rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.14412/1995-4484-2005-50rsp-334Research ArticleСтатьиArticlesPFAPA ( Periodic fever, aphtous stomatitis,pharingitis, cervical adenitis -периодическая лихорадка, афтозный стоматит, шейный аденит) или синдром Маршалла у детейPFAPA (Periodic fever, aphtous stomatitis, pharingitis, cervical adenitis) or Marshall’s syndrome in childrenКузьминаН. Н.KuzminaN N-MovsisyanG RMovsisyanG R-200515102005435№5 (2005)8083Copyright © Кузьмина Н.Н., Movsisyan G.R., 20052005Кузьмина Н.Н., Movsisyan G.R.Kuzmina N.N., Movsisyan G.R.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/334

PFAPA или синдром Маршалла (периодическая лихорадка, афтозный стоматит, фарингит, шейный аденит) одно из редких периодических лихорадочных состояний, встречающихся в детском возрасте. Причина, вызывающая этот синдром, который может продолжаться в течение ряда лет, неизвестна. В межприступный период ребенок чувствует себя совершенно здоровым, растет и развивается нормально. Дифференциальный диагноз следует проводить с другими состояниями, сопровождающимися периодической лихорадкой: болезнью Бехчета, циклической нейтропенией, семейной средиземноморской лихорадкой, семейной Ирландской лихорадкой, синдромом гипериммуноглобулинемии D, системным вариантом ювенильного идиопатического артрита, хроническим тонзиллитом, рядом инфекционных заболеваний. Для лечения синдрома PFAPA используются многие лекарственные препараты: антибиотики, нестероидные противовоспалительные средства, плаквенил, противовирусные препараты, глюкокортикоды, циметидин, нередко проводится тонзиллэктомия. Анализ литературных данных показывает, что лучшие результаты при лечении этого заболевания может давать тонзиллэктомия ( иногда в сочетании с аденотомией). Приводится клиническое наблюдение впервые диагностированного в отечественной педиатрической ревматологии синдрома PFAPA у ребенка 2-х лет.

PFAPA (periodic fever, aphtous stomatitis, pharingitis, cervical adenitis) or Marshall’s syndrome is one of the rare periodic fever conditions appearing in children. Its cause is unknown. This syndrome may continue for several years. During interictal period the child is quite well, grows and develops normally. The disease should be differentiated from Behcet’s disease, cyclic neutropenia, familial Mediterranean fever, familial Ireland fever, hyperimmunoglobulinemia D syndrome, systemic juvenile idiopathic arthritis, chronic tonsillitis, some infectious diseases. Many drugs are used for the treatment of PFAPA syndrome: antibiotics, non-steroidal anti-inflammatory drugs, chloroquin, antiviral drugs, glucocorticoids, cimetidin. Tonsillectomy is used quite often. Analysis of the literature data shows that best results may be achieved with tonsillectomy (sometimes in combination with ade- notomy). PFAPA in a child of 2 years age diagnosed for the first time in Russian pediatric rheumatology is described.

синдром Маршаллапериодическая лихорадкатонзиллиттонзиллэктомияредкие болезни у детейPFAPAMarshall’s syndromePFAPAperiodic fevertonsillitistonsillectomyrare diseases in childrenReferences<div><p>Abramson J.S., Givner L.B., Thompson J.N. Possible role оГ tonsillectomy and adenoidectomy in children with recurrent fever and tonsillopharyngitis. Pediat. Infect. Dis.J., 1989,8,119120.</p><p>Arav-Boger R., Spirer Z. Periodic syndromes of childhood. Adv. Pediatr. 1997,44, 389-428.</p><p>Berlucchi М., Meini A,, Plebani A. et al. Update on treatment of PFAPA-syndrome: report of five cases with review of the literature. Ann. Otol. Rhinol. Laryngol., 2003, 112, 365-369.