Long Term Outcome of Neurosarcoidosis

Catherine Chapelon¹, Bastien Joubert¹, Lucie Biard², David Saadoun³, Mathieu Resche Regon⁴, Fanny Domont¹, Lea Savey¹, Didier Dormont¹ and Patrice Cacoub⁵, ¹Hôpital Pitié Salpétrière, AP-HP, UPMC, Univ Paris 06, Paris, France, ²Biostatistics, Saint-Louis Hospital, Paris, France, ³Department of Internal Medicine and clinical Immunology. French National Reference Center for Autoimmune Diseases. DHU I2B (Inflammation, Immunotherapy and Biotherapy), UPMC, Paris VI, Hôpital Pitié Salpétrière, AP-HP, UPMC, Univ Paris 06, Paris, France, ⁴biostatistics Saint Louis Hospital, paris, France, ⁵Internal Medicine Department, University Hospital “Pitié-Salpêtrière”, “Pierre et Marie Curie Paris VI” University, Paris, France

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Neurosarcoidosis and outcomes

Session Information

Date: Monday, November 9, 2015

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster Session II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Although clinical and imaging features of neurosarcoidois (NS) have been described, few studies have reported on the long-term outcome. We report the long-term outcome of a large series of NS, focusing on overall and relapse-free survival and dependency status.

Methods: Retrospective monocenter analysis of NS patients fulfilling international criteria for NS. All NS patients had an evaluation including physical examination, dependence scoring by Expanded Disability Status Scale (EDSS), cerebral spinal fluid (CSF) analysis, brain and/or spine MRI; and for thirty-four cases a pathological study of nerve/muscle tissue. Endpoints were rates of and factors associated with: (i) overall survival after NS diagnosis (OS), (ii) relapse-free survival after NS diagnosis (RFS), and (iii) dependence at the end of the follow-up (FU), as assessed by EDSS ≥2.5. The RFS was calculated using Kaplan-Meier estimator and a multivariate Cox proportional hazard ratio model was selected to assess prognostic factors of RFS.

Results:

Among a cohort of 690 sarcoidosis patients, 242 patients with NS were identified i.e. definite NS in 34 patients (14%), probable NS in 149 (62%) and possible in 59 (24%). Median age at NS diagnosis was 41 years (IQR 30; 52), 50% were female and 53% Caucasian. Multivisceral sarcoidosis was noted in 214/242 of NS (88%) including cardiac involvement in 85 patients. Symptoms of NS preceded extra-neurological involvement in 55/242 patients (23%), while neurological symptoms were concomitant or followed extra-neurological involvement in 135/242 (56%) and 52/242 (21%) patients, respectively.

Main neurological involvements of NS were central nervous system in 184/242 patients (76%) including spinal cord in 63 patients, cranial nerve in 85/242 (35%), and peripheral nerve and/or muscle in 42/242 (17%). Overall, 148/242 patients (61%) had more than one neurological involvement. Analysis of CSF showed unspecific abnormalities in 90/242 patients (37%). The initial EDSS score was ≥2.5 in 159/242 patients (36%). Steroids were given to 224/242 patients (93%) associated to i.v. cyclophosphamide in 90/242 (37%), methotrexate in 97/242 (40%), mycophenolate mofetil in 57/242 (24%) and infliximab in 23/242 (10%).

After a median FU of 96 months, 22/242 patients (9%) died including 6 patients with no NS relapse. The OS rates at 1, 5, and 10 years were 98.8%, 95.4%, and 91.3%, respectively. At the end of FU, 25% of patients had an EDSS ≥2.5. Factors independently associated with dependence were CSF protein ≥1g/l and presence of a CNS lesion at MRI (p<0.05). The RFS rates at 1, 5 and 10 years were 69.9%, 38.7% and 30.7%, respectively. Factors independently associated with poor RFS were older age at NS diagnosis, neurological signs preceding multivisceral sarcoidosis diagnosis and CSF protein ≥1g/L (p<0.01). There was no impact of international criteria on OS and RFS.

Conclusion: Neurosarcoidosis is a severe condition with low relapse-free survival rates. However, the overall survival rate is > 90% at 10 years. International criteria of NS showed no impact on overall or relapse-free survival rates.

Disclosure: C. Chapelon, None; B. Joubert, None; L. Biard, None; D. Saadoun, None; M. Resche Regon, None; F. Domont, None; L. Savey, None; D. Dormont, None; P. Cacoub, None.

To cite this abstract in AMA style:

Chapelon C, Joubert B, Biard L, Saadoun D, Resche Regon M, Domont F, Savey L, Dormont D, Cacoub P. Long Term Outcome of Neurosarcoidosis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/long-term-outcome-of-neurosarcoidosis/. Accessed .

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