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THU0397 Clinical Presentation and Characterization of Neurosarcoidosis: Analysis in 719 Patients (Sarcogeas-Semi Registry)
  1. P. Brito Zeron1,
  2. J. Mañá2,
  3. R. Pérez-Alvarez3,
  4. S. Retamozo1,
  5. H. Gheitasi1,
  6. J. Fernández-Martín3,
  7. M. Pérez-de-Lis3,
  8. X. Bosch4,
  9. M. Ramos-Casals1
  1. 1Josep Font Autoimmune Diseases Laboratory, Department of Autoimmune Diseases, Hospital Clinic, IDIBAPS
  2. 2Department of Internal Medicine, Hospital de Bellvitge, Barcelona
  3. 3Department of Internal Medicine, Hospital do Meixoeiro, Vigo
  4. 4Department of Internal Medicine, ICMiD, Hospital Clinic, Barcelona, Spain

Abstract

Objectives To analyze the main epidemiological and clinical characteristics of patients with neurological involvement related to sarcoidosis.

Methods In May 2013, the Study Group on Autoimmune Diseases (GEAS-SEMI) created the national registry of patients with sarcoidosis (SARCOGEAS-SEMI Registry). Patients presenting with neurological features were specifically evaluated.

Results In December 2013, a total of 719 patients with sarcoidosis were included in the SARCOGEAS-SEMI Registry; 52 (7.2%) had neurosarcoidosis, 22 men and 30 women, with a mean age at diagnosis of neurosarcoidosis of 50.1 years (range 23-84 years). The main neurological involvement consisted of cranial neuropathies in 27 (52%) patients, followed by CNS involvement in 23 (44%), peripheral neuropathies in 6 (10%) and muscular involvement in 4 (8%). The most frequent cranial neuropathies included involvement of VII pair (17 cases) followed by VIII pair (5 cases) and V and VI pairs (4 cases each); other cranial nerves affected included IX (n=3), III (n=3), XI (n=2), I (n=1), II (n=1) and XII (n=1); 12/27 (44%) patients showed multiple cranial neuropathies. With respect to CNS involvement, meningeal involvement (15%) and vascular/demyelinating lesions (10%) were the most frequent involvements; other features consisted of parenchymatous cerebral lesions (n=4), encephalopathy (n=4), hypothalamic involvement (n=3), seizures (n=2) and hydrocephalia (n=1). All patients received corticosteroids (10 received methylprednisolone pulses) and 9 immunosuppressants (methotrexate in 5, azathioprine in 4, cyclophosphamide in 2). One patient refractory to standard therapy was treated with infliximab. Nearly half the cases showed improvement or stabilization (46%), 34% showed complete resolution, 16% worsened and 4% died.

Conclusions Nearly 10% of patients with sarcoidosis present with neurological involvement. The main involvement of neurosarcoidosis consisted of cranial neuropathies in half the cases. Twenty percent of patients showed a worse outcome, with a mortality rate of 4%. Neurological involvement is a significant cause of morbidity and mortality in patients with sarcoidosis.

Disclosure of Interest : None declared

DOI 10.1136/annrheumdis-2014-eular.5374

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