Neuropsychiatric lupus
Abstract
The diagnosis of neuropsychiatric lupus is based on the ACR 1999 nomenclature and case description. However, nonspecific clinical syndromes and diagnostic methods without specific patterns determine a large discrepancy in the reported frequency (14%-75%). A longitudinal, descriptive, observational design was applied based on the evaluation of clinical histories, SLE damage and activity indexes using the SLICC/ACR-DI and SLEDAI-2K, respectively, functional disability using the HAQ and self-reported work disability at 30 days. The number of deaths at one month of follow-up was also recorded. Serum IL-6 was measured at the time of diagnosis, as established by How criteria. Ten cases were described. Four patients debuted with neuropsychiatric involvement. The most frequent syndrome was major depression (n=4), followed by psychosis, seizures, peripheral neuropathy (n=3), stroke and myelitis (n=1). Half the patients (n=5) presented more than one neuropsychiatric diagnosis. The average SLE activity score measured by SLEDAI-2K was 16.5. The average chronic damage score measured by SLICC/ACR-DI was 3. Half the patients scored 2 on the HAQ (n=5) and self-reported work disability at 30-day follow-up was observed in 40% of patients. IL-6 was not detected in plasma, except in one patient with diffuse and focal involvement (value:1.16 pg/ml, reference range in healthy adults: 0-5 pg/ml). No deaths were recorded in the 30-day follow-up after hospital discharge in the study population. Despite having a small number of patients, this case series represents the first in-depth study of patients with SLE and neuropsychiatric involvement. Collaborative studies are needed to include more patients to reliably assess clinical features and possible biomarkers.
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(1) Jeltsh-David, H., and Muller S. Neuropsychiatric systemic lupus erythematosus: pathogenesis and biomarkers. Nature Reviews Neurology. 2014(10),579–596.
(2) Rood MJ, Breedveld FC, Huizinga TW. The accuracy of diagnosing neuropsychiatric systemic lupus erythematosus in a series of 49 hospitalized patients. Clin Exp Rheumatol. 1999. 17:55–61.
(3) ACR Ad Hoc Committee on Neuropsychiatric Lupus Nomenclature. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999; 42:599–608.
(4) Brey RL, Holliday SL, Saklad AR, Navarrete MG, Hermosillo-Romo D, Stallworth CL et al. Neuropsychiatric syndromes in lupus: pre- valence using standardized definitions. Neurology. 2002. 58:1214– 1220.
(5) Ainiala H, Loukkola J, Peltola J et al. The prevalence of neuro- psychiatric syndromes in systemic lupus erythematosus. Neurology. 2001. 57:496–500.
(6) Sanna G, Bertolaccini ML, Cuadrado MJ, Laing H, Khamashta MA, Mathieu A, et al. Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. J Rheumatol. 2003 a; 30 (5): 985-92.
(7) Hanly JG, McCurdy G, Fougere L, Douglas JA, Thompson K. Neuro- psychiatric events in systemic lupus erythematosus: attribution and clinical significance. J Rheumatol 2004; 31 (11): 2156-62.
(8) Jarpa E, Babul M, Calderón J, González M, Martínez M, Bravo- Zehnder M, et al. Common mental disorders and psychological distress in systemic lupus erythematosus are not associated with disease activity. Lupus 2011; 20 (1): 58-66.
(9) León T, Henríquez C, Calderón J, Massardo L. Actualización en Lupus Neuropsiquiátrico con énfasis en déficit cognitivo. Rev Med Chile 2012; 140: 1333-41.
(10) Vasudevan A, Krishnamurthy AN. Changing worldwide epidemiology of systemic lupus erythematosus. Rheumatic Disease Clin North Am 2010; 36 (1): 1-13.
(11) Petri M, Orbai AM, Alarcón GS, Gordon C, Merril JT, Fortin PR, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012; 64: 2677-86.
(12) Hanly JG, McCurdy G, Fougere L, Douglas JA, Thompson K. Neuro- psychiatric events in systemic lupus erythematosus: attribution and clinical significance. J Rheumatol 2004; 31 (11): 2156-62.
(13) Bortoluzzi A, Scirè CA, Govoni M. Attribution of neuropsychiatric manifestations to systemic lupus erythematosus. Front. Med. 2018, 5, 68.
(14) Bendorius M, Po C, Muller S, and Jantsch-David H. From Systemic Inflammation to Neuroinflammation: The Case of Neurolupus. Int. J. Mol. Sci. 2018,19, 3588.
(15) Hanly JG. Diagnosis and management of neuropsychiatric SLE. Nat. Rev. Rheumatol. 2014, 10, 338–347.
(16) Bertsias GK, LIoannidis JP, Aringer M, Bollen E, Bombardieri S, Bruce IN et al. EULAR recommendations for the management of Systemic Lupus Erythematosus with neuropsychiatric mani- festations: report of a task force of the EULAR standing committee for clinical affairs. Ann Rheum Dis. 2010; 69: 20172-82.
(17) Appenzeller S, Costallat LTL, and Cendes F. Neurolupus. Archives of Neurology. 2006;63(3).458.
(18) Monahan RC Beaart-van de Voorde LJJ, Steup-Beekman GM, Magro-Checa C, Huizinga TWJ, Hoekman J, et al. Neuropsychiatric symptoms in systemic lupus erythematosus: Impact on quality of life. Lupus 2017, 26, 1252–1259.
