Drug-induced lupus-like syndrome associated with severe autoimmune hepatitis

Abstract

Atorvastatin and other members of the statin family are widely used for the treatment of hypercholesterolaemia in order to reduce the risk of atherosclerosis and cardiovascular disease. Atorvastatin-induced adverse events are mostly mild and only a few cases of lupus-like syndrome or severe acute hepatitis have been documented. In this case report we describe a patient who developed an atorvastatin-induced severe autoimmune hepatitis. In addition, this patient presented with a concomitant systemic lupus-like syndrome which has been already described for statins but not in association with severe liver disease. Although the drug was immediately withdrawn the disease persisted and even deteriorated to a fulminant disease with evidence of acute hepatic failure. The patient failed to respond to conventional immunosuppression with corticosteroids and azathioprine. Only the introduction of intense immunosuppressive therapy, as used in solid organ transplantation, led to a complete and sustained recovery of the patient. Interestingly, the patient was HLA DR3- and HLA DR4-positive, which are well-known genetic factors associated with autoimmune diseases. This case is the first report of a drug-induced lupus-likesyndrome concomitant with a severe autoimmune hepatitis in a genetically predisposed patient.

Drug-induced liver injury: Summary of a single topic clinical research conference

“…53 These three patients double the reported instances of this association. [54][55][56] Classical features of AILD include strong female preponderance (true for most auto-immune diseases), chronicity for Ͼ6 months (albeit arbitrary), the presence of hyperglobulinemia, circulating autoantibodies, extrahepatic clinical autoimmune syndromes, overrepresentation of HLA DR3 or DR4, and a low rate of spontaneous remission. 57 …”

A Familial Case of Autoimmune Hepatitis

“…The following individuals could be assessed: mother (case 1), three sons, and four daughters including the second daughter (case 2) (Fig. 3) (6,7), viruses (8)(9)(10)(11), and alteration of hormonal status (e.g., pregnancy) (12) (1,(6)(7)(8)(9)(10)(11)(12) …”

Statins and hepatotoxicity: Focus on patients with fatty liver

“…8,15 It recently has been reported that statins may induce or unmask autoimmune liver disease, although the mechanism by which they induce this phenomenon is unknown. [17][18][19] However, this phenomenon appears to be quite rare. Irreversible liver damage leading to death or liver transplantation appears to be extremely uncommon with statins, especially when one considers the magnitude of their use worldwide.…”