Recurrent Polyserositis. (Familial Mediterranean Fever, Periodic Disease).
Authors: Ernest N. Ehrenfeld, Marcel Eliakim, Moshe Rachmilewitz
Journal: Annals of Internal Medicine (1982)

Abstract  The clinical and laboratory features of fifty-five patients with recurrent polyserositis are presented. All patients were Jews, forty-five of Sephardi or Oriental origin, ten of Ashkenazi parentage.  A familial incidence was established in 30 per cent of the families. One generation was affected twice as frequently as two generations.  The symptoms consisted of attacks of fever (all cases), abdominal pain (98 per cent), chest pain (38 per cent), joint pain (31 per cent), gastrointestinal disturbances, headache, and other less frequent manifestations.  The most frequent signs were colloid body-like dots in the eye grounds (50 per cent), palpable spleen (40 per cent), systolic murmur over the apex and parasternally (29 per cent) and palpable (probably enlarged) liver (27 per cent). The main signs during an attack were extreme tenderness and rigidity of the abdominal wall, decreased motility of the diaphragm, and occasional pleural and joint effusion.  The attacks lasted from one to four days, and appeared at irregular intervals of two days to several months. The longest remission (still persisting) was five years. Cold seemed to precipitate, and pregnancy to suppress, the appearance of attacks.  The chief laboratory findings during an attack were leukocytosis, increased sedimentation rate and increased serum  α  2  globulin level. An abnormal serum β globulin appeared during an attack in two cases.  Hypersensitivity to old tuberculin was observed in eight of forty-three patients examined. In five of these, attacks could be provoked by the injection of minute quantities of tuberculin. Two of three attempts at desensitization resulted in remissions for as long as eighteen months.  Transient electrocardiographic abnormalities were found in four of thirty-one patients examined. In two of these the changes were suggestive of pericarditis, while in the remaining two the changes were non-specific and confined to the T waves.  Electroencephalographic abnormalities were observed in seven of sixteen patients examined. In two the changes were present only during an attack. The changes were diffuse and non-specific in five, and of the convulsive type in the remaining two.  Amyloidosis developed in four patients (7.2 per cent). Two additional patients had albuminuria without evidence of amyloidosis. Two of the patients with amyloidosis died of renal failure. Autopsy revealed amyloid deposits in the skin, heart, kidneys, skeletal muscle, spleen, ovaries and adrenals.  Except for the patients with amyloidosis, the prognosis was good. The average duration of the disease at the time of the study was nine years and one month. One patient survived more than a thousand attacks in the course of thirty-nine years, with no apparent ill effects on his general condition.  Numerous kinds of treatment were of no avail. ACTH and corticosteroids suppressed the attacks in eleven of fifteen patients, but could not be given indefinitely because of the relatively large doses required, and because the dangers involved were not justified by the temporary nature of the effects.  The pathogenesis of the disease is discussed in the light of present knowledge.