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The diagnosis of recurrent hereditary polyserositis (RHP; also known as familial Mediterranean fever) remains one of exclusion since there has been no specific diagnostic laboratory test. A previous study suggested that the disorder is related to abnormal catecholamine metabolism. Plasma dopamine beta-hydroxylase (DBH) activity was assayed spectrophotometrically in 91 RHP patients and 162 controls. The activity was significantly higher in untreated symptom-free patients and in patients with acute attacks, than in controls (mean [SEM] 155.8 [14.1] vs 43.3 [1.9] mumol/min/1 p less than 0.0001). Colchicine treatment reduced DBH activity to control levels. The test showed a high diagnostic accuracy and specificity for RHP, whether the patient was symptom-free or having an acute attack. Moreover, it is easy to carry out.

The diagnosis of familial Mediterranean fever has been one of exclusion. In a placebo-controlled, double-blind, cross-over study a challenge with a 10 mg dose of metaraminol infusion was followed within 48 h by a typical disease-like attack in all of 21 patients with familial Mediterranean fever but in none of 21 control subjects. The induced attacks were milder and of shorter duration than the spontaneous ones. The metaraminol-induced symptoms were similar to the natural disease attacks and could be prevented with prophylactic colchicine therapy. No significant side-effects were observed.