We present a 46-year-old female from a major city in Mexico. She has had poorly controlled type 2 diabetes mellitus (DM) and high blood pressure for 12 years, with no prior chronic kidney disease (CKD) diagnosis. In February 2022, following a course of antibiotics for a urinary tract infection caused by Proteus mirabilis, she developed progressive bilateral bimalleolar edema that later progressed to ascites. In March 2022, the patient began experiencing melena. An upper endoscopy (image 1) revealed four varicose cords measuring less than 5 mm in the mucosa of the distal third of the esophagus, extending into the middle third. The gastric mucosa showed subepithelial hemorrhage, but no erosions. These findings indicated the presence of esophageal varices without high-risk stigmata, along with severe portal hypertensive gastropathy.
Due to the clinical presentation of fluid overload, ascites, and upper gastrointestinal bleeding attributed to portal hypertension, along with poorly controlled type 2 diabetes mellitus, she received a clinical diagnosis of chronic hepatic cirrhosis caused by non-alcoholic fatty liver disease (NAFLD). Ultrasound revealed an enlarged liver with normal echogenicity, no focal or diffuse lesions, no biliary dilatation, and a tortuous and irregular portal vein with portosystemic collaterals. The spleen was enlarged, but the splenic vein was not assessed. Kidney dimensions were normal. She was treated for esophageal varices related to cirrhosis with spironolactone, furosemide, and propranolol but continued to experience a weight gain of over 20 kg due to fluid overload.
Referral to the nephrology service came after the patient's blood urea and creatinine levels increased from 1.2 mg/dl March 22 to 2.35 mg/dl January 23, likely due to a type 2 hepatorenal syndrome and uncontrolled hypertension. The patient had ambulatory blood pressure over 160/90 and normal liver function tests but exhibited low plasma albumin levels and nephrotic range proteinuria. Medication adjustments were made for decongestion, and further tests, including a hepatic and biliary ultrasound, showed a normal liver, but also a patent portal vein with cavernomatous degeneration (image 2), adequate flow in suprahepatic veins, and portal vein diameter and velocities. The spleen and vein were normal. Triphasic computed tomography of the liver confirmed normal liver appearance, collateral vessel presence, and recanalization from the right to the left portal vein due to cavernomatous degeneration, suggestive of portal hypertension and findings of hypertensive gastropathy.
Hospitalization was initiated to investigate a possible nephrotic syndrome and rule out secondary membranous nephropathy. Serological tests for ANA, Anti-DNA, HBV, HCV, and HIV were negative. C3 and C4 levels were normal, but the patient couldn't afford Anti-PLA2R testing. Ca125 levels were high at 239.8 (table 1). Cytologic evaluation of ascitic pleural fluids revealed no malignant cells. A repeat upper endoscopy found no esophageal varices, but the stomach mucosa exhibited subepithelial hemorrhage, indicative of portal hypertension. A kidney biopsy (image 4) demonstrated glomerular changes consistent with Diabetic Nephropathy Class IV (RPS), which was surprising given the prior lack of CKD diagnosis.