Pos0957 epidemiology of Neurosarcoidosis Disease in Northern Spain 1999-2019: A Population-Based Study

Abstract

BackgroundNeurosarcoidosis (NS) is a serious and relative uncommon complication of sarcoidosis [1].Data on incidence is scarce and varies worldwide.ObjectivesTo estimate NS epidemiology in Northern Spain.MethodsPatients diagnosed with sarcoidosis at a University hospital in Northern Spain, between January 1999 and December 2019 were assessed. Sarcoidosis diagnosis was established according to ATS/ERS/WASOG criteria as follows: compatible clinical and radiological presentation, histopathologic confirmation, and exclusion of other granulomatous diseases. NS was diagnosed according to the NS Consortium Consensus Group [2].Demographic and clinical data were collected. The incidence of sarcoidosis between 1999-2019 was estimated by gender, age, and year of diagnosis.ResultsNS was observed in 30 of 234 (12.8%) (19 women/ 11 men) (mean age: 55.0±15.8 years) patients with sarcoidosis. The underlying neurological manifestations were chronic headache (n=13, 43.4%), peripheral neuropathy (n=6, 20.0%), cranial neuropathy (n=5, 16.7%), spinal cord abnormalities (n=3, 10.0%) and aseptic meningitis (n=3, 10.0%).A comparison between different geographical areas is summarized inTable 1. There are wide variations in frequency (US:4.8% to France:33.9%), gender predominance and age at diagnosis (31 to 55 years) depending on the geographical area. Nevertheless, most of the patients were diagnosed in the 5thdecade of life.Annual incidence of NS in our population area in the 1999-2019 period was 0.11 per 100,000 people, 95% (CI:0.11-0.26); 0.08 (0.07-0.24) in men, 0.13 (0.09-0.24) in women. There were variations in annual incidences, ranging from a minimum value of 0.08 in 2013-2014 to a maximum of 0.19/100,000 people in 1999-2000. A downward trend in annual incidence over time was observed. Nevertheless, the correlation was weak (r2=0.1135) (Figure 1).ConclusionThe epidemiological characteristics of NS is very different in frequency. Frequency estimated in this study was similar to that of other countries.References[1] Riancho-Zarrabeitia L, et al. Clin Exp Rheumatol 2014; 32:275-84. PMID:24321604.[2] Stern BJ, et al. JAMA Neurol. 2018;75:1546-1553.Table 1.Main clinical features and treatment of neurosarcoidosis in different geographical areasAuthor, yearCountrycasesMale n (%)Age at onset years mean ± SDSNS%SNSSNSGascón-Bayarri et. al., 2011Spain445306.7ND10 (33.4)ND48.3±NDLeonhard et. al, 2016The NetherlandsND52NDND22 (48.0)44±ND43.0±NDJoubert et. al, 2017France69023433.9ND117 (50.0)ND31.5±NDDorman et. al, 2019USA1706824.8691 (40.6)43 (52.4)49±10.845.0±11.4Arun et. al, 2020UKND80NDND35 (44.0)ND47.8±NDGoel et al., 2020IndiaND12NDND4 (33.4)ND44.0± 9.2Sambon et. al, 2022Belgium1802212.2ND14 (64.0)ND40.5±NDByg et al., 2022DenmarkND20NDND11 (55.0)ND51.6±NDPresent study, 2023Spain2343012.8105 (44.9)11 (36.7)48.4±14.855.0±15.8Abbreviations:ND: non data, NS: neurosarcoidosis, S: SarcoidosisFigure 1.Trends in age at neurosarcoidosis diagnosis in Cantabria, Spain, in 1999-2019 by genderAcknowledgements:NIL.Disclosure of InterestsAlba Herrero-Morant: None declared, Lara Sanchez-Bilbao: None declared, Iñigo Gonzalez-Mazon: None declared, David Martínez-López: None declared, Carmen Álvarez-Reguera: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Lilly, Janssen, UCB, and Celgene, Raúl Fernández-Ramón: None declared, Ricardo Blanco Speakers bureau: Abbvie, Pfizer, Roche, Lilly, Bristol-Myers, Janssen, Galapagos and MSD, Consultant of: Abbvie, Pfizer, Roche, Lilly, Bristol-Myers, Janssen and MSD, Grant/research support from: Abbvie, MSD, Novartis and Roche.