Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.
ADVANCED SEARCH
Log in
Top
Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2020

Open Access 01-12-2020 | Obsessive-Compulsive Disorder | Case Report

Adolescent Sjogren’s syndrome presenting as psychosis: a case series

Authors: Erin K. Hammett, Cristina Fernandez-Carbonell, Courtney Crayne, Alexis Boneparth, Randy Q. Cron, Suhas M. Radhakrishna

Published in: Pediatric Rheumatology | Issue 1/2020

Login to get access

Abstract

Background

Neurological involvement has been reported in up to 80% of adults with Primary Sjogren’s syndrome (pSS) with psychiatric abnormalities including anxiety, depression, and cognitive dysfunction being common. Psychosis due to pSS has been reported in adult patients but has never been previously reported in the adolescent/pediatric literature. Here we describe for the first time four cases of adolescent Sjogren’s syndrome that presented with psychotic symptoms. Rituximab treatment was followed by improvement of psychiatric symptoms in all patients.

Case presentation

1: 16 year old female without significant past medical history presented to the emergency department with 4 days of abnormal behavior, tremors, insomnia, polyphagia, polyuria, and suicidal ideation.
2: 16 year old female with a 4 year history of severe anxiety, OCD, and tic disorder treated with fluoxetine with partial benefit presented with an abrupt and severe worsening of anxiety, OCD and new auditory hallucinations.
3: 19 year old female without significant past medical history presented with a 3 day history of progressively altered behavior, incoherent speech, insomnia, headache, and tangential thoughts.
4: 17 year old female without significant past medical history presented with new onset suicidal ideation, paranoia, confusion, and emotional lability.