(19) Magro-Checa C Beaart-van de Voorde LJJ, Middelkoop HAM, Dane ML, van der Wee NJ van Buchem MA, et al. Outcomes of neuro- psychiatric events in systemic lupus erythematosus based on clinical phenotypes; prospective data from the Leiden NP SLE cohort. Lupus 2017, 26, 543–551.
(20) Zirkzee E, Huizinga T, Bollen E, van Buchem M, Middelkoop H, van der Wee N, et al. Mortality in neuropsychiatric systemic lupus erythematosus (NPSLE). Lupus 2014, 23, 31–38.
(21) Bertsias JPA, Ioannidis J, Boletis S, Bombardiri R, Cervera C, Dostal C, et al. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. Ann Rheum Dis 2008; 67: 195–205.
(22) Traynor AE, Burt RK. Hematopoietic stem cell Transplantation for active systemic lupus erythematosus. Rheumatology (Oxford). 1999; 38:767–772.
(23) Hermosillo-Romo D, Brey RL. Diagnosis and management of patients with neuropsychiatric systemic lupus erythematosus (NPSLE). Best Pract Res Clin Rheumatol. 2002a; 16:229–244. https://doi.org/10.1053/berh.2001.0223
(24) Hermosillo-Romo D, Brey RL. Neuropsychiatric involvement in systemic lupus erythematosus. Curr Rheumatol. 2002b; Rep 4:337– 344.
(25) Appenzeller S, Shoenfeld Y, and Freire de Carvalho J. Neurologic Manifestations of Autoimmune Diseases. Autoimmune Diseases. 2012, Article ID 683212, 2 pages. doi:10.1155/2012/683212
(26) Appenzeller S. Neurologic Manifestations in Autoimmune Diseases. Rev Bras Reumatol. 2016;56(3):189-190.
(27) Weiner SM, and Peter HH. Neuropsychiatric involvement in systemic lupus erythematosus. Part 1: clinical presentation and pathogenesis. Med Klin (Munich). 2002.15;97(12):730-7.
(28) Weiner SM, Otte A, Uhi M, Brink I, Schumacher M, and Peter HH. Neuropsychiatric involvement in systemic lupus erythematosus. Part 2: diagnostic and therapy. Med Klin (Munich). 2003.15;98(2): 79-90.
(29) Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey- Goldman R, et al. 2019 European League Against Rheumatism/ American College of Rheumatology Classification Criteria for Sys- temic Lupus Erythematosus Arthritis Rheumatol. 2019 Sep;71(9): 1400-1412.
(30) How A, Dent PB, Liao Sh-K, Denburg JA. Antineural Antibodies in Neuropsychiatric Systemic Lupus Erythematosus. Arthritis and Rheumatism.1985; 28 (7): 789-95.
(31) Gladman D, Ginzler E, Goldsmith C, Fortin P, Liang M, Urowitz M, et al. The development and initial validation of the systemic lupus international collaborating clinics American College of Rheuma- tology Damage Index for Systemic Lupus Erythematosus. Arthritis Rheum. 1996. 39:363–369.
(32) Gladman DD, Ibáñez D, Urowitz MB. Systemic Lupus Erythematosus Disease Activity Index 2000. J Rheumatol 2002; 29: 288–91.
(33) Esteve‐Vives J, Batlle‐Gualda E, Reig A, et al. Versión Española del Health Assessment Questionnaire (HAQ, por sus siglas en inglés) Traducida para la adaptación del HAQ a la Población Española. www.reuma.com
(34) Ainiala H, Hietaharju A, Loukkola J, Peltola J, Korpela M, Metsänoja R, et al. Validity of the new American College of Rheumatology criteria for neuropsychiatric lupus syndromes: A population-based evaluation. Arthritis Rheum. 2001, 45, 419–423.
(35) Bertsias GK, and Boumpas DT. Pathogenesis, diagnosis, and management of neuropsychiatric SLE manifestations. Nat Rev Rheumatol. 2010;6:358-67.
(36) Pons-Estel BA, Bonfa E, Soriano ER, et al. First Latin American clinical practice guidelines for the treatment of systemic lupus erythematosus: Latin American Group for the Study of Lupus (GLADEL, Grupo Latino Americano de Estudio del Lupus) Pan-American League of Associations of Rheumatology (PANLAR). Ann Rheum Dis 2018; 77: 1549–57.
(37) Díaz-Cortés D, Correa-González N, Díaz MC, Gutiérrez JM, Fernández-Ávila DG. Compromiso del Sistema nervioso central en el lupus eritematoso sistémico. Rev Colomb Reumatol. 2015; 22(1):16-30.
(38) Trysberg E, Carlsten H and Tarkowski A. Intrathecal cytokines in Systemic Lupus Erythematosus with central nervous system involvement. Lupus. 2000; 9: 498-503.
(39) Zigmond A., Snaith, R. The Hospital Anxiety and Depression Scale. Acta Psychiatrica Scandinavica. 1983; 67, 361-370.
(40) N Iikuni 1, H Okamoto, T Yoshio, E Sato, S Kamitsuji, T Iwamoto, et al. Raised monocyte chemotactic protein-1 (MCP-1)/CCL2 in cerebrospinal fluid of patients with neuropsychiatric lupus. Annals of the Rheumatic Diseases. 2006 (65) 2:253–6.
(41) McCune WJ, MacGuire A, Aisen A, Gebarski S. Identification of brain lesions in neuropsychiatric systemic lupus erythematosus by magnetic resonance scanning. Arthritis Rheum. 1988 b; 31:159–166.
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