another hospital since 28 years of age presented to our hospital with acute-onset diplopia and persistent hiccups that were severe enough to cause vomiting.

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SOCS1 deficiency-crossroads of autoimmunity and autoinflammation-two case reports.

Front Pediatr

January 2025

Department of Pediatric Oncology, Hematology and Clinical Immunology, Medical Faculty, Center of Child and Adolescent Health, Heinrich-Heine-University and University Hospital, Duesseldorf, Germany.

Suppressors of cytokine signaling (SOCS) proteins play a critical role in regulating immune signaling pathways. Deficiency of SOCS1 leads to various autoimmune pathologies. We present two unrelated patients with distinct clinical manifestations.

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Background: Patients with pericarditis may show elevation of C-reactive protein (CRP) and pericardial effusion at presentation. There are limited data on the prognostic implications of this inflammatory phenotype.

Objectives: Aim of the present study is to evaluate the outcome of the inflammatory phenotype in a cohort of patients with acute pericarditis.

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Acute pericarditis, the predominant pericardial disease, often lacks a clear etiology, with 15-30% of patients experiencing recurrence, rising to 20-50% in those with prior relapses. Autoimmune mechanisms significantly contribute to recurrence, with interleukin-1 identified as a pivotal inflammatory mediator. While NSAIDs, colchicine, and steroids remain staples for acute cases, the spotlight in recurrent pericarditis management has shifted toward immunosuppressive medications.

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