PO15 - Real-world neurosarcoidosis: clinical phenotypes, management, and outcomes in a single-center cohort
Martina Ceraudo (Italy)1; Giulia Zinni (Italy)1; Alessandro Dell'Edera (Italy)1; Riccardo Scarpa (Italy)1; Marcello Rattazzi (Italy)1; Anna Palmieri (Italy)2; Francesco Cinetto (Italy)1;
1 - Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca' Foncello Hospital, University of Padova, Padova, Italy.; 2 - 4: Neurology Unit, AULSS2 Marca Trevigiana, Ca' Foncello Hospital, Treviso, Italy.;
Keywords: Neurosarcoidosis; Phenotypes; Biologics;
Select the theme: Special Topics in Extrathoracic Sarcoidosis
Type: Original Papers
Presentation: Poster Presentation

Introduction: Neurosarcoidosis (NS) is a rare and heterogeneous manifestation of sarcoidosis, with diagnostic and therapeutic challenges.

Objectives: To describe clinical features, treatment and outcomes of a cohort of patients with neurosarcoidosis.

Materials and Methods: Single-centre retrospective study on patients with neurosarcoidosis followed at our multidisciplinary clinic between 2009 and 2026 (minimum follow-up of 12 months). Continuous variables are reported as mean ± SD or median (IQR), categorical variables as n (%).

Results: 57 NS (42 men, 15 women) were included from a cohort of 387 sarcoidosis. Median age at onset was 46.2±12.8 and at diagnosis 48.8±12.2 years (median diagnostic delay 4 months, IQR 11, 0-350). No sex differences were observed. According to the JAMA2018 criteria, 16 patients (28.1%) were classified as possible, 37 (64.9%) as probable, and 4 (7%) as definite.

63.2% patients presented CNS involvement at onset, mainly with focal lesions (41.7%), leptomeningitis (36.1%) and myelitis/myelopathy (30.6%). During follow-up, pulmonary (80.7%) and lymph node involvement (70.2% hilar-mediastinal; 57.9% extrathoracic) were most frequent, followed by skin (24.6%) and ocular (22.8%). Most patients received corticosteroids (96.5%), 15.8% as monotherapy. For steroid-sparing or refractory cases, 43 patients (75.4%) received at least one DMARD, 31.6% more than one. Methotrexate was the most commonly used (32 patients). Biologic therapy was administered to 27 (47.4%), generally after previous treatment failure, with only 1 patient as first-line therapy. Infliximab was the predominant biologics (23 patients, 85%), while adalimumab was used in selected cases.

Mortality was significantly higher in neurosarcoidosis compared with sarcoidosis alone (8.8% vs 1.5%; OR 6.25, 95% CI 1.75–22.34; p=0.008).

Conclusions: Neurosarcoidosis is a heterogeneous condition with complex management, often requiring immunosuppressive/biologic therapy. Our findings reveal that patients with neurological involvement are at significantly higher risk of mortality than those with systemic sarcoidosis alone. Early diagnosis and specialist care are crucial to optimize outcomes.