We report on a 22-year-old girl with a history of recurrent febrile episodes, chronic arthritis, urticarial rash, and neurological symptoms including right hemiparesis, internal hydrocephalus, mental retardation, progressive deafness, and visual impairment. Treatment starting at age 20 months, including different combinations of immunosuppressive and antiinflammatory drugs such as corticosteroids and anti-TNFalpha antibody, was unsuccessful. Four years ago, we found a heterozygous S595G mutation in the NLRP3 gene of this patient. This prompted us to introduce anakinra, which resulted in considerable improvement of the patient's complaints.

Background: Juvenile chronic arthritis (JCA) is the commonest autoimmune rheumatic disease in childhood and presents different clinical subtypes. Juvenile chronic arthritis is considered to be of a polygenic nature and its genetic background is still under investigation. The clinical profile of JCA in the Greek population has not been studied completely. This study retrospectively analyzed the clinical and immunological features of JCA i n Greek children presented between 1989 and 1994. Human leukocyte antigen (HLA)-positive or -negative ociations in the different clinical subtypes were also detected. The findings of this study were correlated with those reported from other populations.Merhodr a.nd results: Antinuclear antibodies (ANA) anti-ds DNA and anti-extractable nuclear antigen antibodies were estimated by immunofluorescent and ELISA assays. Human leukocyte antigen typing was performed by niicrolymphocytotoxicity. using inimunobeads. The peak ages of JCA onset were between 2 and 5 years and also between 9 and 12 years. There was a high female predominance in pauciarticular and polyarticular groups. The most common disease was pauciarticular (58.7%) followed by systemic (25"/0) arthritis. The incidence of eye involvement was 12.5% and presented only in the pauciarticular group. Overall, ANA positivity was 53.7'/0, increasing to 90% in pauciarticular cases associated with chronic uveitis. In the early onset (EOPA) pauciarticular subtype, positive-HLA associations with alleles DR11 and DR8 were shown. In the late onset pauciarticular (LOPA) group only B27 allele was increased. Concluswns: The results of this retrospective study did not reveal major differences between JCA in Greek children compared with other Caucasian series. Key wordsauto-antibodies. chronic uveitis. Greek population. human leukocyte antigen associations, juvenile chronic arthri ti 5 .Juvenile chronic arthritis (JCA) is the most commonly encountered disease in pediatric rheumatology.' It is a heterogeneous group of disorders and represents three subtypes; systemic. polyarticular and pauciarticular. classified by their mode of onset and the disease course during 6months. A number of studies show that JCA is an autoimmune rheumatic disease of polygenic nature.' The genetic background of the disease has been under investigation and some interesting immunogenetic associations have been revealed.'.' Clinical and immunogenetic data on JCA in the Greek population is very limited.The aim of this study was to analyze the clinical features, immunological finding\ and immunogenetic associations of JCA in Greek children over a 5 year period.

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Messaritakis J, Psychou F, Dracou C, Nicolaidou P, Kakourou T. Arthritis and vasculitis during the incubation period of varicella. Acta Prediatr 1994;83:681-3. Stockholm. ISSN Arthritis and vasculitis are very rare complications of varicella occurring almost exclusively after the eruption of the characteristic rash. We report two children, aged three and seven years, who developed vasculitis and polyarthritis (patient No. 1) and arthritis (patient No. 2) 13 and 9 days, respectively, before the onset of their typical varicella. Arthritis and vasculitis so early in the incubation period of varicella have not been described previously. In all previously reported cases the arthritis occurred after or together with the onset of varicella and only in one instance preceded the exanthem by 2 days. Although this may have been an unfortunate coincidence, an association between varicella zoster virus and these manifestations should be seriously considered. Clinicians confronted with such cases must be cautious and search for varicella infection, especially in times of epidemics. Arthritis, incubation period, skin vasculitis, varicella J Messaritakis, 1st Department of Paediatrics, A thens University, Aghia Sophia, Children's Hospital, Athens 115-27. GreeceArthritis and vasculitis are uncommon complications of varicella in children. A review of the reported cases reveals that these complications usually appear after the onset of the varicella exanthem, very seldom concomitantly and in one case preceded the exanthem by only 2 days (1-8). We present two patients in whom aseptic arthritis and vasculitis preceded the onset of varicella by 13 and 9 days, respectively. Case reportsCase 1 A three-year-old boy was referred to us because of fever, polyarthritis and a purpuric rash. There was no history of sore throat, rash, diarrhoea or exposure to other known infections except varicella, to which he was intentionally exposed by his parents 7 days before his admission. At that time (1991) a small epidemic of the disease occurred in our country. The physical examination revealed an acutely ill boy with high fever, a fainting macular, purpuric rash extending all over his right arm, both legs and buttocks, his palms and left ear. His wrists were severely painful, warm and swollen, with limitation of motion. Severe pain with loss of full range of motion was noticed in both hips and ankles. His symptoms subsided gradually withn 8 days, except for the arthritis of his left hip and wrist, which persisted. On the 13th day after his admission, his temperature increased again to 39 "C and a typical varicella rash appeared. His condition gradually improved. In a few days he was walking normally, but the arthritis of his left wrist persisted for almost 6 weeks. At follow-up 4 months later, he was erfectly well.White blood count was 15 x 10 cells/l with 82% neutrophils, 16% lymphocytes and 2% monocytes, with normal platelets and haematocrit. Erythrocyte sedimentation rate (ESR) was 90 mm/h, CRP 94 mg/l, serum C3 1190 mg/l, C4 360 mg/l, normal im...

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