</p><p>Berlucchi М., Nicolai P. Marshall's syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome. Orphanet. Encyclopedia. January 2004</p><p>Chajck Т., Fainaru M. Behcet’s disease: report of 41 cases and a review of the literature. Medicine, 1975, 54, 179-196.</p><p>Condemi J.J. The autoimmune diseases. JAMA. 1987, 258, 2920-2929.</p><p>Dahn К.А., Glode М.P., Chan K.H. Periodic fever and pharyngitis in young children. A new disease for the otolaryngologist? Arch. Otolaryngol. Head Neck. Suig., 2000, 126, 1146-1149.</p><p>Dode C., Cuisset L., Delpech M. etalTNFRSFIA-asso- ciated periodic syndrome (TRAPS), Muckle-Wfells syndrome (MWS) and renal amyloidosis. J. Nephrol., 2003,16, 435437.</p><p>Ehrenfeld E.N., Eliakim M,, Rachmilewitz M. Recurrent polyseriositis (familial Mediterranean fever; periodic disease); a report of fifty-five cases. Am. J. Med., 1961, 31, 107-123.</p><p>Feder H.M. Periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr. Infect. Dis. J. 1989, 8, 186-187.</p><p>Feder H.M. Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr. Infect. Dis. J., 1992, 11, 318-321.</p><p>Feder H.M. Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome. Curr. Opin. Pediatr., 2000, 12, 253-256.</p><p>Galanakis E., Papadakis C.E., Giannoussi E., et al. PFAPA syndrome in children evaluated for tonsillectomy. Arch. Dis. Child, 2002,86,434-435.</p><p>Gedalia A., Adar A., Gorodicher R. Familial Mediterranean fever in children. J. Rheumatol, (suppl.), 1992, 35, 1-9.</p><p>GhateJ.V., Jorizzo J.L. Behcet's disease and complex aph- thosis. J. Am. Acad. Dermatol., 1999,40, 1-20.</p><p>Grose C., Schnetzer R., Ferrante A., Vladutio A.O. Children with hyperimmunoglobulinemia D and periodic fever syndrome. Pediatr Infect. Dis. J., 1996, 15, 72-77.</p><p>Jorizzo J.L,, Sams W.M., Jegasothy B.V., Olansky J. Cimetidine as an immunomodulaion chronic mucocutaneous candidiasis as a model. Ann Intern. Med., 1980, 92, 192-195.</p><p>Lee W.l, Yang M.H., Lee K.F., et al. PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis). Clin. Rheumatol., 1999, 18, 207-213.</p><p>Lekstom-Himes J.A., Dale J.K., Kingma D.W. et al. Periodic illness associated with Epstein-Barr virus infection. Clin. Infect. Dis., 1996, 22, 22-27.</p><p>Marshall G.S., Edwards К.М., Butler J. et. al. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J. Pediatr., 1987, 110, 43-46.</p><p>Marshall G.S, Edwards K.M. PFAPA syndrome (letter]. Pediatr. Infect. Dis. J., 1989, 8, 658-659.</p><p>Padeh S., Brezniak N., Zemer D. et. al. Z. Periodic, fever, aphthous stomatitis, paryngitis and adenophaty syndrome: clinical characteristics and outcome. J. Pediatr., 1999, 135, 98-101</p><p>Raimann H.A. Periodic disease: a probable syndrome including periodic fever, benign paroxysmal peritonitis, cyclic neutropenia and intermittent arthralgia. JAMA, 1948, 136, 239-244.</p><p>Raimann H.A., de Berardinis C.T. Periodic (cyclic) neutropenia, an entity. Blood , 1949,4,1109-1116.</p><p>Reeves W.G., Mitchell J.R.A. Hyperimmunoglobulinaemia D and periodic fever. Lancet, 1984, 1, 1463-1464.</p><p>Thomas K.T., Feder H.M., Lawton A.R, Edwards K.M. Periodic fever syndrome in children. J. Pediatr., 1999, 135,15-21.</p><p>Van der Meer J.W.M, Vbssen J.M, Radi J., van Nieuwkoop J.A. et. al. Hyperimmunoglobulinemia D and periodic fever a new syndrome. Lancet, 1984, 1, 1087-1090.</p><p>Williamson L.M., Hull D., Mehta R. el.al. Familial hibemian fever. Quart. J. Med., 1982, 51, 469-480</p></div><br /><div><p>Abramson J.S., Givner L.B., Thompson J.N. Possible role оГ tonsillectomy and adenoidectomy in children with recurrent fever and tonsillopharyngitis. Pediat. Infect. Dis.J., 1989,8,119120.</p><p>Arav-Boger R., Spirer Z. Periodic syndromes of childhood. Adv. Pediatr. 1997,44, 389-428.