#3928 NEPHROTIC SYNDROME IN LIVER DISEASES: PRESENTATION OF TWO UNUSUAL AND INSTRUCTIVE CASES
Authors: Ledó Nóra , Dobi Deján , Folhoffer Anikó 
Published: June 2023

Background and Aims

However, there are known associations between chronic liver diseases and glomerulopathies, here we report two rare cases, where diagnostic and therapeutic challenges influenced the patients’ care.

Method

The patients’ medical parameters were obtained from the electronical charts of the hospital. Kidney biopsy samples were examined with immunofluorescence (frozen samples), light microscopy (paraffin embedded tissue, 2-3 um sections) and electron microscopy (1 um, toluidine blue). The follow-up time was 24 months for case 1 and 23 months for case 2. Both patients gave written consent to the presentation of the reports.

Results

Case 1: A 42-year-old man with recent pulmonary embolism was presented in our hospital with nephrotic syndrome and reduced kidney function (estimated glomerular filtration rate (eGFR): 43 ml/min/1.73 m2, urinary protein/creatinine ratio (UPCR): 2124 mg/mmol, serum albumin: 24 g/L). Kidney biopsy confirmed primary membranous nephropathy (MN). The anti-phospholipase-A-2 receptor (aPLA2R) was positive in high titer, and PLA2R positivity was also seen in the renal sample. Additionally, an active hepatitis C (HCV) infection with viremia was confirmed. The nephrotic syndrome was worsened with severe edema requiring ultrafiltration; therefore, after one-week direct anti-HCV therapy, methylprednisolone was started. Immunosuppressive therapy had to be determined soon due to severe infections. With combined direct antiviral treatment, HCV PCR test became negative, which was followed by the disappearance of the aPLA2R antibodies. The patient's kidney function stabilized and there are no symptoms of nephrotic syndrome (eGFR: 52 ml/min/1.73 m2, serum albumin: 37 g/L, UPCR: 321 mg/mmol).

Case 2: A 66-year-old woman was admitted to the hospital with coronavirus disease (COVID)- associated pneumonia. At admission, nephrotic syndrome was diagnosed (eGFR: 29 ml/min/1.73 m2, serum albumin: 22 g/L, proteinuria: 8 g/day). Additionally, elevated gamma glutamyl transferase was registered. Immune serology was negative for aPLA2R, but antinuclear antibodies (anti-cytoplasm, chromatin, Ro), and anti-mitochondrial antibodies (AMA-M2) were positive. Biopsies were performed after the COVID infection. Liver biopsy confirmed primary biliary cholangitis (stage 1-2), while the kidney biopsy showed secondary membranous nephropathy with signs of potential monoclonal gammopathy (monoclonal immunoglobulin-G1-kappa deposits). After the exclusion of malignancies, monoclonal gammopathy, and potential other causes of the secondary MN, PBC-associated MN was diagnosed. Based on case reports in the literature, cyclosporine A treatment was started. The patient reached complete remission in 5 months (no significant proteinuria, stable eGFR around 34 ml/min/1.73 m2).

Conclusion

The association of an active HCV infection and an aPLA2R positive MN has not been reported in the literature. Nephrotic syndrome and the aPLA2R positivity disappeared related to the direct antiviral treatment, which may indicate a diagnosis of secondary MN. PBC-associated secondary MN is a known but rare entity, where calcineurin inhibitor treatment may reduce the symptoms of nephrotic syndrome.