Nodular regenerative hyperplasia explained

Nodular regenerative hyperplasia
Synonyms:Nodular regenerative hyperplasia of the liver
Complications:Portal hypertension

Nodular regenerative hyperplasia (NRH) is a rare liver disease, characterised by the growth of nodules within the liver, resulting in liver hyperplasia. While in many cases it is asymptomatic and thus goes undetected – or is only discovered incidentally while investigating some other medical condition – in some people it results in non-cirrhotic portal hypertension (NCPH). NCPH is generally less severe than the much more common portal hypertension due to cirrhosis. Complications of NCPH can include jaundice, ascites, splenomegaly, and bleeding esophageal varices. Most people with NRH retain normal liver function – even among the subset who go on to develop NCPH – and liver failure in NRH is uncommon. Only a small proportion of NRH patients will ever require liver transplantation.

The causes of NRH are poorly understood, although it is believed to be related to abnormal blood flow in the small vessels of the liver. NRH development may be preceded by an attack of sinusoidal obstructive syndrome.[1] Some cases are known to be caused by treatment with azathioprine, an immunosuppressant drug commonly used to prevent rejection in organ transplantation (especially of the kidneys), and to treat various auto-immune disorders.[2] [3] Other drug treatments, such as with oxaliplatin, can also contribute. Medical conditions associated with NRH include cystic fibrosis,[1] common variable immunodeficiency[1] [4] and chronic granulomatous disease. Cases may also arise after solid-organ transplantation.[1]

Notes and References

  1. Book: LiverTox: Clinical and Research Information on Drug-Induced Liver Injury. Nodular Regenerative Hyperplasia. National Institute of Diabetes and Digestive and Kidney Diseases. Bethesda, Maryland. 4 May 2019. https://www.ncbi.nlm.nih.gov/books/NBK548385/. National Library of Medicine. 31689031. 1 July 2024.
  2. Daniel F . Azathioprine induced nodular regenerative hyperplasia in IBD patients . Gastroenterol. Clin. Biol. . 29 . 5 . 600–3 . May 2005 . 15980758 . 10.1016/S0399-8320(05)82136-0. vanc . Cadranel JF . Seksik P . 3 . Cazier . Alain . Duong Van Huyen . Jean-Paul . Ziol . Marianne . Coutarel . Pierre . Loison . Patrick . Jian . Raymond.
  3. Vernier-Massouille G . Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine . Gut . 56 . 10 . 1404–9 . October 2007 . 17504943 . 10.1136/gut.2006.114363 . vanc . Cosnes J . Lemann M . 3 . Marteau . P . Reinisch . W . Laharie . D . Cadiot . G . Bouhnik . Y . De Vos . M . 2000290.
  4. Ganapathi L, Cochran RL, Robbins GK, Barmettler S, Holland SM, Ababneh EI. Case 20-2024: A 73-Year-Old Man with Recurrent Fever and Liver Lesions. The New England Journal of Medicine. 390. 10.1056/NEJMcpc2309383. 24. 2309-2319. 2024. 38924735.