[Systemic lupus erythematosus complicated by autoimmune nodopathy: A case report]

Area of Science:

• Neurology
• Rheumatology
• Immunology

Background:

• Systemic lupus erythematosus (SLE) is a complex autoimmune disease with diverse clinical manifestations.
• Autoimmune nodopathy (AN) is a rare subtype of peripheral neuropathy, recently characterized by specific autoantibodies.
• The co-occurrence of SLE and AN is exceptionally rare, with limited understanding of their shared pathophysiology.

Purpose of the Study:

• To report a unique case of SLE complicated by contactin-1 (CNTN1) antibody-positive autoimmune nodopathy (AN).
• To provide insights into the early recognition and precise management of this rare comorbidity.
• To review existing literature and discuss potential shared pathogenic mechanisms and therapeutic strategies.

Main Methods:

• Detailed clinical case presentation of a 48-year-old female patient with progressive neurological symptoms and edema.
• Comprehensive laboratory investigations including cerebrospinal fluid analysis, electromyography, antibody testing (anti-CNTN1, anti-dsDNA, ANA), and renal biopsy.
• Review of relevant medical literature on SLE, autoimmune nodopathy, and their co-occurrence.

Main Results:

• The patient presented with peripheral neuropathy, nephrotic syndrome, and later developed SLE criteria, including positive ANA and anti-dsDNA antibodies.
• Diagnosis of SLE, CNTN1 antibody-positive AN, and lupus nephritis was confirmed.
• Treatment with B-cell-depleting therapy (rituximab) combined with corticosteroids and hydroxychloroquine led to significant clinical improvement and laboratory normalization over a 2-year follow-up.

Conclusions:

• This case highlights the importance of considering autoimmune nodopathy in patients with peripheral neuropathy and SLE, and vice versa.
• B-cell-depleting therapy appears to be a cornerstone in managing this rare comorbidity.
• Further research is needed to elucidate shared pathogenic mechanisms and optimize treatment protocols for coexisting SLE and AN.