Syndrome de Marshall: résultats d'une enquête nationale rétrospective

Abstract

Marshall's syndrome or periodic fever syndrome was first described in 1987 in the USA based on observations of 12 children under the age of five with periodic fever (> 38 degrees C) and accompanying aphtous stomatitis, pharyngitis, and cervical adenopathy (PFAPA). In 1998, a national retrospective study was carried out in France by the pediatric infectious pathology group, and a semeiological analysis was made of 22 cases. The main characteristics of Marshall's syndrome found in this patient population were in agreement with those reported in the literature. The onset of symptoms occurred between the age of 3 months and 12 years, with a mean age of 5 years; no geographical or ethnic predisposing factors were noted. The diagnosis of symptoms was subsequently established at an age ranging from 5 months to 16 years, with a mean age of 6.5 years. It was determined that following an initial phase of generalized clinical manifestations (asthenia, cranial neuritis, dysphagia, anorexia), the symptoms become stereotyped, with the sudden appearance of high fever (> 40 degrees C), shivering, aphtous stomatitis, pharyngitis, and cervical adenopathy. Other symptoms such as cranial neuritis, arthralgia, and abdominal pain may also be present (50% of cases in the present study), but due to their variability of appearance they are of lesser diagnostic value. The main characteristic of Marshall's syndrome is its periodic aspect; with fever occurring every 6 to 9 weeks, with a mean interval of 66 days before recurrence of fever compared to the shorter interval of 21 to 28 days reported in the literature. After excluding the presence of an infection, the differential diagnosis includes the following: familial Mediterranean fever, hyper IgD syndrome, and feverish neutropenia. During the periods of fever, an inflammatory syndrome with hyperleucocytosis and a marked increase in C-reactive protein (CRP) levels and sedimentation rate is observed. The most effective treatment seems to be the early administration of corticoids during the initial phase, prior to the appearance of more specific symptoms. The prognosis is excellent, with a progressive decrease in the incidence of periodic fever and an absence of complications. However, the etiology of Marshall's syndrome has not yet been determined.