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Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
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Staring at an abscess, but lupus stares back…
Sandeep Surendran1, Thomas George2, Suma Balan1
1Department of Rheumatology, Amrita Institute of Medical Sciences, Kochi, India.
The Journal of the Royal College of Physicians of Edinburgh
|September 16, 2020
Summary
Systemic lupus erythematosus (SLE) can rarely cause tumefactive demyelination, leading to facial palsy. Aggressive immunosuppression effectively treated this rare neurological complication in a pediatric patient.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
- Neuropsychiatric involvement in SLE, including demyelinating lesions, is uncommon.
- Macrophage activation syndrome (MAS) is a severe complication of autoimmune diseases.
Observation:
- A 12-year-old male presented with active SLE, lupus nephritis, and secondary MAS.
- The patient developed left-sided upper motor neuron (UMN) facial palsy.
- This palsy was attributed to tumefactive demyelination linked to SLE.
Findings:
- Tumefactive demyelinating lesions are an exceptionally rare presentation in neuropsychiatric SLE.
- The case highlights a unique neurological manifestation of SLE in a pediatric patient.
- Facial palsy secondary to demyelination is a significant finding in this context.
Implications:
- This case underscores the importance of considering rare neurological complications in SLE.
- Prompt diagnosis and aggressive immunosuppressive therapy are crucial for managing such severe manifestations.
- The successful treatment with steroids and cyclophosphamide suggests a potential therapeutic strategy for similar rare cases.
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