Nodular regenerative hyperplasia: Evolving concepts on underdiagnosed cause of portal hypertension

Table 1 Diseases and conditions coexisting with nodular regenerative hyperplasia¹

Disease	No. of cases	Ref.
Pulmonary hypertension	32	[11,24-44]
Rheumatoid arthritis/Felty’s syndrome	30	[31,41,44-55]
Human immunodeficiency virus infection	20	[21,56-64]
Lupus erythematosus	12	[27,40,65-70]
Crohn’s disease/ulcerative colitis	13	[17,28,56,71-76]
Celiac disease	9	[76-80]
Scleroderma/CREST	7	[25,32,81-85]
Antiphospholipid syndrome	11	[78,79,86-89]
Sarcoidosis	9	[90]
Post-transplant	18	[16,37,91-99]
Extrahepatic cancers	26	[26,59,100-104]
Lymphomas	12	[29,53,81,105-113]
Macroglobulinemia	5	[114,115]
Mixed cryoglobulinemia	3	[25,116,117]
ITP/aplastic anemia	4	[3,12,118,119]
Primary biliary cirrhosis	6	[25,82,120-122]
Krabbe disease	4	[123,124]
Congenital absence of portal vein	4	[15,125,126]
Portal vein thrombosis	2	[14,127]
Familial pulmonary fibrosis	4	[128]
Chronic glomerulonephritis	5	[51,117,129-131]
Cystinosis	2	[24]
Myasthenia	2	[132,133]
Polyarteritis nodosa	2	[134,135]
Common variable immunodeficiency syndrome	2	[136,137]
Turner’s syndrome	2	[138,139]
Castleman’s disease	2	[78,140]
Idiopathic eosinophilic syndrome	2	[141,142]

¹Only associations reported in the literature twice or more have been shown. ITP: Idiopathic thrombocytopenia. CREST: Calcinosis, raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.