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

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.

Distal Renal Tubular Acidosis in Sjögren's Syndrome: A Case Report

“…Sjögren's syndrome often affects middle-aged women, with a sex-adjusted prevalence of 2.2-10.3 per 10,000 individuals [3]. It is often diagnosed based on the American-European Consensus Group's (AECG) criteria for Sjögren's syndrome, which details ocular symptom/sign(s) of three months' duration, oral symptom/sign(s), lymphocytic sialadenitis on minor salivary gland biopsy, and auto-antibodies (anti-Ro/SSA and -La/SSB) as the basis for a diagnosis [4,5]. The diagnosis of primary Sjögren's syndrome requires patients to meet four of the six criteria, which must include histopathology or autoantibodies, or any three of four objective criteria [4].…”

Depression revealing primary Sjögren's syndrome with neurological involvement

“…Neurological manifestations are variously estimated during pSS depending on the series: 8.5-70% and are by far dominated by peripheral neuropathies. 3,6 The central nervous system involvement is much rarer with very polymorphic clinical aspects. 3,6 Psychiatric manifestations are classified among the central neurological disorders of this connective tissue disease, and their prevalence is variously estimated according to the series and the recruitment services: 20-70% of cases.…”

“…3,6 The central nervous system involvement is much rarer with very polymorphic clinical aspects. 3,6 Psychiatric manifestations are classified among the central neurological disorders of this connective tissue disease, and their prevalence is variously estimated according to the series and the recruitment services: 20-70% of cases. 9 They may be the predominant manifestations of the disease, 9 but pSS remains an underestimated cause of neuropsychiatric disorders.…”

“…1,2 With a prevalence of 0.1-3% and an incidence of 3.9-5.3 per 100,000, pSS is the most frequent connective tissue disease, but remains very underdiagnosed. 3 Systemic extra-glandular manifestations of this disease are rare (10-15% of cases) but often serious and condition the prognosis. 1,4 The most frequent of these disorders including pulmonary, renal, neurological, and digestive.…”

“…5 The specific neurological involvement of this disease (neuro-Sjögren) is not uncommon but very polymorphic and often underdiagnosed. 3,6 Depression, classified among the manifestations of central neuro-Sjögren (involvement of the central nervous system) 6,7 can be seen in 32 to 46% of cases. 8 The inaugural forms of the disease are exceptional and represent a real diagnostic challenge for clinicians.…”