Budd-Chiari syndrome causes
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Causes
- Budd-Chiari syndrome is associated with a wide range of etiologies.
- On the basis of underlying cause Budd- Chairi can be:
- Primary (75%): thrombosis of the hepatic vein
- Secondary (25%): invasion/compression of the hepatic vein by an outside structure like(e.g. a tumor, abscess or cysts)
Causes include:
- Myeloproliferative disorders:
- Myeloproliferative and other hemotologic abnormalities are one of the most common causes of Budd Chiari Syndrome.
- V617F mutation in Janus tyrosine kinase-2 (JAK2) is found in 80% of patients with polycythemia vera and 50% of patients with essential thrombocythemia or idiopathic myelofibrosis.Budd Chiari syndrome patients that test negative for this mutation should have bone marrow biopsy performed.
- Other associated hematologic causes include:
- Paroxysmal nocturnal hemoglobinuria
- Antiphospholipid syndrome
- factor V leiden mutation
- prothrombin gene mutation
- methylene tetrahydrofolate reductase gene mutation
- Malignancy
- Malignancy is commonly associated with compression or invasion of vessels and hypercoagulable state.
- Budd-Chiari is commonly associated with
- hepatocellular carcinoma(associated with membranous obstruction of inferior vena cava)
- adrenal gland or kidney malignancy
- right atrial sarcoma
- pancreatic cancer
- lung carcinoma
- gastric carcinoma
- Infections and benign liver lesions
- Cause extrinsic compression of inferior venacava. May be associated with hypercoagylable state. These lesions include:
- hepatic cysts and abscesses
- hepatic adenoma
- hepatic mucinous cystic neoplasm (cystadenoma)
- syphilitic gumma
- invasive aspergillosis
- zygomycosis (mucormycosis)
- aortic aneurysm
- Cause extrinsic compression of inferior venacava. May be associated with hypercoagylable state. These lesions include:
- Oral contraceptives and pregnancy
- Hypercoagulable state in women using oral contraceptives (for more than two weeks), pregnant , or those who have delivered a child within the previous two months accounts for nearly 20 percent of cases of the Budd-Chiari syndrome
- Other hypercoagulable states
Hypercoagulable conditions associated with Budd-Chiari include: ●G1691A factor V (Leiden) gene mutation associated with activated protein C resistance ●G20210A factor II gene mutation ●Antiphospholipid syndrome ●Antithrombin deficiency ●Protein C deficiency ●Protein S deficiency ●Paroxysmal nocturnal hemoglobinuria
- Behçet's syndrome
- Vasculitis in Behçet's syndrome can predispose to thrombosis.
- Membranous webs are usually are found near the entrance of the right hepatic vein into the inferior vena cava, may be due to a congenital anomaly or a myeloproliferative disease.
- More common in patients from South Africa, India, and Asia. Potentially treatable cause of Budd-chiari.
- Miscellaneous
- Miscellaneous causes of the Budd-Chiari syndrome include:
- Systemic lupus erythematosus
- Mixed-connective tissue disease
- Sjögren's syndrome
- inflammatory bowel disease
- hypereosinophilic syndrome
- idiopathic granulomatous venulitis
- sarcoidosis
- protein-losing enteropathy
- minimal change nephrotic syndrome
- neurofibromatosis
- alpha-1 antitrypsin deficiency
- trauma
- Miscellaneous causes of the Budd-Chiari syndrome include:
- Idiopathic
- Upto 20 percent of cases of the Budd-Chiari syndrome are idiopathic.