Portal hypertension as a clinical manifestation of hepatic lesions in sarcoidosis

: The paper is devoted to differential diagnostics of portal hypertension (PH) in view of frequent diagnostic mistakes (10-15%) associated with this pathology. PH syndrome is usually interpreted by practitioners as a clinical manifestation of liver cirrhosis even if it is not necessarily related to the involvement of this organ. In patients with hepatic pathology, PH is likely to develop not only in case of fibrotic transformation and false lobule formation but also in case of disturbed blood flow in the portal vein, e.g. when granulomatous lesion of parenchyma takes place. Pathophysiological mechanisms and clinical peculiarities of intra-, sub-, suprahepatic and mixed forms of this condition are discussed. Etiological factors leading to the development of PH syndrome are considered. An original observation is presented of a patient with generalized sarcoidosis and histologically confirmed thoracic lymph nodes, spleen, lungs, liver; moderate granulomatous hepatitis, septal fibrosis (Knodel histology activity index 1-1-3-3- (8 scores)), PH, grade 1 oesophageal varicosis, splenomegaly, splenorenal anastomoses. The involvement of liver in the pathological process did not exceed 20%. Clinical, laboratory and instrumental studies rarely reveal manifestations of these changes. The diagnosis is verified histologically (discovery of granulomas formed by epithelial and giant multinuclear cells without signs of caseous necrosis. The reported case is distinctive for the development of PH in association of multiple giant heatic granulomas preventing the normal blood flow.