Polyarteritis nodosa in a patient with type 1 autoimmune hepatitis.

Polyarteritis nodosa is a systemic necrotizing vasculitis that affects small- and medium-sized arteries. Liver involvement in patients with polyarteritis nodosa has been described, and ranges from asymptomatic elevation of aminotransferases to hepatic aneurysm rupture. We describe the case of a patient with type 1 autoimmune hepatitis and compensated liver cirrhosis who developed classic polyarteritis nodosa, complicated with cytomegalovirus and repeated urinary tract infections. After a long bedridden hospitalization, the patient's condition was stabilized. She is currently in good health, with well-controlled blood pressure, and stable kidney and liver function. To our knowledge, this is the first case report in the literature with concurrent appearance of both diseases.

Spur cells and spur cell anemia in hospitalized patients with advanced liver disease: Incidence and correlation with disease severity and survival.

AIM: Spur cell anemia (SCA) is a form of acquired hemolytic anemia seen in patients with advanced cirrhosis and particularly in patients with alcoholic cirrhosis. The aim of the present study was to evaluate the incidence of spur cells and spur cell anemia in patients with advanced liver disease and to correlate the presence of spur cell anemia with survival. METHODS: During a 33-month period, all patients with advanced cirrhosis (Child-Pugh-Turcott score [CPT]>/=7] who were hospitalized in our department for various reasons were included in this study. RESULTS: A total of 54 patients were included in the study; 26 patients had spur cells on peripheral blood smear (median 4, range 1-14). Patients with spur cells had more advanced liver disease compared with those without spur cells (CPT score, P < 0.0001 and MELD score, P < 0.0001), lower hemoglobin levels (P < 0.0001), higher bilirubin levels (total/unconjugated, P < 0.0001), higher reticulocyte count (P < 0.0001) and more prolonged international normalized ratio (INR; P < 0.0001). Patients with 5% spur cells or more had more advanced disease compared with patients with 1-4% spur cells (CPT score, P = 0.004 and MELD score, P = 0.003), lower hemoglobin levels (P = 0.033), more elevated bilirubin levels (total/unconjugated, P = 0.006) and more prolonged INR (P = 0.04). Three-month survival was lower in patients with spur cells compared with patients without spur cells (P = 0.017 and P = 0.104, respectively). Patients with 5% spur cells or more had lower 3-month survival compared with those with 1-4% spur cells (P = 0.014). CONCLUSION: Presence of spur cells in patients with advanced cirrhosis is not always accompanied by spur cell anemia. The presence of 5% spur cells or more and/or hemolytic anemia is associated with poor prognosis and these patients might have to be given priority for liver transplantation.

Sarcoidosis-induced pericarditis in a patient with portopulmonary hypertension: a case report.

Portopulmonary hypertension is a rare and severe complication of patients with cirrhosis. Sarcoidosis, a disease of unknown etiology, is also a cause of pulonary hypertension and right heart dysfunction. We report the case of a 51-year-old male patient, suffering from cirrhosis due to Wilson's disease, portal hypertension and pulmonary hypertension (PH), who developed severe pericarditis. Wilson's disease was diagnosed 8 years before his last admission to our hospital and was being successfully treated with D-penicillamine. PH was recognized 2 years before admission and being treated with bosentan. The patient complained for dyspnea at rest and the 2D echocardiogram revealed a significant amount of pericardial fluid. All other causes of acute pericarditis were excluded and his laboratory, imaging and histopathological investigation showed evidence of sarcoidosis. He underwent a therapy with corticosteroids (methylprednisolone) and his follow-up examination showed remarkable decrease of the levels of mean pulmonary artery pressure and pericardial fluid.

Late onset ulcerative colitis complicating a patient with Budd-Chiari syndrome: a case report and review of the literature.

We report a case of a 33-year-old female patient with Budd-Chiari syndrome because of polycythemia vera. A transjugular intrahepatic portal-systemic shunt was performed because of refractory ascites 7 months after diagnosis. She had a stable hepatic function receiving anticoagulants until 3 years later when she presented with bloody diarrheas, liver function deterioration with prolonged prothrombin time and hypoalbuminemia, encephalopathy, and ascites. Colonoscopy revealed ulcerative pancolitis and the patient was treated with corticosteroids and antibiotics. Hepatic function was stabilized in parallel to controlling ulcerative colitis and the patient is in good health until now receiving maintenance therapy for ulcerative colitis and anticoagulants for Budd-Chiari syndrome.

Inflammatory pseudotumor of the liver successfully treated with nonsteroidal anti-inflammatory drugs: a challenge diagnosis for one not so rare entity.

Inflammatory pseudotumor of the liver is a rare, benign lesion characterized by a well-circumscribed mass of chronic inflammatory cell infiltration and proliferating fibrous tissue. Its etiology remains unclear, although inflammatory processes have been proposed. It is often misdiagnosed as a malignant tumor, and the management has been traditionally surgical. We report the case of a 16-year-old boy who was referred from another hospital with a fever of >38 degrees C with rigor and right upper quadrant pain which he had suffered from for 5 days. The ultrasonographic computed tomography and MRI findings were not diagnostic, and we performed a needle biopsy from the lesion that was consistent with inflammatory pseudotumor (of liver, mixed fibrous tissue and chronic inflammatory cell infiltration). The patient was treated with nonsteroidal anti-inflammatory drugs and had an uneventful clinical course. During follow-up, the lesion subsequently shrank to completely vanish 1 year later.