Nodular Regenerative Hyperplasia of the Liver in Rheumatic Disease: Cases and Review of the Literature

Nodular regenerative hyperplasia (NRH) is a rare disease that is characterized by benign transformation of the hepatic parenchyma into small nodules with little to no fibrosis. Nodular regenerative hyperplasia is a cause of noncirrhotic portal hypertension. Symptoms can range from asymptomatic disease to more serious complications of portal hypertension such as esophageal varices and ascites. Nodular regenerative hyperplasia has been described in association with a variety of different rheumatologic, hematologic, and oncologic diseases, as well as in immune deficiency states and with exposures to certain toxins. Diagnosis is made by histology, and the treatment involves addressing the underlying disease. The first description of this rare disease was actually described in a patient with rheumatoid arthritis, neutropenia, and splenomegaly (Felty’s Syndrome). We describe 2 cases of NRH associated with underlying rheumatic disorders, in one of which NRH was actually the presenting feature of the patient’s underlying autoimmune condition. Subsequently, we provide a brief review of the literature of NRH in autoimmune disease with respect to epidemiology, cause, clinical manifestations, diagnosis, and treatment.

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Noncirrhotic portal hypertension and nodular regenerative hyperplasia of the liver in dogs with mucopolysaccharidosis type I

Hepatology ◽

10.1002/hep.510280214 ◽

1998 ◽

Vol 28 (2) ◽

pp. 385-390 ◽

Cited By ~ 18

Author(s):

Michael F. McEntee ◽

Kathy N. Wright ◽

Ian Wanless ◽

Robert DeNovo ◽

John F. Schneider ◽

...

Keyword(s):

Portal Hypertension ◽

Nodular Regenerative Hyperplasia ◽

Type I ◽

Mucopolysaccharidosis Type ◽

Mucopolysaccharidosis Type I ◽

Noncirrhotic Portal Hypertension

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Noncirrhotic Portal Hypertension in HIV-Infected Patients: A Case Control Evaluation and Review of the Literature

AIDS Patient Care and STDs ◽

10.1089/apc.2010.0160 ◽

2010 ◽

Vol 24 (11) ◽

pp. 697-703 ◽

Cited By ~ 13

Author(s):

Miriam Cesari ◽

Monica Schiavini ◽

Giulia Marchetti ◽

Ilaria Caramma ◽

Massimiliano Ortu ◽

...

Keyword(s):

Portal Hypertension ◽

Case Control ◽

Review Of The Literature ◽

Noncirrhotic Portal Hypertension ◽

Control Evaluation

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Portal Hypertension, Nodular Regenerative Hyperplasia of the Liver, and Obstructive Portal Venopathy due to Metastatic Breast Cancer

Case Reports in Pathology ◽

10.1155/2013/826284 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Andrew T. Turk ◽

Matthias J. Szabolcs ◽

Jay H. Lefkowitch

Keyword(s):

Breast Cancer ◽

Portal Hypertension ◽

Breast Carcinoma ◽

Portal Vein ◽

Metastatic Breast ◽

Nodular Regenerative Hyperplasia ◽

Metastatic Breast Carcinoma ◽

Noncirrhotic Portal Hypertension ◽

Diagnostic Difficulties ◽

Recurrent Breast

Nodular regenerative hyperplasia (NRH) of the liver is associated with noncirrhotic portal hypertension, rheumatologic and hematologic disorders, administration of certain drugs, and other underlying conditions. This report describes a 64-year-old man with clinically presumed cirrhosis who presented to our institution with coffee-ground emesis, esophageal varices, ascites, and encephalopathy. Eleven years earlier he had been treated for breast cancer with mastectomy and chemo-radiotherapy. He died suddenly, and the autopsy showed no evidence of cirrhosis but instead demonstrated NRH with extensive emboli of recurrent breast carcinoma within the portal vein and its intrahepatic branches. Neoplastic occlusion of the portal vein as a cause of presinusoidal noncirrhotic portal hypertension has not previously been reported for metastatic breast carcinoma. This case highlights the importance of obstructive portal venopathy in the pathogenesis of NRH as well as the diagnostic difficulties that may be encountered in determining the cause of portal hypertension.

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DIAGNOSTIC CRITERIA FOR DISSEMINATED INTRAVASCULAR COAGULATION SYNDROME AND SEPSIS-INDUCED COAGULOPATHY

Pain anesthesia and intensive care ◽

10.25284/2519-2078.2(95).2021.238302 ◽

2021 ◽

pp. 25-38

Author(s):

S. O. Tarasenko ◽

S. O. Dubrov ◽

G. G. Suslov

Keyword(s):

Intensive Care ◽

Clinical Practice ◽

Disseminated Intravascular Coagulation ◽

Thrombus Formation ◽

Clinical Manifestations ◽

Underlying Disease ◽

Multiple Organ ◽

Review Of The Literature ◽

Intravascular Coagulation ◽

Current State

The clinical manifestations of disseminated intravascular coagulation syndrome (DIC) depend on the predominance of the sum of the vectors of hypercoagulation and hyperfibrinolysis and are strongly associated with the underlying disease, against which DIC is formed. The issue of understanding the complex pathogenesis, timely diagnosis of overt DIC and early manifestations of DIC remain an urgent challenge for intensive care physicians and leading specialized societies to study the problems of hemostasis and thrombus formation. This review of the literature analyzes the pathways of DIC development, the current state of the possibility of using diagnostic markers to detect DIC, especially in sepsis. The diagnosis of sepsis-induced coagulopathy against the background of the development of multiple organ failure is highlighted as a separate issue. Diagnostic scales are presented in the form of comparative tables for a more convenient perception of information, memorization and further implementation in clinical practice.

