Against the above background, in 1992, an international panel met in Brighton, United Kingdom, to review diagnostic criteria for AIH. The panel, which became the International Autoimmune Hepatitis Group, noted that there are no features that are pathognomonic of AIH and that diagnosis requires the finding of a combination of suggestive abnormalities together with careful exclusion of other possible causes of chronic liver disease. In particular, it was noted that the archetypal young female patients described above comprised a relatively small proportion of the cases seen in clinical practices; most patients were older, and although females predominated (4:1), the condition also affected males. It was also noted that only about 20% of patients had positive LE cell tests; some patients presented with ANA, others with SMA and some with both, while a small proportion (about 3% to 4% overall) had liver-kidney microsomal autoantibodies (anti-LKM1) without ANA or SMA; and, importantly, 10% to 20% of patients in some series had none of these markers at presentation (Figure 1).
The report of that meeting, termed the ‘Brighton report’, recommended that a diagnosis of AIH should be considered in any patient presenting with an unexplained acute or chronic hepatitic illness or biochemical liver test abnormalities in the absence of signs or symptoms of liver disease. It included a set of descriptive criteria and a diagnostic scoring system, which the panel suggested could be used to classify patients as having either definite or probable AIH according to how closely their presenting features conformed to classic ‘lupoid’ hepatitis. This approach took account of the wider spectrum of the disease that was being recognized. Your drugs could cost you less – buy ampicillin to start the treatment soon.
Figure 1) Frequency of lupus erythematosus (LE) cells, antinuclear antibodies (ANA) and smooth muscle autoantibodies (SMA) in patients with presumed autoimmune hepatitis. Note that patients were not tested for type 1 liver-kidney microsomal antibodies or other autoantibodies. +ve Positive. Data from reference 45
Typical presentation: The panel noted that AIH can present at almost any age, and approximately 70% to 80% of patients are female. Onset is usually insidious, with various signs or symptoms fluctuating with a periodicity of a few months to one or two years. Lethargy (often profound fatigue) is a prominent feature and may be the dominant symptom. Other common complaints include general malaise, nausea, anorexia (with consequent weight loss), upper abdominal discomfort or pain, oligomenorrhea in women, mild pruritus, skin rashes (various, but often macu-lo-papular), arthralgia (with or without myalgia) and occasionally persistent low grade pyrexia. There is often a background of other autoimmune or allergic conditions in the patient or first-degree relatives. About 75% of patients are jaundiced or report prior icteric episodes, but importantly, 25% have no history of jaundice. Up to 30% already have cirrhosis at presentation, and cutaneous stigmata of chronic liver disease may be evident. Ascites and/or peripheral edema may be present even in those without cirrhosis.