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Title: Systemic Lupus Erythematosus Presenting As Mixed Sensori-motor Axonal Polyneuropathy
Authors: Mutahra Khaliq, Nauman Ismat Butt, Muhammad Sohail Ajmal Ghoauri, Fahad Qaisar, Tayyaba Hashmi, Sabir Ali
Journal: Journal of Islamabad Medical and Dental College (JIMDC)
Publisher: Healers Educational Society
Country: Pakistan
Year: 2024
Volume: 13
Issue: 2
Language: English
DOI: 10.35787/jimdc.v13i2.1074
Systemic Lupus Erythematosus (SLE) is an autoimmune disease that has multisystem manifestations. However, axonal polyneuropathy is rare in SLE. We report the case of a 41-years old lady who presented with gradual onset, progressively worsening bilateral lower limb weakness for 1 month along with tingling, prickling and burning sensation. There was history of dry eyes, oral ulcers, dry mouth, dental caries, lethargy and fatigue. On examination, poor oral hygiene, coarse dry tongue, oral ulcers, dental caries and conjunctival pallor were noted. Neurological examination of lower limbs revealed flaccid LMNL paralysis with paresthesia of both feet extending up to the mid-shin level. She had normochromic normocytic anemia with hemoglobin 10.8 g/dl and raised ESR 40 mm/hour. NCS revealed mixed sensori-motor axonal polyneuropathy. ANA, anti-DsDNA (138 IU/ml), Anti-Sm (>200 U/ml) and anti-Ro/SSA antibodies (146 U/ml) were positive with low serum C3 (29.3 mg/dl) and low serum C4 (07 mg/dl). She was diagnosed as SLE and started on plasmapheresis, steroids, hydroxycholoroquine and azathioprine with marked improvement at follow-up.
To report a case of Systemic Lupus Erythematosus (SLE) presenting as mixed sensori-motor axonal polyneuropathy and discuss its management.
Case report detailing a 41-year-old female patient's presentation, examination findings, laboratory investigations (including NCS, ANA, anti-DsDNA, Anti-Sm, anti-Ro/SSA, C3, C4), diagnosis, and treatment.
graph TD;
A["Patient Presentation with Symptoms"] --> B["Clinical Examination"];
B --> C["Laboratory Investigations"];
C --> D["Nerve Conduction Studies NCS"];
C --> E["Autoantibody Profile & Complement Levels"];
D --> F["Diagnosis: SLE with Polyneuropathy"];
E --> F;
F --> G["Treatment Initiation"];
G --> H["Plasmapheresis"];
G --> I["Immunosuppressants Steroids, Hydroxychloroquine, Azathioprine"];
H --> J["Follow-up & Outcome Assessment"];
I --> J;
J --> K["Marked Improvement"];
The discussion highlights the rarity of axonal polyneuropathy in SLE, the diagnostic criteria for SLE (SLICC), potential pathogenesis of peripheral neuropathy in SLE, and treatment strategies, emphasizing the importance of timely diagnosis and adequate treatment for improved outcomes.
The patient presented with progressive bilateral lower limb weakness, tingling, and burning sensations. Investigations revealed normochromic normocytic anemia, raised ESR, mixed sensori-motor axonal polyneuropathy on NCS, positive SLE autoantibodies (ANA, anti-DsDNA, Anti-Sm, anti-Ro/SSA), and low serum C3 and C4. The patient showed marked improvement with plasmapheresis, steroids, hydroxychloroquine, and azathioprine.
Systemic Lupus Erythematosus can present with rare manifestations like mixed sensori-motor axonal polyneuropathy. Prompt diagnosis and aggressive immunosuppressive therapy, including plasmapheresis, steroids, hydroxychloroquine, and azathioprine, can lead to significant improvement in neurological function and quality of life.
1. The patient was 41 years old. (Confirmed in the text)
2. Anti-DsDNA level was 138 IU/ml. (Confirmed in the text)
3. Low serum C3 level was 29.3 mg/dl. (Confirmed in the text)
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