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Re: Leukotrienes and familial mediterranean fever

Dear Editor,

Frenkel J et al found an increased urinary leukotriene E4 during febrile attacks in Hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS), but not in children with fever due to viral or bacterial infections. Starting from the well known alteration of the cholesterol metabolism, due to Mevalonate kinase (MVK) deficiency in HIDS and Mevalonic aciduria (MA), they investigated the role of the cysteinyl leukotrienes in the febrile attacks typical of HIDS and concluded providing a probable new therapeutic approach for these patients, leukotrienes receptor antagonists.

For 5 years, we have been following a number of Italian patients with periodic fever, in particular Familial Mediterranean Fever (FMF), well assessed by clinical and genetic analysis of MEFV.

Two of them suffered from FMF and bronchial asthma, too, so we prescribed them montelukast 10 mg/d p.o. chronically.

Now, after 6 months of therapy, they report an improvement in respiratory symptoms, but also a surprising effect of the drug on articular pain, both during the attacks and out of the time of the febrile bouts. A third patient is starting montelukast just in these days.

We can’t provide their urinary levels of LTE4, but these good responses to montelukast suggest that cysteinyl leukotrienes might have a role in the systemic inflammation of FMF, too; neverthless it is known that a high-fat diet can trigger the attack in patients with FMF.