</p><p>Berlucchi М., Meini A,, Plebani A. et al. Update on treatment of PFAPA-syndrome: report of five cases with review of the literature. Ann. Otol. Rhinol. Laryngol., 2003, 112, 365-369.</p><p>Berlucchi М., Nicolai P. Marshall's syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome. Orphanet. Encyclopedia. January 2004</p><p>Chajck Т., Fainaru M. Behcet’s disease: report of 41 cases and a review of the literature. Medicine, 1975, 54, 179-196.</p><p>Condemi J.J. The autoimmune diseases. JAMA. 1987, 258, 2920-2929.</p><p>Dahn К.А., Glode М.P., Chan K.H. Periodic fever and pharyngitis in young children. A new disease for the otolaryngologist? Arch. Otolaryngol. Head Neck. Suig., 2000, 126, 1146-1149.</p><p>Dode C., Cuisset L., Delpech M. etalTNFRSFIA-asso- ciated periodic syndrome (TRAPS), Muckle-Wfells syndrome (MWS) and renal amyloidosis. J. Nephrol., 2003,16, 435437.</p><p>Ehrenfeld E.N., Eliakim M,, Rachmilewitz M. Recurrent polyseriositis (familial Mediterranean fever; periodic disease); a report of fifty-five cases. Am. J. Med., 1961, 31, 107-123.</p><p>Feder H.M. Periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr. Infect. Dis. J. 1989, 8, 186-187.</p><p>Feder H.M. Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr. Infect. Dis. J., 1992, 11, 318-321.</p><p>Feder H.M. Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome. Curr. Opin. Pediatr., 2000, 12, 253-256.</p><p>Galanakis E., Papadakis C.E., Giannoussi E., et al. PFAPA syndrome in children evaluated for tonsillectomy. Arch. Dis. Child, 2002,86,434-435.</p><p>Gedalia A., Adar A., Gorodicher R. Familial Mediterranean fever in children. J. Rheumatol, (suppl.), 1992, 35, 1-9.</p><p>GhateJ.V., Jorizzo J.L. Behcet's disease and complex aph- thosis. J. Am. Acad. Dermatol., 1999,40, 1-20.</p><p>Grose C., Schnetzer R., Ferrante A., Vladutio A.O. Children with hyperimmunoglobulinemia D and periodic fever syndrome. Pediatr Infect. Dis. J., 1996, 15, 72-77.</p><p>Jorizzo J.L,, Sams W.M., Jegasothy B.V., Olansky J. Cimetidine as an immunomodulaion chronic mucocutaneous candidiasis as a model. Ann Intern. Med., 1980, 92, 192-195.</p><p>Lee W.l, Yang M.H., Lee K.F., et al. PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis). Clin. Rheumatol., 1999, 18, 207-213.</p><p>Lekstom-Himes J.A., Dale J.K., Kingma D.W. et al. Periodic illness associated with Epstein-Barr virus infection. Clin. Infect. Dis., 1996, 22, 22-27.</p><p>Marshall G.S., Edwards К.М., Butler J. et. al. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J. Pediatr., 1987, 110, 43-46.</p><p>Marshall G.S, Edwards K.M. PFAPA syndrome (letter]. Pediatr. Infect. Dis. J., 1989, 8, 658-659.</p><p>Padeh S., Brezniak N., Zemer D. et. al. Z. Periodic, fever, aphthous stomatitis, paryngitis and adenophaty syndrome: clinical characteristics and outcome. J. Pediatr., 1999, 135, 98-101</p><p>Raimann H.A. Periodic disease: a probable syndrome including periodic fever, benign paroxysmal peritonitis, cyclic neutropenia and intermittent arthralgia. JAMA, 1948, 136, 239-244.</p><p>Raimann H.A., de Berardinis C.T. Periodic (cyclic) neutropenia, an entity. Blood , 1949,4,1109-1116.</p><p>Reeves W.G., Mitchell J.R.A. Hyperimmunoglobulinaemia D and periodic fever. Lancet, 1984, 1, 1463-1464.</p><p>Thomas K.T., Feder H.M., Lawton A.R, Edwards K.M. Periodic fever syndrome in children. J. Pediatr., 1999, 135,15-21.</p><p>Van der Meer J.W.M, Vbssen J.M, Radi J., van Nieuwkoop J.A. et. al. Hyperimmunoglobulinemia D and periodic fever a new syndrome. Lancet, 1984, 1, 1087-1090.</p><p>Williamson L.M., Hull D., Mehta R. el.al. Familial hibemian fever. Quart. J. Med., 1982, 51, 469-480</p></div><br />

The authors declare that there are no conflicts of interest present.