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Hepatic involvement in Wegener's granulomatosis: a case report.

Goritsas C, Paissios NP, Trigidou R, Delladetsima J - J Med Case Rep (2010)

Bottom Line: A liver biopsy demonstrated the presence of mild non-specific lobular hepatitis and periodic acid-Schiff positive Lafora-like inclusions in a large number of his liver cells.The patient was treated with prednisone and cyclophosphamide, which was followed by subsequent remissions of chest X-ray findings and liver function studies.We favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Sotiria General Hospital, 152 Mesogeion Avenue, Athens, 11527, Greece.

ABSTRACT

Introduction: We report the case of a 58-year-old Caucasian Greek man who presented with dry cough, fever, bilateral alveolar infiltrates and acute hepatitis.

Case presentation: After a lung biopsy, the patient was diagnosed with Wegener's granulomatosis. The diagnosis was supported by the presence of anti-proteinase-3 anti-neutrophil cytoplasmic antibodies. A liver biopsy demonstrated the presence of mild non-specific lobular hepatitis and periodic acid-Schiff positive Lafora-like inclusions in a large number of his liver cells. The patient was treated with prednisone and cyclophosphamide, which was followed by subsequent remissions of chest X-ray findings and liver function studies.

Conclusion: What makes this case worth reporting is the coexistence of liver inflammation with a biochemical profile of severe anicteric non-viral, non-drug induced hepatitis coinciding with the diagnosis of Wegener's granulomatosis. Our paper may be the first report of hepatic involvement in a patient diagnosed with Wegener's granulomatosis. The aetiological link between the two diseases is supported by the reversion of hepatitis after the immunosuppression of Wegener's granulomatosis. We favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis.

No MeSH data available.


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Lung biopsy (fine needle biopsy), periodic acid Schiff stain ×400 magnification.
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Figure 1: Lung biopsy (fine needle biopsy), periodic acid Schiff stain ×400 magnification.

Mentions: A biopsy of our patient's nasal mucosa did not reveal any changes that were compatible with necrotizing vasculitis. A complete ophthalmologic examination was also performed and was negative for ocular abnormalities. Our patient's liver function tests kept worsening and his transaminase levels reached a 15-fold increase within a week following admission. However, his bilirubin remained within normal values (1.2 mg/dl). A fine needle biopsy of his pulmonary 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 surrounded 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 apoptotic cells and moderate sinusoidal reaction were also observed. A large number of liver cells exhibited periodic acid Schiff-positive Lafora-like inclusions. Occasional portal tracts showed mild leucocytic infiltrations (Figure 3).


Hepatic involvement in Wegener's granulomatosis: a case report.

Goritsas C, Paissios NP, Trigidou R, Delladetsima J - J Med Case Rep (2010)

Lung biopsy (fine needle biopsy), periodic acid Schiff stain ×400 magnification.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2821396&req=5

Figure 1: Lung biopsy (fine needle biopsy), periodic acid Schiff stain ×400 magnification.
Mentions: A biopsy of our patient's nasal mucosa did not reveal any changes that were compatible with necrotizing vasculitis. A complete ophthalmologic examination was also performed and was negative for ocular abnormalities. Our patient's liver function tests kept worsening and his transaminase levels reached a 15-fold increase within a week following admission. However, his bilirubin remained within normal values (1.2 mg/dl). A fine needle biopsy of his pulmonary 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 surrounded 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 apoptotic cells and moderate sinusoidal reaction were also observed. A large number of liver cells exhibited periodic acid Schiff-positive Lafora-like inclusions. Occasional portal tracts showed mild leucocytic infiltrations (Figure 3).

Bottom Line: A liver biopsy demonstrated the presence of mild non-specific lobular hepatitis and periodic acid-Schiff positive Lafora-like inclusions in a large number of his liver cells.The patient was treated with prednisone and cyclophosphamide, which was followed by subsequent remissions of chest X-ray findings and liver function studies.We favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Sotiria General Hospital, 152 Mesogeion Avenue, Athens, 11527, Greece.

ABSTRACT

Introduction: We report the case of a 58-year-old Caucasian Greek man who presented with dry cough, fever, bilateral alveolar infiltrates and acute hepatitis.

Case presentation: After a lung biopsy, the patient was diagnosed with Wegener's granulomatosis. The diagnosis was supported by the presence of anti-proteinase-3 anti-neutrophil cytoplasmic antibodies. A liver biopsy demonstrated the presence of mild non-specific lobular hepatitis and periodic acid-Schiff positive Lafora-like inclusions in a large number of his liver cells. The patient was treated with prednisone and cyclophosphamide, which was followed by subsequent remissions of chest X-ray findings and liver function studies.

Conclusion: What makes this case worth reporting is the coexistence of liver inflammation with a biochemical profile of severe anicteric non-viral, non-drug induced hepatitis coinciding with the diagnosis of Wegener's granulomatosis. Our paper may be the first report of hepatic involvement in a patient diagnosed with Wegener's granulomatosis. The aetiological link between the two diseases is supported by the reversion of hepatitis after the immunosuppression of Wegener's granulomatosis. We favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis.

No MeSH data available.


Related in: MedlinePlus