Loma Linda University Medical Center Loma Linda, CA, United States
Emily Lin, MD, Brian T. Lee, MD Loma Linda University Medical Center, Loma Linda, CA
Introduction: Porto-sinusoidal vascular disease (PSVD) and idiopathic non-cirrhotic portal hypertension (INCPH) have recently been recognized in the spectrum of vascular liver disease. Patients can have a heterogeneous presentation of clinical and histologic findings. Here we report the first known case of a patient who developed massive ascites and was found to have PSVD and INCPH in the setting of diffuse aortitis.
Case Description/Methods: A 69-year-old man with no history of liver disease presented with massive ascites. He initially developed ascites after an abdominal hernia repair, resulting in fluid removal. Physical exam was notable for abdominal distention with tense ascites, lower extremity edema, and temporal wasting, but no stigmata of chronic liver disease. Prior ascitic fluid analysis showed a low serum-ascites albumin gradient (SAAG) of 0.9 g/dL (2.3 - 1.4 g/dL) with a fluid protein of 3.7 g/dL; cytology was negative for malignancy. Laboratory results revealed white blood cell count 15.29 K/µL, hemoglobin 10.1 g/dL, creatinine 1.5 mg/dL, aspartate aminotransferase 7 U/L, alanine aminotransferase 12 U/L, alkaline phosphatase 93 U/L, total bilirubin < 0.2 mg/dL, albumin 2.9 g/dL, total protein 6.8 g/dL, and INR 1.5. Immunologic workup resulted in elevated ANA titers, erythrocyte sedimentation rate, and C-reactive protein levels but otherwise showed no other positive results. Echocardiogram was normal. Chest and abdominal computed tomography demonstrated diffuse wall thickening of the entire aorta (Figure 1A, 1B), renal pelvis, and ureters along with an enlarged main portal vein, portosystemic collaterals (Figure 1B, 1C), ascites, and peritoneal thickening. A transjugular liver biopsy was done with a hepatic venous pressure gradient of 3 mmHg (13 – 10 mmHg). Histology was consistent with obliterative portal venopathy (OPV) (Figure 1D, 1E, 1F). Due to concern for large vessel vasculitis, the patient was started on corticosteroid therapy with IV methylprednisolone with transition to oral prednisone. He had improvement of his ascites during outpatient follow-up.
Discussion: This is the first known case of PSVD and INCPH manifesting as OPV and massive ascites in the setting of idiopathic large vessel vasculitis. This case study reveals an uncommon association between vasculitis and PSVD and INCPH, highlighting the heterogenous clinical presentation of this relatively under-recognized disease entity.
Figure: Figure 1. (A) Coronal view of chest CT showing thickened aortic arch. (B) Axial view of abdominal CT showing thickened abdominal aorta (arrow) and portosystemic collaterals (asterisk). (C) Coronal view of abdominal CT showing patent portal vein and portosystemic collaterals. (D, E) Liver histology identifying herniated portal vein (arrow, asterisk) into hepatic parenchyma (H&E stain). (F) Increased number of portal vascular channels (arrows).
Emily Lin indicated no relevant financial relationships.
Brian Lee indicated no relevant financial relationships.
Emily Lin, MD, Brian T. Lee, MD. P1877 - Porto-Sinusoidal Vascular Disease in a Patient With Diffuse Aortitis and Massive Ascites, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.