A thirty eight years old female was diagnosed with SLE with mucocutaneous, musculoskeletal, haematological and renal involvement in April 2020. She is a dedicated kindergarten teacher who frequently involved in gardening work in the kindergarten compound for the past 15 years. The antinuclear antibody (ANA) and double-stranded DNA antibodies were positive. The ANA showed homogenous pattern with the titre of 1:1280. She was started on hydroxychloroquine 200 mg OD and prednisolone 40 mg OD upon diagnosis of SLE. She was treated as active lupus nephritis in view of heavy proteinuria of 3.39 g/24 h. Intravenous (IV) cyclophosphamide 500 mg was then given to treat her active lupus nephritis. 1 month after her first dose of IV cyclophosphamide treatment, she presented with high grade fever and altered consciousness with Glasgow Coma Scale of 14/15. On physical examination, she had left hemiparesis with 2/5 strength in both upper and lower extremities. There was neck stiffness but all the cranial nerves function were intact. Crepitations were heard over the right lung and a few rounded erythematous subcutaenous nodules were found over the forearms which may represent septic nodules. Abdominal examination was unremarkable. She was initially treated as meningoencephalitis with IV ceftriaxone 2 g BD and IV acyclovir 500 mg TDS.
The initial laboratory tests result were as shown in Fig. 1a. Hepatitis C virus, Hepatitis B virus and human immunodeficiency virus (HIV) screening were all negative. The sputum samples were negative for tuberculosis. Plain computed tomography (CT) brain was done which showed hypodensities at the right frontal region and right parieto-occipital region which may represents infective cerebritis or lupus cerebritis. Lumbar puncture was performed with an opening pressure of 15cmH2O and the cerebrospinal fluid (CSF) results were as shown in Fig. 1b. Her antibiotic was subsequently escalated to IV meropenem 2 g TDS and IV immunoglobulin 0.4 g/kg was given for 5 days in view of worsening sepsis.
Subsequent Magnetic Resonance Imaging (MRI) of the brain revealed more than two multifocal rim-enhancing intraaxial brain lesions with the largest lesion measured at 1.5 cm x 1.4 cm. Serial chest radiographs (CXRs) showed pulmonary nodules of varying sizes with upper lobe predominance and bilateral moderate pleural effusion. Contrast enhanced CT thorax demonstrated scattered clusters of lung nodules, mostly peripherally located, a few cavitations with air-fluid levels, suspicious of infective cavitating nodules. One liver and multiple splenic hypodense lesions were seen in the visualised upper abdomen, suspicious of microabscesses. Given the progressive imaging findings and lack of clinical improvement, anti-tuberculous agents were started empirically.
5 days later, both the blood and CSF cultures grew Burkholderia Pseudomallei. Her diagnosis was revised to disseminated melioidosis with CNS, pulmonary, spleen, bone and cutaneous involvement and the anti-tuberculous agents were discontinued. 2 weeks later, the full sensitivity report showed susceptible B. Pseudomallei towards trimethoprim/sulfamethoxazole, ceftazidime and meropenem with minimum inhibitory concentration of 2 ug/mL, 2 ug/mL and 1.5 ug/mL respectively. The antibiotics was then de-escalated to IV ceftazidime 2 g QID based on the sensitivity report. Blood cultures were repeated 1 week and 2 weeks post treatment which showed clearance of bacteraemia. MRI brain 3 weeks post treatment demonstrated treatment response as evidenced by reduction in the size of the largest brain lesion from 1.5 cm x 1.4cm to 0.9 cm x 0.8 cm. The number of intraaxial brain lesions was also reduced. However, new C2 and C3 vertebrae marrow osteomyelitis were observed. She completed 10 weeks of intensive therapy (2 weeks of IV meropenem and 8 weeks of IV ceftazidime) and was started on single strength trimethoprim/sulphamethoxazole (80 mg/400 mg) three tablets twice a day as eradication therapy. The strength of the left upper and lower extremities improved to 3/5 upon discharge.
The liver and spleen microabscesses resolved based on the repeated imaging. MRI brain and cervical was performed 20 weeks post treatment to decide on the duration of eradication therapy. MRI brain demonstrated a small residual right frontal lobe enhancing focus measuring 0.4 cm with no new cerebral lesion. MRI cervical showed improved osteomyelitis changes at C2 vertebrae. The strength of the left upper and lower extremities improved to 4/5 at this time. The eradication therapy was extended to 12 months in view of presence of cervical vertebral osteomyelitis and residual cerebral lesion. Currently, the strength of her left upper and lower extremities further improved to 5/5 and she is ambulating independently. Her SLE activity was well controlled with hydroxychloroquine 200 mg OD, azathioprine 50 mg OD and prednisolone 10 mg OD.