Citation

Hammett EK, Fernandez-Carbonell C, Crayne C, Boneparth A, Cron RQ, Radhakrishna SM. Pediatr Rheumatol Online J 2020; 18(1): e15.

Copyright

(Copyright © 2020, Holtzbrinck Springer Nature Publishing Group - BMC)

DOI

10.1186/s12969-020-0412-8

PMID

32046763

PMCID

PMC7014743

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.

Language: en

Keywords

Humans; Female; Adolescent; Young Adult; Treatment Outcome; Suicidal Ideation; Psychosis; Psychotic Disorders; Antipsychotic Agents; Glucocorticoids; Rituximab; Antibodies, Monoclonal, Humanized; Antirheumatic Agents; Central nervous system (CNS) Sjogren’s syndrome; Pediatric Sjogren’s syndrome; Sjogren's Syndrome