Goritsas et al. Journal of Medical Case Reports 2010, 4:9 http://www.jmedicalcasereports.com/content/4/1/9 On examination, his temperature was 36.8°C, heart rate was 68 beats/min, respiratory rate was 16 breaths/ min, and blood pressure was 140/70 mmHg. His lungs were clear to auscultation bilaterally. His abdomen was neither tender nor distended, and his liver was palpable 4 cm below the costal margin. The findings on the rest of the examination procedures were normal, as was his echocardiogram. Laboratory investigation showed the following data: white blood cell count, 10.7 × 109/L (with 66% neutrophils); hemoglobin, 11.4 g/dL; mean cell volume, 93.9fl; platelet count, 437 × 109/L; erythro- cyte sedimentation rate, 92 mm/1 h; C-reactive protein, 8.8 mg/dl; creatinine, 0.9 mg/dL; serum urea nitrogen, 34 mg/dL; aspartate aminotransferase, 781 U/L; alanine aminotransferase, 512 U/L; alkaline phosphatase, 322 U/ L; albumin, 3.1 g/dL; a1-antitrypsin, 184 mg/dL; IgG, 1980 mg/dl; IgA, 413 mg/dl; and IgM, 55.6 mg/dl. A urine analysis was negative for glycosuria and protei- nuria, while urine microscopy revealed 3 to 5 red blood cells per high power field and the presence of occasional dysmorphic red blood cells. The tests for hepatitis B, hepatitis C, hepatitis A and human immunodeficiency viruses all returned negative results. The results from the immunologic assays denoted the presence of ANCA against proteinase 3, which was confirmed by both immunofluorescence assay and Elisa anti-proteinase 3 antibodies. At the same time, tests for antinuclear antibodies, anti-smooth mus- cle antibodies and anti-liver kidney microsomal antibo- dies were all negative. An upper abdomen CT scan also yielded normal results. A biopsy of our patient’s nasal mucosa did not reveal any changes that were compatible with necrotizing vascu- litis. A complete ophthalmologic examination was also performed and was negative for ocular abnormalities. Our patient’s liver function tests kept worsening and his transa- minase levels reached a 15-fold increase within a week fol- lowing admission. However, his bilirubin remained within normal values (1.2 mg/dl). A fine needle biopsy of his pul- monary lesions was obtained and the histological findings were compatible with Wegener’s granulomatosis. The characteristic pathological features were irregular areas of necrosis surrounded by inflammatory granulation tissue (Figure 1) and neutrophilic microabscesses were sur- rounded by a granulomatous reaction with an occasional multinucleate Langhan’s giant cell (Figure 2). A liver biopsy was also performed, and histological evaluation revealed mild non-specific lobular hepatitis characterized by randomly distributed focal hepatocellular necrosis with collections of neutrophils and lymphocytes. A few apopto- tic cells and moderate sinusoidal reaction were also observed. A large number of liver cells exhibited periodic acid Schiff-positive Lafora-like inclusions. Occasional por- tal tracts showed mild leucocytic infiltrations (Figure 3). Page 2 of 5 Figure 1 Lung biopsy (fine needle biopsy), periodic acid Schiff stain ×400 magnification. Our patient later received a combined treatment of 1 mg/kg/day prednisone and 2 mg/kg/day of cyclopho- sphamide. Our patient’s status gradually improved with a remission of both the chest X-ray findings and the liver function studies 25 days after the initiation of treat- ment (Figure 4). After all signs of active disease had dis- appeared, a gradual tapering of corticosteroid and cyclophosphamide therapy was initiated. Eighteen months after his hospitalization, the patient is free of symptoms, with normal results for liver function tests and without any sign of relapse. Discussion Our patient had a long history of rhinitis that may represent a slowly progressive mild Wegener’s granulo- matosis. The main onset of symptoms was the acute Figure 2 Lung biopsy (fine needle biopsy), hematoxylin and eosin stain ×400 magnification.