Published online Nov 6, 2018. doi: 10.12998/wjcc.v6.i13.688
Peer-review started: July 11, 2018
First decision: August 3, 2018
Revised: August 7, 2018
Accepted: October 9, 2018
Article in press: October 9, 2018
Published online: November 6, 2018
Processing time: 118 Days and 7 Hours
A 48 year-old Chinese woman with polyarthritis, irregular fever and trichomadesis was also found to have splenomegaly during physical examination.
The diagnosis of noncirrhotic portal hypertension (NCPH) was made in the absence of cirrhosis and was associated with systemic lupus erythematosus (SLE), which was diagnosed based on polyarthritis, pancytopenia, reduced complement 3, multiple positive autoantibodies, a positive Coomb’s test and protein in urine.
Portal hypertension etiology, such as cirrhosis and other obstructive diseases, were considered.
Reduced complement 3, multiple positive autoantibodies, positive Coomb’s test and protein in urine.
Abdominal ultrasonography (USG) and magnetic resonance imaging (MRI) demonstrated splenomegaly and portal hypertension.
The patient was treated with methylprednisolone, hydroxychloroquine and metoprolol tartrate to reduce SLE disease activity, control portal hypertension and its complications. In severe conditions, such as variceal bleeding, endoscopic variceal ligation or transjugular intrahepatic portosystemic shunting is recommended.
NCPH is commonly called nodular regenerative hyperplasia or idiopathic portal hypertension in previous reports due to the lack of standardized nomenclature and diagnostic criteria. Most reported cases of NCPH associated with SLE had severe complications in late stages. In our patient, who was in the early stage of NCPH, splenomegaly and portal hypertension were identified upon physical examination and diagnostic imaging. MRI, which is more sensitive than CT scan or USG, is an alternative non-invasive diagnostic technique that can both reveal signs of portal hypertension as well as differentiate features commonly seen in cirrhosis.
NCPH, which is characterized by portal hypertension, splenomegaly and hypersplenism, should be considered in SLE patients with clinical manifestations of portal hypertension in the absence of cirrhosis. MRI can be performed to rule out cirrhosis. The recognition of early clinical presentation and the associated risk factors of NCPH can contribute to a reduction in missed and delayed diagnosis of this disorder.