La maladie de Still de l´enfant et la leucémie aigue lymphoblastique ont des similitudes cliniques et biologiques qui posent des problèmes de retard de diagnostic et de prise en charge. En effet, si la maladie de Still est un diagnostic d´exclusion en rhumatologie, la polyarthrite associée à l´hyperleucocytose en contexte fébrile qui la caractérise est souvent rencontrée au cours des leucémies aigues lymphoïdes au stade précoce. Nous rapportons le cas d´une fillette de 4 ans, traitée comme maladie de Still de l´enfant et chez qui le diagnostic de LAL a été posé au bout de 2 mois grâce à une biopsie de la moelle osseuse.
Objective: Although the discordance in the disease activity scores based on 28 joints (DAS28) has been widely investigated, few data exist among black African (BA) populations. We aimed first to evaluate the correlation and second to assess the concordance between various DAS28 scores in the BA patients with rheumatoid arthritis (RA). Methods: We conducted a multicenter cross-sectional study in adult patients who had RA from Guinea and Cameroon. These patients were diagnosed according to the 2010 ACR/EULAR criteria. Outcome measures were DAS28-ESR and various DAS28-CRP scores. Paired t-test, agreement kappa coefficient, spearman's correlation coefficient, Bland-Altman plots, and ROC curves were used to analyze different DAS28 scores. Results: Of 74 patients analyzed, 85% were female, mean age of these patients was 50 years, and median disease duration was 36 months (IQR: 12-90). Four DAS28 scores comparisons were performed. DAS28-ESR was on average higher than standard DAS28-CRP (Δ = 0.58, [95% CI: 0.45-0.71]; p < 0.0001) and DAS28-CRP without patient's global VAS assessment (Δ = 0.51 [0.46-0.75]; p < 0.0001). Correlation between DAS28 scores was moderate ranging from 0.6 to 0.82. Overall agreement was lower for the conventional DAS28-ESR (weighted kappa: Range, 37.3% to 55.3%) than Castrejon DAS28-CRP (72.6% to 87.1%). DAS28-CRP-3 has a higher predictive power to evaluate disease activity than DAS28-ESR-3 (AUC, 0.97 [0.95-1.00] vs. 0.80 [0.68-0.91], p = 0.0062). Conclusion: The current study provides information on correlation and discrepancy between different disease activity scores in BA patients with RA, and suggests that these markers were not interchangeable.
Juvenile idiopathic arthritis (JIA) constitutes a group of arthritis of unknown origin that begins before the age of 16 years. Still´s disease is the systemic form of this condition. Its clinical presentation is marked by fever, rash and sometimes joint pain, in the absence of evidence of another aetiology of the fever. We present the cases of two boys aged 4 and 10 years admitted for fever, with a cerebral origin for the first and no infectious site for the second. Fever persisted after antimalarial treatment and adequate antibiotics. Ferritinaemia, elevated sedimentation rate, lactate dehydrogenase (LDH), triglycerides, and increased serum transaminases, all in the absence of evidence of other inflammatory or malignant diseases were suggestive of Still's disease. Both children received a corticosteroid therapy with progressive dose reduction associated to methotrexate during treatment. Fever disappeared within a few hours after initiation of corticosteroid therapy, with considerable improvement in clinical state. To the best of our knowledge, these cases are among the rare cases of childhood Still disease reported in sub-Saharan Africa. These cases highlight the importance of investigating non-infectious causes of persistent fever in children, in a context of infectious disease endemicity.
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