The patient is a 40-year-old housewife, native of India, resident of the UAE for several years, who had been at her normal state of health up until a month before she presented to a local hospital with complaints of intermittent low grade fever, myalgia, headache, lethargy and behavioral changes in the form of increasing social withdrawal and self-neglect. There had been no known TB exposure or recent travel (her last trip was 5 months prior to presentation to Oman). She denied any personal or family history of autoimmune diseases or TB. On presentation, she was hemodynamicaly stable with a low-grade temperature (37.8C). She had a Glasgow Coma Score of 11/15. There were no meningeal signs and the remainder of the exam was unremarkable. Initial workup including metabolic profile, CBC and CXR were negative, and a lumbar puncture was performed due to abnormal GCS in the absence of other explanations.
The initial cerebrospinal fluid analysis revealed pleocytosis (WBCs 88 cells/muL, lymphocytes 100 %), glucose 63.61 mg/dL (3.53 mmol/L), and elevated protein (404 mg/dL). Neither Gram stain nor acid-fast stain reveal any bacteria. Brain MRI showed diffuse leptomeningeal enhancement. At the time, given her 1 month history of intermittent low grade fevers, associated with headaches and her due to her being from a high risk area, anti-TB therapy was initiated (rifampin, isoniazid, pyrazinamide, ethambutol and dexamethasone 4 mg intravenous twice daily). After 14 days she had slight improvement in the level of consciousness but developed a severe drug eruption and all medications were discontinued. Her mental status declined over the ensuing days and was transferred to our facility for further care as a case of TB meningitis.
She was admitted to our ICU as a case of meningitis/encephalitis, she was awake, lying curled on the bed with generalized rigidity. Her temp was 39C and she was saturating at 100 % on room air with normal other vital signs. Repeat investigations were done which included CT head and MRI brain, which were normal, as well as 24 h EEG monitoring which showed no epileptiform discharges. Repeat CSF analysis revealed normal glucose of 68.65 mg/dL (3.81 mmol/L), elevated protein (183 mg/dL), pleocytosis (11cells/muL, 99 % lymphocytes). The Meningitis/Encephalitis cerebrospinal fluid (CSF) panel was negative (Polymerase Chain Reaction (PCR) was done for: Escherichia coli, Haemophilus influenzae, Listeria monocytogenes, Neisseria meningitidis, Streptococcus agalactiae (Group B), Streptococcus pneumoniae, Cryptococcus gattii and neoformans, Cytomegalovirus, Herpes simplex virus 1 and 2, Human herpesvirus 6, Enterovirus, Human parechovirus, and Varicella zoster virus). Additional CSF studies included VDRL, mycobacterial PCR, Gram stain, and culture. Blood serology tested for human immunodeficiency virus (HIV), Brucella, syphilis and Lyme disease were all negative.
Second-line regimen for TB was initiated (moxifloxacin, linezolid, amoxicillin/clavulanate, meropenem, and amikacin IV). Isoniazid was reintroduced slowly after obtaining verbal consent from the next of kin. Additionally, dexamethasone 0.3 mg/kg/day intravenously (IV) for two weeks, then the dose was tapered (0.2 mg/kg/day IV for a week, 0.1 mg/kg/day IV for the 4th week, then 4 mg per day orally and further tapered by 1 mg off the daily dose each week (total 8 weeks). However, the diagnosis of CNS tuberculosis came into question due to the lack of evidence from microbiology and failure of empiric treatment. Other investigations that were done included a quantiferon gold assay which was negative, a repeat CT head and MRI brain, which were normal, as well as CT of the chest, abdomen and pelvis which showed no evidence of related TB findings. The possibility of autoimmune disease was then considered. Laboratory tests results are displayed in Table 1.
Based on the 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for SLE, our patient's clinical and biochemical picture fits with the diagnosis of SLE. The classification for SLE requires the presence of a positive antinuclear antibodies (ANA) as an entry criterion. Additive criteria consist of seven clinical (ie, constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal) and three immunologic (ie, antiphospholipid antibodies, complement proteins, SLE-specific antibodies) categories, each of which are weighted from 2 to 10. Patients are classified as having SLE with a score of 10 or more points.
Our patient had positive ANA titers, which made her eligible for the 2019 EULAR/ARCP criteria. She presented with a one-month history of intermittent fevers (two points) associated with neuropsychiatric symptoms (three points). She had low C3 and C4 levels (four points), and positive anti-smith antibodies (six points). This gave her a total score of 15, consisted with SLE. Once her final AFB cultures came out and her rheumatology work up came highly suspicious for SLE. We discontinued her TB medications and started her on a tapering dose of Prednisolone starting at 40 mg daily with a 10 mg taper each week. She followed up with a rheumatologist at an outside hospital, which confirmed our diagnosis of SLE and stared her on hydroxychloroquine 200 mg per day and prednisone 5 mg daily. She is doing well and has returned to her baseline.