Conclusion

Psychosis is more common in autoimmune disease than previously known. To our knowledge, the four teenage women described above are the first reported patients with adolescent pSS manifesting as psychosis. pSS should be considered in the differential diagnosis of young patients with new psychiatric disorders, even in the absence of sicca symptoms. Psychiatric symptoms improved with rituximab infusions in all 4 of our patients, which suggests rituximab may be an effective treatment option that should be considered early after the diagnosis of pSS-associated psychiatric disturbance.
Literature
1.
Virdee S, Greenan-Barrett J, Ciurtin C. A systematic review of primary Sjögren's syndrome in male and paediatric populations. Clin Rheumatol. 2017;36(10):2225–36.CrossRef
2.
Tucker L. Sjogren’s Syndrome. In: Petty RE, Laxer RM, Lindsley CB, Wedderburn L, editors. Textbook of pediatric rheumatology. 7th ed: Philadelphia: Saunders; 2016. p. 427–35.
3.
Cox PD, Hales RE. CNS Sjögren's syndrome: an underrecognized and underappreciated neuropsychiatric disorder. J Neuropsychiatry Clin Neurosci. 1999;11(2):241–7.CrossRef
4.
Mauch E, Völk C, Kratzsch G, Krapf H, Kornhuber HH, Laufen H, et al. Neurological and neuropsychiatric dysfunction in primary Sjögren's syndrome. Acta Neurol Scand. 1994;89(1):31–5.CrossRef
5.
Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004;83(5):280–91.CrossRef
6.
Massara A, Bonazza S, Castellino G, Caniatti L, Trotta F, Borrelli M, et al. Central nervous system involvement in Sjögren's syndrome: unusual, but not unremarkable--clinical, serological characteristics and outcomes in a large cohort of Italian patients. Rheumatology (Oxford). 2010;49(8):1540–9.CrossRef
7.
Ampelas JF, Wattiaux MJ, VanAmarongen AP. Troubles psychiatriques du lupus erythmateux dissemine et du syndrome de Gougerot-Sjogren. L’ Encephale. 2001;27(6):588–99.
8.
Lin CE. One patient with Sjogren's syndrome presenting schizophrenia-like symptoms. Neuropsychiatr Dis Treat. 2016;12:661–3.CrossRef
9.
Rosado SN, Silveira V, Reis AI, Gordinho A, Noronha C. Catatonia and psychosis as manifestations of primary Sjögren's syndrome. Eur J Case Rep Intern Med. 2018;5(6):000855.PubMedPubMedCentral
10.
Hietaharju A, Yli-Kerttula U, Häkkinen V, Frey H. Nervous system manifestations in Sjögren's syndrome. Acta Neurol Scand. 1990;81(2):144–52.CrossRef
11.
Spezialetti R, Bluestein HG, Peter JB, Alexander EL. Neuropsychiatric disease in Sjögren's syndrome: anti-ribosomal P and anti-neuronal antibodies. Am J Med. 1993;95(2):153–60.CrossRef
12.
Kang JH, Lin HC. Comorbidities in patients with primary Sjogren's syndrome: a registry-based case-control study. J Rheumatol. 2010;37(6):1188–94.CrossRef
13.
Ong LTC, Galambos G, Brown DA. Primary Sjogren's syndrome associated with treatment-resistant obsessive-compulsive disorder. Front Psychiatry. 2017;8:124.CrossRef
14.
Alexander EL, Beall SS, Gordon B, Selnes OA, Yannakakis GD, Patronas N, et al. Magnetic resonance imaging of cerebral lesions in patients with the Sjögren syndrome. Ann Intern Med. 1988;108(6):815–23.CrossRef
15.
Alexander EL. Neurologic disease in Sjögren's syndrome: mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. A clinical review and update of immunopathogenesis. Rheum Dis Clin N Am. 1993;19(4):869–908.
16.
Moll JW, Markusse HM, Pijnenburg JJ, Vecht CJ, Henzen-Logmans SC. Antineuronal antibodies in patients with neurologic complications of primary Sjögren's syndrome. Neurology. 1993;43(12):2574–81.CrossRef
17.
Alexander EL, Lijewski JE, Jerdan MS, Alexander GE. Evidence of an immunopathogenic basis for central nervous system disease in primary Sjögren's syndrome. Arthritis Rheum. 1986;29(10):1223–31.CrossRef
18.
Alexander E. Central nervous system disease in Sjögren's syndrome. New insights into immunopathogenesis. Rheum Dis Clin N Am. 1992;18(3):637–72.
19.
Ferreiro JE, Robalino BD, Saldana MJ. Primary Sjögren's syndrome with diffuse cerebral vasculitis and lymphocytic interstitial pneumonitis. Am J Med. 1987;82(6):1227–32.CrossRef
20.
Sanders ME, Alexander EL, Koski CL, Frank MM, Joiner KA. Detection of activated terminal complement (C5b-9) in cerebrospinal fluid from patients with central nervous system involvement of primary Sjogren's syndrome or systemic lupus erythematosus. J Immunol. 1987;138(7):2095–9.PubMed
21.
Alexander EL, Ranzenbach MR, Kumar AJ, Kozachuk WE, Rosenbaum AE, Patronas N, et al. Anti-Ro (SS-A) autoantibodies in central nervous system disease associated with Sjögren's syndrome (CNS-SS): clinical, neuroimaging, and angiographic correlates. Neurology. 1994;44(5):899–908.CrossRef
22.
Jacob CO, Plitt JR, Fronek Z. HLA class II associated tumor necrosis factor alpha synthesis in central nervous system disease in Sjogren’s syndrome. Arthritis Rheum. 1992;35:S121.
23.
Vivino FB, Carsons SE, Foulks G, Daniels TE, Parke A, Brennan MT, et al. New treatment guidelines for Sjögren's disease. Rheum Dis Clin N Am. 2016;42(3):531–51.CrossRef
24.
Mekinian A, Ravaud P, Larroche C, Hachulla E, Gombert B, Blanchard-Delaunay C, et al. Rituximab in central nervous system manifestations of patients with primary Sjögren's syndrome: results from the AIR registry. Clin Exp Rheumatol. 2012;30(2):208–12.PubMed
25.
Tokunaga M, Saito K, Kawabata D, Imura Y, Fujii T, Nakayamada S, et al. Efficacy of rituximab (anti-CD20) for refractory systemic lupus erythematosus involving the central nervous system. Ann Rheum Dis. 2007;66(4):470–5.CrossRef
26.
Shiboski CH, Shiboski SC, et al. 2016 American College of Rheumatology/European league against rheumatism classification criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis and Rheumatology. 2016;69(1):35–45.CrossRef
27.
Arciniegas DB. Psychosis. Continuum (Minneapolis, Minn). 2015; 21(3 Behavioral neurology and neuropsychiatry): 713–736.
28.
Keshavan MS, Kaneko Y. Secondary psychoses: an update. World Psychiatry. 2013;12(1):4–15.CrossRef
29.
Chen SJ, Chao YL, Chen CY, Chang CM, Wu EC, Wu CS, et al. Prevalence of autoimmune diseases in in-patients with schizophrenia: nationwide population-based study. Br J Psychiatry. 2012;200(5):374–80.CrossRef
30.
Eaton WW, Byrne M, Ewald H, Mors O, Chen CY, Agerbo E, et al. Association of schizophrenia and autoimmune diseases: linkage of Danish national registers. Am J Psychiatry. 2006;163(3):521–8.CrossRef
31.
Sibbitt WL, Brandt JR, Johnson CR, Maldonado ME, Patel SR, Ford CC, et al. The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus. J Rheumatol. 2002;29(7):1536–42.PubMed
32.
Pelizza L, Bonacini F, Ferrari A. Psychiatric disorder as clinical presentation of primary Sjögren's syndrome: two case reports. Ann General Psychiatry. 2010;9:12.CrossRef
33.
Malinow KL, Molina R, Gordon B, Selnes OA, Provost TT, Alexander EL. Neuropsychiatric dysfunction in primary Sjögren's syndrome. Ann Intern Med. 1985;103(3):344–50.CrossRef
34.
Shen CC, Yang AC, Kuo BI, Tsai SJ. Risk of psychiatric disorders following primary Sjögren syndrome: a Nationwide population-based retrospective cohort study. J Rheumatol. 2015;42(7):1203–8.CrossRef
35.
Soliotis FC, Mavragani CP, Moutsopoulos HM. Central nervous system involvement in Sjogren's syndrome. Ann Rheum Dis. 2004;63(6):616–20.CrossRef
36.
Lieberman SM. Childhood Sjogren syndrome: insights from adults and animal models. Curr Opin Rheumatol. 2013;25(5):651–7.CrossRef
Metadata
Title
Adolescent Sjogren’s syndrome presenting as psychosis: a case series
Authors
Erin K. Hammett
Cristina Fernandez-Carbonell
Courtney Crayne
Alexis Boneparth
Randy Q. Cron
Suhas M. Radhakrishna
Publication date
01-12-2020
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2020
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/s12969-020-0412-8

Other articles of this Issue 1/2020

Pediatric Rheumatology 1/2020 Go to the issue

Research article

The association of MEFV gene mutations with the disease risk and severity of systemic juvenile idiopathic arthritis

Case Report

Atrial thrombus as a complication of SLE and APS in an 8-year-old child

Research article

The use of S100 proteins testing in juvenile idiopathic arthritis and autoinflammatory diseases in a pediatric clinical setting: a retrospective analysis

Research article

Assessment and treatment of Down syndrome-associated arthritis: a survey of pediatric rheumatologists

Research article

Prosthetic temporomandibular joint reconstruction in a cohort of adolescent females with juvenile idiopathic arthritis

Research article

Effect of different doses of aspirin on the prognosis of Kawasaki disease