A 23-year-old Caucasian female presented with a 3 week history of right upper quadrant pain, fevers, bilateral ankle pain and swelling with an accompanying rash. Her past medical history consisted of a previous hospital admission 3 months earlier due to an acute inflammatory polyarthritis associated with fever. During that admission the patient had developed a maculopapular rash over the trunk and limbs. Skin biopsies of the right thigh were taken which showed leukocytoclastic vasculitis. Symptoms improved after commencement of prednisone and the patient was discharged.
When the patient represented she had an elevated C-reactive protein 168 mg/L (normal: <12), erythrocyte sedimentation rate 76 mm/hr (0-12) and alkaline phosphatase 162 U/L (30-130). All her other blood results were normal. Rheumatoid Factor, ANCA and HLA B27 were negative as were blood cultures and viral serologies. Hepatobiliary scintigraphy was requested to investigate hepatobiliary function (Figures , ). The study was performed with 220 MBq (5.9 mCi) Tc-99m disofenin. The vascular phase appeared normal. The parenchymal phase showed an enlarged left lobe of the liver. Bile production was normal with rapid appearance in the intrahepatic ducts and normal passage of bile into the bowel. However, the gallbladder filled slowly during the study. An outpatient computed tomography scan which had been performed prior to admission showed a thickened gallbladder wall with surrounding fluid but no evidence of stones or duct dilatation (Figure ).
Figure 1 Hepatobiliary scintigraphy of a 23-year-old white female with a three week history of right upper quadrant pain after injection of 220 MBq (5.9 mCi) Tc-99m disofenin. Anterior dynamic views of hepatobiliary scintigraphy showing normal bile production (more ...)
Figure 2 Hepatobiliary scintigraphy of a 23-year-old white female with a three week history of right upper quadrant pain after injection of 220 MBq (5.9 mCi) Tc-99m disofenin. Anterior delayed static view of hepatobiliary scintigraphy demonstrating progressive (more ...)
Select axial image of abdominal computed tomography of a 23-year-old white female with a three week history of right upper quadrant pain showing a thickened gallbladder wall (arrow) with surrounding fluid.
Based on these findings and the clinical picture a laparoscopic cholecystectomy was performed. The postoperative recovery was unremarkable and the patient was discharged two days later and subsequently had no further right upper quadrant pain. Clinical follow-up one month later showed that she was still free of abdominal pain but had developed symptoms consistent with mononeuritis multiplex. Polyarteritis nodosa was considered the most likely diagnosis clinically.
Macroscopic examination of the gallbladder showed no gross abnormality. However, microscopic examination revealed a striking vasculitis affecting small to medium sized vessels, predominantly arteries. There was fibrinoid necrosis of many vessels associated with an inflammatory cell infiltrate, composed predominantly of lymphocytes with very occasional eosinophils (Figure ). Almost every vessel in the histological sections was affected and some showed thrombotic occlusion. The findings were consistent with polyarteritis nodosa.
Figure 4 Histopathology of the gallbladder of a 23-year-old white female with a three week history of right upper quadrant pain showing fibrinoid necrosis of a small artery, associated with lymphocytes and macrophages infiltrating the wall. Occasional eosinophils (more ...)