#2909 DOUBLE GLOMERULOPATHY OF THIN BASEMENT MEMBRANE DISEASE AND IGA NEPHROPATHY PRESENTING WITH NEPHROTIC SYNDROME IN AN ADULT WOMAN
Authors: Polito Johannes Kepler , Polito Eratosthenes 
Published: June 2023

Background and Aims

We report a case of a 44-year-old woman with no known comorbid conditions who, eventually was diagnosed to have simultaneous occurrence of IgA nephropathy and thin basement membrane disease atypically presenting with heavy proteinuria, hypoalbuminemia, bipedal edema, ascites, bilateral pleural effusion, and hemodynamic instability, but with a surprisingly normal light microscopic findings and an Oxford MEST C score of 0 on renal biopsy. who, clinically improved with immunosuppression and angiotensin II receptor blocker therapy. Physical exam revealed an overweight female, with facial swelling, no rashes, periorbital and a Grade II bipedal pitting oedema. The presence of decreased breath sounds and dullness on percussion on both lower lung fields were also observed. Shifting dullness and fluid wave on abdominal exam. The patient was afebrile, no desaturation at room air and a palpatory blood pressure of 70 mmHg. Secondary causes of glomerulonephritis such as Hepatitis B and Hepatitis C infection were ruled out. Antinuclear antibody (ANA) test, compliment (C3 and C4) with anti-dsDNA determination to rule out SLE were unremarkable as well. Serum creatinine was elevated at 2.63 with estimated GFR of 22 mL/min. Further work-up with 24-hour total urine protein was 22,026.2 grams/day The patient underwent percutaneous kidney biopsy which showed IgA Nephropathy with a MEST C score of 0 with simultaneous occurrence of thin basement membrane disease.

Method/ Treatment

Pulse steroid therapy was started with Methylprednisone 1-gram intravenous infusion once daily for 3 days followed with Prednisone (1mg/kg/day) 60 mg a day and a low-dose of Angiotensin receptor blocker, Candesartan, 4mg once daily for the proteinuria.

Results

After 8 weeks of corticosteroid and ARB therapy, patient was seen at the clinic with resolution of bipedal edema, no demonstrable ascites and clear breath sounds on chest and lungs auscultation. Blood pressure was 130/80 with no proteinuria on dipstick, random urine protein: creatinine ratio (UPCR) of 0.07, creatinine of 0.97 mg/dl and BUN of 17 mg/dl. Prednisone was then reduced with the goal to taper the dose for a period of 6 months.

Conclusion/ Significance

Both IgA nephropathy and thin basement membrane disease usually presents with hematuria, hypertension, and varying degrees of proteinuria. However, nephrotic syndrome as its presentation has not been well characterized to date, and whether this adds to the mortality is not yet established. Although the most widely accepted system, the Oxford MEST-C scores is utilized in predicting renal outcomes, its role in double glomerulopathies has not been validated. Even though many investigators have indicated that the presence of heavy proteinuria at the initial evaluations was almost always associated with progressive renal failure and that there is no effective medical treatment aside from supportive care, in this study, a trial of immunosuppression yielded improved clinical outcomes.