NELL-1 positive membranous nephropathy in association with antisynthetase syndrome

Area of Science:

• Nephrology
• Immunology
• Rheumatology

Background:

• Membranous nephropathy (MN) is a leading cause of nephrotic syndrome, often progressing to kidney failure.
• Primary MN is commonly associated with anti-phospholipase A2 receptor (anti-PLA2R) antibodies, but other autoantigens are increasingly recognized.
• Antisynthetase syndrome is an autoimmune disorder characterized by specific autoantibodies and various clinical manifestations.

Purpose of the Study:

• To report a unique case of nephrotic syndrome in a patient with newly diagnosed antisynthetase syndrome.
• To investigate the underlying cause of membranous nephropathy in this patient.
• To explore a potential novel association between antisynthetase syndrome and a specific autoantigen in MN.

Main Methods:

• Clinical case presentation of a male patient in his mid-60s.
• Diagnosis of nephrotic syndrome and antisynthetase syndrome.
• Kidney biopsy with histopathological examination and immunohistochemical staining for autoantigens.

Main Results:

• The kidney biopsy confirmed membranous nephropathy (MN).
• Immunohistochemical staining revealed positivity for the neural epidermal growth factor-like 1 protein (NELL-1) autoantigen.
• The patient's presentation suggests a link between antisynthetase syndrome and NELL-1 positive MN.

Conclusions:

• This case suggests a possible novel association between antisynthetase syndrome and NELL-1 positive membranous nephropathy.
• The findings expand the spectrum of known autoantigens implicated in MN.
• Further research is warranted to confirm and elucidate this potential association.