Abstract: TH-PO516
Anti-PLA2R Membranous Nephropathy Associated With Multiple Sclerosis: First Case Report
Session Information
- Glomerular Diseases: Clinical, Outcomes, Trials - I
November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials
Authors
- Cunha, Milena Vizioli, Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Silveira, Vinícius Sousa da, Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Souza, Lucas Assis Santos de, Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Silva, Guilherme Diogo, Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Lucato, Leandro Tavares, Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Cavalcante, Livia Barreira, Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Jorge, Lectícia, Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Dias, Cristiane B., Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Noronha, Irene L., Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
- Yu, Luis, Universidade de Sao Paulo, Sao Paulo, São Paulo, Brazil
Introduction
Membranous nephropathy (MN) is a common cause of adult nephrotic syndrome, 80% are considered primary, in which the main antigens are the M-type phospholipase A2 receptor (PLA2R), thrombospondin type 1 domain-containing 7A (THSD7A) and neuro epidermal like growth factor 1 (NELL-1). Recently, new antigens have been described, including contactin-1, a podocyte and neuronal adhesion molecule with similarities with PLA2R and THSD7A, responsible for a MN associated with chronic inflammatory demyelinating polyneuropathy (CIDP).
Case Description
57 years old, white male, who complained of legs swelling, foamy urine and arterial hypertension in the last 4 months. He also complained of left acute painful vision loss 5 days ago. His laboratory exams demonstrated: Creatinine1.4g/dl; Urine1 – protein 5g/L, Leukocytes0, Eritrocytes0; urine protein/creatinine ratio8.8g/g; serum albumin2.1g/dl; cholesterol270mg/dl; Complement C3 and C4 normal. Serum anti-PLA2R 406UR/ml (Elisa). Screening for other autoimmune diseases, infections and cancer resulted negative.
Kidney biopsy revealed stage 1/2 MN (Fig 1) and immunofluorescence showed diffuse granular subepithelial deposits of IgG and C3.
Cranial NMR revealed periventricular white matter alterations suggestive of demyelinating CNS disease (Fig 2) and optic neuritis.
Discussion
This is the first report of an anti-PLA2R MN with concurrent MS. A common pathogenesis is suggested, similarly to MN associated to CIDP. We suggest that the immune response in the primary MN may have unmasked other podocyte antigens, such as contactin 1, causing simultaneously the neurological involvement. Further investigation is necessary for pathogenesis elucidation.
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