Central nervous system lupus presenting as paranoia

Sir,

Pediatric data suggest that the incidence of systemic lupus erythematosus (SLE) with onset before the age of 19 years is probably between 6 and 18.9 cases per 100,000.[1] SLE has a tendency for widespread organ involvement. Central nervous system (CNS) disease is generally associated with a poor prognosis that is only second in severity to renal involvement. The american college of rheumatology (ACR) criteria proposed 19 syndromes under the common definition of neuropsychiatric SLE.[2]

Miss X, a 16-year-old unmarried female diagnosed to have SLE in July 2004, was admitted with chief complaints of dyspnea in September 2008. On evaluation, she had lower respiratory tract infection (LRTI) and was put on antibiotics. On the second day, she became suspicious of her surroundings, would repeatedly check the floor beneath the bed and the ward, believed that the paramedical staff has joined hands with the terrorists and that she is being discussed amongst the outsiders. These symptoms were accompanied by feeling of heaviness in the head and headache. In the physical examination, she had discoid rash and inflamed right ankle joint. The laboratory investigations revealed positive tests for anti-nuclear antibody (ANA), anti ds-DNA Ab and anti-ribosomal P Ab. Keeping all this in view, a diagnosis of CNS lupus was made.

In view of her psychotic features, the patient was put on Tab. Olanzapine 5 mg twice daily and Tab. Lorazepam 1 mg in the night, apart from being put on mycophenolate mofetil during the hospitalization. After 2 days, the symptoms had significantly reduced (assessed by clinical interview). The patient remained asymptomatic in the follow-up period after 2 months in November 2008 on Tab. Olanzapine 5 mg BD. Patient's antipsychotic was stopped in the next 2 months and she had been doing well. The last follow-up visit was in early 2011.

Neuropsychiatric SLE (NP-SLE) occurs in 20-70% of pediatric SLE patients.[3] Most patients with NP-SLE have the initial neuropsychiatric signs and symptoms within the first year of diagnosis of SLE. Cognitive dysfunction and depression are the most common neuropsychiatric disorders in patients with SLE, occurring in up to 80–50% of patients.[4] The diagnosis of psychosis is made in 30–50% of pediatric patients with neuropsychiatric involvement. Characteristically, the hallucinations have features of an organic psychosis frequently accompanied by headaches, cognitive dysfunction, and confusion. Suicidal ideations are common. Recovery is complete, but relapses are not uncommon. Steroid-induced psychosis is uncommon in SLE and may be differentiated from NP-SLE by the absence of other features of CNS involvement, including headache, confusion, and concentration difficulties, and the presence of mania, head banging, and excessive crying (uncommon features in NP-SLE). A prompt response to treatment with increased doses of steroids may also help in differentiating SLE-related psychosis from steroid-induced psychosis. International Research Alliance called the systemic lupus international collaborating clinics (SLICC) have examined the association between anti-ribosomal P (anti-P) antibodies and psychosis.[5]

Our patient presented with neuropsychiatric signs after 4 years of the disease onset, which is atypical. However, recovery was complete for the neuropsychiatric disorder.

Managing the psychotic symptoms with antipsychotics along with the underlying treatment needs further studies that would provide us with guidelines as to the dose and duration of treatment.