REVIEWS   

Gazzetta Medica Italiana Archivio per le Scienze Mediche 2000 October;159(5):159-63

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: Italian

Marshall’s syndrome. A new periodic fever

Cau C.

DEA - dell’Azienda ospedaliera S. Giovanni Addolorata - Roma

A syn­drome of peri­odic ­fever has ­been iden­ti­fied ­recently by Mar­shall in 12 chil­dren ­observed at two ­major ­referral cen­ters. The ­acronym ­used to ­describe the syn­drome is ­PFAPA (for ­fever, aph­thosis, sto­ma­titis, pha­ryn­gitis and cer­vical ade­nitis). ­Attacks char­ac­ter­ized by ­abrupt ­onset of ­fever, mal­aise, ­chills, aph­tosis, sto­ma­titis, pha­ryn­gitis, hed­ache and ­tender cer­vical ade­nop­athy ­occur at 4- to 6 ­week inter­vals ­over ­periods of ­years. ­These epi­sodes of ill­ness ­resolve spon­ta­ne­ously in 4 to 5 day. ­Mild leu­ko­cy­tosis and ele­va­tion of the eryth­ro­cyte sed­i­men­ta­tion ­rate ­during ­attacks are the ­only labor­a­tory abnor­mal­ities. ­Affected chil­dren ­grow nor­mally. In ­some chil­dren the syn­drome ­resolves, ­whereas symp­toms fn ­others per­sist for ­years and com­pli­ca­tions ­have not ­been ­described. A ­number of ­other syn­dromes char­ac­ter­ized by recur­rent ­fever ­have ­been ­reported and com­pared ­with ­PFAPA: ­familial Med­i­ter­ra­nean ­fever, the ­hyper-IgD syn­drome, ­Behçet dis­ease, hered­i­tary angioe­dema, ­cyclic neu­tro­penia, ­familial Hiber­nian ­fever. ­Attacks may ­aborted by ­short ­courses of pred­ni­sone but do not ­respond to non ster­oidal ­anti-inflam­ma­tory ­agents. Ace­tam­i­no­phen and anti­bi­otic ­drugs ­were ­reported to ­have ­modest ther­a­peutic ben­efit Cimet­i­dine was sug­gested as a pro­phy­lactic treat­ment for ­PFAPA in 1992. It ­appeared to be ­highly effec­tive in pre­venting ­attacks in ­some ­patients. How­ever, we ­cannot ­explain why, ­when cimet­i­dine was dis­con­tinued, ­PFAPA syn­drome did not ­recur. Per­haps a per­ma­nent ­change in ­these ­patients’ ­immune ­systems fol­lowed the 6 to 8 ­months of cimet­i­dine ­therapy. ­Another pos­sibility is spon­ta­neous remis­sion.