Goritsas et al. Journal of Medical Case Reports 2010, 4:9 http://www.jmedicalcasereports.com/content/4/1/9 although virtually any organ can be involved with vascu- litis, granulomas or both, gastrointestinal involvement is very uncommon and the liver is extremely rarely affected [9]. Only isolated cases associated with primary biliary cirrhosis and hepatic granulomas are reported. Shah et al. [10], reported a case of Wegener’s granulo- matosis with perivascular and periportal granuloma with Langhan’s giant cells. Boissy et al. [11] reported a case of Wegener’s granulomatosis in which portal inflamma- tory granuloma and vasculitis with portal tract fibrosis and venous lesions were found. Zen et al. [12] reported a case of incomplete septal cirrhosis associated with Wegener’s granulomatosis and Sjögern’s syndrome. A very rare form of hepatotoxicity was reported only twice in the literature [13,14]. Cyclophosphamide-induced noncaseating granuloma- tous hepatitis in Wegener’s granulomatosis was also ruled out from the start in this case, because hepatitis was apparent well before the initiation of Wegener’s granulomatosis treatment with cyclophosphamide. In our case, neither vasculitis of the portal tracts nor gran- ulomas that would have revealed a possible necrotizing angiitis (Wegener’s granulomatosis type) of the liver was detected. Nevertheless, this possibility cannot be excluded, since only seven portal tracts were examined. The histological findings of mild hepatic necrosis in aci- nar zones 3 and 2 with concomitant mild inflammation of predominantly neutrophils and mononuclear cells with portal tracts and the nearly normal periportal par- enchyma are compatible with vascular damage resulting in the hypoperfusion of the liver. This hypothesis is reinforced by the immediate response of liver function tests after immunosuppressive therapy with prednisone and cyclophosphamide was initiated. The histological finding of ground-glass inclusions within hepatocytes is common in HBV infection and has also been reported in drug-induced liver damage (cyana- mide, disulfiram), Lafora’s disease (myoclonus epilepsy), type IV glycogenosis, fibrinogen storage disease, and acute veno-occlusive disease [15]. There have also been several reports of immunosuppressed patients on numerous medications with ground-glass hepatocellular inclusions [16]. Our patient had normal serum levels of a1-antithrypsin, was HBV seronegative, had no history of drug abuse, and did not demonstrate clinical findings of myoclonus epilepsy or seizures under a normal elec- troencephalogram test. Recently, Lefkowitch et al. [17] suggested that ground- glass inclusions within hepatocytes are the result of dis- turbed glycogen metabolism. Whether this cytoplasmic change in our patient was an incidental finding due to his disturbed glycogen metabolism or was related to the liver involvement of Wegener’s granulomatosis cannot be ascertained. In this case, liver biopsy showed non- Page 4 of 5 specific hepatitis with mild lobular activity. The absence of histological findings compatible with Wegener’s gran- ulomatosis could be attributed to sampling, since liver damage in Wegener’s granulomatosis is expected to be focal and not diffused. Conclusion To the best of our knowledge, this report presents one of the first cases of hepatic involvement in a Wegener’s granulomatosis patient. The aetiological link between the two diseases is supported by the reversion of hepati- tis after the immunosuppression of Wegener’s granulo- matosis, and we favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis. The simultaneous finding of Lafora body-like inclusions in our patient’s hepatocytes makes the case even more intriguing, even when an incidental coinci- dence cannot be ruled out. Consent Written informed consent was obtained from the patient for publication of this case report and any accompany- ing images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Author details 1Department of Internal Medicine, Sotiria General Hospital, 152 Mesogeion Avenue, Athens, 11527, Greece. 2Department of Pathology, Sotiria General Hospital, 152 Mesogeion Avenue, Athens, 11527, Greece. 3Department of Pathology, Laiko University Hospital, Ag Thoma, Athens, 11527, Greece. Authors’ contributions GC and NP were the patient’s physicians. RT performed the histological examination of the nasal mucosa and the lung biopsy. JD and RT performed the histological examination of the liver biopsy. 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