Abstract: PO1500
Pemphigus Vulgaris and PLA2R-Associated Membranous Nephropathy: Two IgG4-Related Diseases in the Same Patient
Session Information
- Glomerular Diseases: The Excitement of Clinical Cases
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Costa, Camila Lucena, Hospital dos Servidores do Estado de Pernambuco, Recife, PE, Brazil
- Correia, Matheus Ribeiro Barros, Hospital Getulio Vargas, Recife, PE, Brazil
- Sette, Luis H.B.C., Universidade Federal de Pernambuco, Recife, PE, Brazil
- Costa, Denise MARIA DO NASCIMENTO, Universidade Federal de Pernambuco, Recife, PE, Brazil
- Oliveira, Camila Barbosa Lyra, Universidade Federal de Pernambuco, Recife, PE, Brazil
- Cavalcante, Maria Alina G.M., Universidade Federal de Pernambuco, Recife, PE, Brazil
- Valente, Lucila Maria, Universidade Federal de Pernambuco, Recife, PE, Brazil
- Vajgel, Gisele, Universidade Federal de Pernambuco, Recife, PE, Brazil
Introduction
The relationship between bullous skin diseases and glomerulopathies has been increasingly recognised. Other bullous diseases were previously reported in association with membranous nephropathy (MN), but the association between MN and pemphigus vulgaris (PV) has not been reported in the literature yet.
Case Description
A 39-years-old smoker woman presented with trunk blisters and worsened mouth ulcers after stopping the treatment for MN. One year before she had nephrotic syndrome and the kidney biopsy was positive for anti-PLA2R and IgG4 (and negative for THSD7A) in the immunohistochemistry (IHQ). Serum anti-PLA2R was negative. Four months earlier she was started on cyclosporine, but stopped it due to mouth ulcers. She had nephrotic range proteinuria, no edema, when the painful blisters spread out on the trunk, back, limbs, scalp as well as ulcers in the oral cavity and oesophagus. The diagnosis of PV was confirmed by skin and oesophagus biopsy and IHQ showed the presence of IgG4 subclass antibody in the epithelial tissue. She received pulse and oral steroids along with azathioprine for the PV. The skin lesions were slowly healing and no more new blisters have appeared.
Discussion
The humoral auto-immune response in pemphigus produces anti-desmoglein 1 and 3, both IgG4. Desmogleins are responsible for adhesion in stratified squamous epithelia, when damaged produces to the blistering eruptions. There is a genetic predisposition between PV and MN, with HLA-DQA1, HLA-DRB1 and the thrombospondin gene (THSD7A). Environmental factors such as smoking and air pollution could act as a second trigger for the development of auto-immune diseases. PLA2R can be expressed in the bronchiolar tissue and in macrophages of the lung, however there is no evidence of histological damage in the pulmonary tissue. Although we found no description on the expression of PLA2R in the skin, in an attempt to find a common antigen for both diseases, we did search for anti-PLA2R in the skin, but it was negative.
In conclusion, this is the first described case of association of pemphigus vulgaris and PLA2R-associated MN, both IgG4-related conditions that involve the production of different autoantibodies directed to skin and kidney antigens.