Lupoid Hepatitis of Late Onset

Abstract

Three cases of lupoid hepatitis according to the definition of Mackay et al. (1965) are presented.

To comply with this definition such cases should have (a) evidence of continuing hepatitis, as manifested by elevated levels of transaminases, associated with a relapsing jaundice; (b) hypergammaglobulinaemia; (c) liver biopsy showing cirrhosis, ‘piece mealrsquo; liver cell necrosis and lymphocyte and plasma cell infiltrate; and (d) improvement on immunosuppressive agents such as corticosteroids. Furthermore there should be evidence of systemic lupus erythematosus in the form of anti-nuclear factor or positive L.E. cell test or minor manifestations of S.L.E.

Each patient was aged 50 or over at the onset of symptoms, an unusual finding, as most patients studied have been aged 21 or under at onset. The modes of presentation were (1) recurrent obstructive ***aundice; (2) recurrent haematemesis and melaena; and (3) polyarthritis and hepatomegaly. The co-existence of other diseases thought to have an auto-immune basis is noted in 2 cases.

The need for a common definition of this disorder is stressed as the condition has been described by various authors with varying criteria for diagnosis. The possible aetiologies of the condition are discussed. It is thought that these studies lend support to the suggestion that lupoid hepatitis is a disease of auto-immune aetiology.