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Noncirrhotic Portal Hypertension due to Nodular Regenerative Hyperplasia Treated with Surgical Portacaval Shunt

Case Reports in Medicine ◽

10.1155/2012/965304 ◽

2012 ◽

Vol 2012 ◽

pp. 1-5 ◽

Cited By ~ 5

Author(s):

Lisa M. Louwers ◽

Jared Bortman ◽

Alan Koffron ◽

Veslav Stecevic ◽

Steven Cohn ◽

...

Keyword(s):

Portal Hypertension ◽

Lupus Erythematosus ◽

Beta Blockers ◽

Portacaval Shunt ◽

Nodular Regenerative Hyperplasia ◽

Noncirrhotic Portal Hypertension ◽

Parenchymal Liver ◽

Abdominal Mri ◽

Synthetic Function ◽

History Of

Nodular regenerative hyperplasia (NRH) is an uncommon condition, but an important cause of noncirrhotic intrahepatic portal hypertension (NCIPH), characterized by micronodules of regenerative hepatocytes throughout the liver without intervening fibrous septae. Herein, we present a case of a thirty-seven-year-old female with systemic lupus erythematosus (SLE) who was discovered to have significant esophageal varices on endoscopy for dyspepsia. Her labs revealed a slight elevation in the alkaline phosphatase and mild thrombocytopenia. Abdominal MRI revealed seven focal hepatic masses, splenomegaly, no ascites, and a patent portal vein. Ultrasound-guided core biopsy was reported as focal nodular hyperplasia. However, her varices persisted despite treatment with beta-blockers and four additional upper endoscopies with banding. She was subsequently referred for a surgical opinion. At that time, given her history of SLE, azathioprine use, and portal hypertension, suspicion for NRH was raised. Given her normal synthetic function and lack of parenchymal liver disease, the patient was offered surgical shunting. During shunt surgery, a liver wedge biopsy was also performed and this confirmed NRH. An upper endoscopy six weeks after shunting verified complete resolution of varices. Currently, fifteen months after surgery duplex ultrasonography demonstrates shunt patency and the patient is without recurrence of her portal hypertension.

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Nodular Regenerative Hyperplasia: A Rare Cause of Noncirrhotic Portal Hypertension in SLE Patients

The American Journal of Gastroenterology ◽

10.14309/00000434-201210001-01085 ◽

2012 ◽

Vol 107 ◽

pp. S437

Author(s):

Kathlynn Caguiat ◽

Marina Kaufman ◽

Vijaya Dasari ◽

Ayse Aytaman

Keyword(s):

Portal Hypertension ◽

Nodular Regenerative Hyperplasia ◽

Noncirrhotic Portal Hypertension

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Nodular regenerative hyperplasia of the liver: a cause of noncirrhotic portal hypertension

Cleveland Clinic Journal of Medicine ◽

10.3949/ccjm.51.3.559 ◽

1984 ◽

Vol 51 (3) ◽

pp. 559-564 ◽

Cited By ~ 1

Author(s):

W. R. Hart ◽

R. J. Tuthill

Keyword(s):

Portal Hypertension ◽

Nodular Regenerative Hyperplasia ◽

Noncirrhotic Portal Hypertension

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Nodular Regenerative Hyperplasia

Journal of Investigative Medicine High Impact Case Reports ◽

10.1177/2324709617690742 ◽

2017 ◽

Vol 5 (1) ◽

pp. 232470961769074

Author(s):

Mindy Lee ◽

Manhal Izzy ◽

Ashwin Akki ◽

Kathryn Tanaka ◽

Harmit Kalia

Keyword(s):

Portal Hypertension ◽

Pneumocystis Carinii ◽

Undetectable Viral Load ◽

Gastric Varices ◽

Nodular Regenerative Hyperplasia ◽

Noncirrhotic Portal Hypertension ◽

First Case ◽

Bleeding Prophylaxis ◽

Intrahepatic Portosystemic Shunt ◽

History Of

Introduction: Nodular regenerative hyperplasia (NRH) is a known etiology of noncirrhotic portal hypertension. Cases of biopsy-proven NRH in human immunodeficiency virus (HIV)–positive patients have been described. While these patients often have normal synthetic liver function, several reports described disease progression to liver failure. Case: We here present a 26-year-old woman with history of congenital HIV on antiretroviral therapy complicated by Pneumocystis carinii pneumonia at age 14. CD4 counts have been >300 with undetectable viral load. She was referred to our Hepatology service for evaluation of splenomegaly, elevated liver tests, and thrombocytopenia. On initial presentation, she reported easy bruising and gingival bleeding, and abdominal imaging showed evidence of portal hypertension without associated cirrhosis. Upper endoscopy was significant for large esophageal varices without bleeding stigmata. Liver biopsy showed minimal fibrosis around the portal areas without significant inflammation. The lobules showed focal zones of thin hepatocyte plates on reticulin stain with adjacent areas showing mild regenerative changes. The diagnosis of NRH was made and patient was placed on propranolol for variceal bleeding prophylaxis. Two years later, the patient presented with bleeding gastric varices warranting transjugular intrahepatic portosystemic shunt. Postprocedure course was complicated by mild encephalopathy. Subsequent magnetic resonance imaging showed a 1.7 × 1.3 cm lesion suggestive of hepatocellular carcinoma (HCC). The patient was deemed to be a candidate for liver transplantation, and she is now delisted due to ongoing pregnancy. Conclusion: This report describes the first case of HCC in an HIV patient with NRH. The possible association of NRH with HCC warrants further investigation.

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