Doctors should consider a diagnosis of primary Sjogren’s syndrome when faced with adolescent patients who show psychotic symptoms such as suicidal thoughts, auditory hallucinations, and paranoia, according to a case series.
Titled “Adolescent Sjogren’s syndrome presenting as psychosis: a case series,” the report was published in Pediatric Rheumatology.
Neurological involvement has been reported in a significant number — up to 80% — of adults with primary Sjogren’s syndrome. Many times, in fact in 50–80% of cases, such reports precede diagnosis. Psychiatric symptoms also have been described in this patient population, with reports of depression, anxiety, and cognitive deficits.
Now, researchers at the Rady Children’s Hospital, in San Diego, described the cases of four adolescent girls, ages 16-19, who were diagnosed with primary Sjogren’s syndrome after showing psychiatric disturbances in clinic evaluations.
One patient, a 16-year old girl, had a four-year history of severe anxiety, obsessive compulsive disorder, and tic disorder, partially under control. The other three girls had no significant past medical history.
While the teenagers had different sets of symptoms, their neuropsychiatric complaints included abnormal behavior, tremors, insomnia, suicidal thoughts, severe anxiety, obsessive compulsive disorder, auditory hallucinations, incoherent speech, headache, paranoia, confusion, and rapid, often exaggerated changes in mood.
None of the patients complained of dryness of the eyes’ cornea and conjunctiva, called keratoconjunctivitis, or dry mouth, known as xerostomia — both common symptoms of Sjogren’s syndrome. Patient one reported drug use and, along with patient three, had a history of sexual assault.
Following clinical assessments, the researchers reported that all four girls tested positive for anti-nuclear antibodies (ANA), which indicate the presence of an autoimmune disorder such as Sjogren’s.
For all of the teens, the diagnosis of primary Sjogren’s syndrome was made only after standard therapy for psychiatric illnesses had failed.
For patient one, the diagnosis was based on positive ANA and increased levels of autoantibody anti-Sjögren’s syndrome type A (SSA), one of the clearest blood test markers for Sjögren’s. The girl also had inflammation of the salivary glands, as determined by biopsy. She was diagnosed according to the criteria of the 2017 American College of Rheumatology (ACR)/European League Against Rheumatism classification (EULAR).
Patient two had positive ANA and had increased levels of autoantibody SSA and SSB. The researchers also found evidence of organic brain disease, revealed by magnetic resonance imaging (MRI) and electroencephalogram (EEG). This teenager did not consent to labial salivary gland biopsy and refused ophthalmology evaluation for Schirmer’s testing, which measures dry eye.
Although not fulfilling the 2017 ACR/EULAR diagnostic criteria, the patient was diagnosed with primary Sjogren’s.
The primary Sjogren’s syndrome diagnosis for patient three was based on positive ANA, increased levels of autoantibody SSA and SSB, and an abnormal Schirmer’s test, which determines if a person’s tear glands produce enough tears to keep the eyes adequately moist. In addition, the diagnosis was based on a salivary gland biopsy that revealed immune infiltrates.
Patient four had positive ANA and increased levels of autoantibody SSA. She also had low values of complement component 4 (C4), which are typical of autoimmune disorders, but had no further signs or symptoms of lupus.
Salivary gland biopsy and/or keratoconjunctivitis testing was not performed, but the girl was diagnosed as presumed primary Sjogren syndrome.
All four teenagers were treated with immunosuppresive medication. Specifically, patients one and three were treated with Rituxan (rituximab), mycophenolate mofetil, and prednisone. The treatment led to significant cognitive improvements and resolution of the girls’ psychotic symptoms.
Patient two was treated with Rituxan, prednisolone, and Gazyva (obinutuzumab), resulting in improvements in mood and sleep and a lessening of auditory hallucinations.
Patient four was treated with Rituxan, methylprednisolone, and prednisone. Her mental status eventually improved over a period of weeks to months and she regained normal cognitive function. She continued to be treated for depression/anxiety but “was able to wean off all antipsychotics.”
“It is impossible to exclude the possibility that these patients had primary psychiatric disease and not secondary psychosis related to” primary Sjogren’s syndrome, the researchers said.
“However, given the suspicion for organic brain disease in these patients as well as the known association of psychosis with [primary Sjogren’s syndrome], the risk benefit analysis favored empiric treatment for presumed [primary Sjogren’s syndrome] and all patients showed improvement following treatment with rituximab,” they said.
Some studies have used Rituxan in a scenario of primary Sjogren’s syndrome with central nervous system involvement, albeit with varying results.
These results, however, suggest that Rituxan “may be an effective treatment option that should be considered early after the diagnosis of [primary Sjogren’s syndrome]-associated psychiatric disturbance,” the scientists said.
So far, the U.S. Food and Drug Administration has approved the use of Rituxan for the treatment of patients with non-Hodgkin’s lymphoma, chronic lymphocytic leukemia, rheumatoid arthritis, granulomatosis with polyangiitis, microscopic polyangiitis, and pemphigus vulgaris.
According to the team, primary Sjogren’s syndrome should be considered in the differential diagnosis — when physicians look at the possible disorders that could be causing the symptoms — of young patients with new psychiatric disorders. The disease should be considered even in the absence of typical Sjogren’s symptoms like dryness of the eyes and mouth, they said.
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