Chorea as the Presenting Clinical Feature of Primary Antiphospholipid Syndrome in Childhood

Sir,

We read with interest the article by Kiechl-Kohlendorfer et al [[6]] reporting on 3 children with chorea as the presenting feature of primary antiphospholipid syndrome (PAPS). We find it difficult to exclude the possibility of acute rheumatic fever (ARF) as the cause of chorea in these patients for the following reasons:

One of the common causes of chorea in childhood is Sydenham chorea related to rheumatic fever [2, 8]. Figueroa et al [5] reported that 80 % of patients were positive for anticardiolipin (aCL) during the acute episode of rheumatic fever versus 40 % when the disease was inactive. The frequency of aCL antibodies was the same in patients whether or not they had chorea. Although Narin et al [7] failed to reproduce these findings, they too had some patients who they felt had ARF and who had high aCL antibody levels. Antiphospholipid antibodies are not specific to PAPS syndrome and can be detected in a wide variety of infectious, neoplastic, inflammatory and autoimmune diseases [1]. Streptococcal antibody titers may be normal at the time of presentation of isolated Sydenham chorea, because of the long latency between acute group A streptococcal infection and the clinical onset of chorea [3]. Therefore a negative ASOT does not rule out ARF as the cause of chorea.

For these reasons, we would counsel strongly against denying a child with possible Sydenham chorea who is aCL-positive appropriate antibiotic prophylaxis [[4]] unless she/he has other classical features of PAPS.

References

• 1 Cassidy J, Petty R. Textbook of Pediatric Rheumatology, 3rd ed. W. B. Saunders Co. 1995: 304
• 2 Congeni B L. The resurgence of acute rheumatic fever in the United States.  Pediatr Ann. 1992;  21 816-820
• 3 Dajani A S, Ayoub E, Bierman F Z. et al . Guidelines for the diagnosis of rheumatic fever: Jones Criteria, Updated 1992.  American Heart Association, Circulation. 1993;  87 302-307
• 4 Dajani A, Paubert K, Ferrieri L. et al . Treatment of acute streptococcal pharyngitis and prevention of rheumatic fever: Statement for Health Professionals.  Pediatrics. 1995;  96 758-764
• 5 Figueroa F, Berrios X, Guiterraz M, Carrion F, Goycolea J, Riedel I, Jacobelli S. Anticardiolipin antibodies in acute rheumatic fever.  J Rheumatol. 1992;  19 1175-1180
• 6 Kiechl-Kohlendorfer U, Ellemunter H, Kiechl S. Chorea as the presenting clinical feature of primary antiphospholipid syndrome in childhood.  Neuropediatrics. 1999;  30 96-98
• 7 Narin N, Kutukculer N, Narin F, Keser G, Doganavsargul E. Anticardiolipin antibody in acute rheumatic fever and chronic rheumatic heart disease: Is there a significant association.  Clinical and Experimental Rheumatology. 1996;  14 567-569
• 8 Swedo S E. Sydenham's chorea: A model for childhood autoimmune neuropsychiatry disorders.  JAMA. 1994;  22 1788-1791