Otitis Media and Relapsing Polychondritis

BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) (Still's disease) associated lung disease (sJIA-LD) is a rare but severe complication, characterized by pulmonary involvement with high morbidity and mortality rates. While the incidence of sJIA-LD has increased, its pathogenesis and risk factors remain poorly…

Objective: To investigate the ocular anterior segment parameters in pediatric Behçet's disease (BD) without ocular involvement. Methods: ), simulated keratometry -flat (Kflat), steep (Ksteep), and average (Kavg)-, and front and back elevation values (Elf, Elb) values measured with a corneal topography device were exam…

BACKGROUND: Pulmonary vascular involvement represents one of the most severe manifestations of Behçet's disease. Pulmonary artery aneurysm (PAA) and pulmonary artery thrombosis (PAT) are rare but potentially life-threatening complications. However, data regarding their frequency, coexistence, and clinical course remai…

Pulmonary nodules are common in ANCA-associated vasculitis (AAV), but their clinical relevance remains unclear. This study assessed the influence of pulmonary nodules and radiological features on clinical characteristics and outcomes in AAV. In this multicenter retrospective cohort, clinical features and outcomes were…

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Objectives: The objective of this study was to evaluate and update the evidence on pharmacologic and interventional treatments for major organ involvement in Behçet syndrome (BS), in order to inform the 2025 update of the European Alliance of Associations for Rheumatology recommendations. Methods: A systematic literat…

We report the case of a 30-year-old female patient with Crohn's disease who was receiving infliximab and mesalazine therapy. She presented with left ear pain accompanied by bloody diarrhea and abdominal pain. Initial treatment with ciprofloxacin and aluminum acetate was ineffective, requiring hospitalization. She was…

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Lymphoproliferative disorders (LPDs) associated with inborn errors of immunity (IEI) are rare entities and their genetic basis is not well defined. We performed targeted deep sequencing using a panel of 529 genes to investigate the single nucleotide variants (SNVs), insertion/deletions (INDELs), structural rearrangeme…

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Vogt-Koyanagi-Harada (VKH) is a multisystem autoimmune disorder affecting the eyes, central nervous system, ears, and skin. VKH accounts for approximately 0.4% to 1.7% of paediatric uveitis cases. This case series aims to describe seven paediatric VKH cases with markedly different courses, with the oldest presenting a…

Type I interferonopathies are a heterogeneous group of monogenic autoinflammatory disorders characterized by dysregulated type I interferon (IFN-I) signaling due to pathogenic variants that affect nucleic acid sensing, processing, or downstream signaling pathways. Mutations in genes including TREX1, RNASEH2A/B/C, SAMH…