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.[1][2][3][4]
  • 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
  • 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
  • Idiopathic
    • Upto 20 percent of cases of the Budd-Chiari syndrome are idiopathic.

References

  1. Afredj N, Guessab N, Nani A, Faraoun SA, Ouled Cheikh I, Kerbouche R, Hannoun D, Amir ZC, Ait Kaci H, Bentabak K, Plessier A, Valla DC, Cazals-Hatem V, Denninger MH, Boucekkine T, Debzi N (2015). "Aetiological factors of Budd-Chiari syndrome in Algeria". World J Hepatol. 7 (6): 903–9. doi:10.4254/wjh.v7.i6.903. PMC 4411532. PMID 25937867.
  2. Lin M, Zhang F, Wang Y, Zhang B, Zhang W, Zou X, Zhang M, Zhuge Y (2017). "Liver cirrhosis caused by chronic Budd-Chiari syndrome". Medicine (Baltimore). 96 (34): e7425. doi:10.1097/MD.0000000000007425. PMC 5571988. PMID 28834866.
  3. Martens P, Nevens F (2015). "Budd-Chiari syndrome". United European Gastroenterol J. 3 (6): 489–500. doi:10.1177/2050640615582293. PMC 4669515. PMID 26668741.
  4. Merz WM, Rüland AM, Hippe V, Poetzsch B, Meyer C, Pollok JM, Gembruch U, Trebicka J (2016). "Pregnancy in Budd-Chiari Syndrome: Case Report and Proposed Risk Score". Medicine (Baltimore). 95 (22): e3817. doi:10.1097/MD.0000000000003817. PMC 4900734. PMID 27258526.


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