Adult-onset Still's disease or systemic-onset juvenile idiopathic arthritis and spondyloarthritis: overlapping syndrome or phenotype shift?

OBJECTIVES: Systemic-onset JIA (SJIA) and adult-onset Still's disease (AOSD) are the same sporadic systemic auto-inflammatory disease. SpA is a group of inflammatory non-autoimmune disorders. We report the observations of eight patients with SJIA/AOSD who also presented features of SpA during their disease evolution and estimate the prevalence of SpA in SJIA/AOSD. METHODS: This was a retrospective national survey of departments of paediatric and adult rheumatology and internal medicine. To be included, SJIA patients had to fulfil the ILAR criteria, AOSD patients the Yamaguchi or Fautrel criteria, and all patients the Assessment of SpondyloArthritis International Society (ASAS) classification criteria for axial or peripheral SpA, ESSG criteria for SpA or Classification Criteria for Psoriatic Arthritis (CASPAR) criteria for PsA. The data were collected with a standardized form. RESULTS: Eight patients (five adults) were identified in one paediatric and two adult departments. In all but one patient, SpA manifestations occurred several years after SJIA/AOSD onset [mean (s.d.) delay 6.2 (3.8) years]. Two patients had peripheral and three axial SpA, and four later exhibited PsA and one SAPHO syndrome. The prevalence of SpA in an adult cohort of 76 patients with AOSD was 6.58% (95% CI 2.17, 14.69), greater than the prevalence of SpA in the French general population (0.3%; 95% CI 0.17, 0.46). The prevalence of SpA in an SJIA cohort of 30 patients was 10% (95% CI 2.11, 26.53), more than that reported in the general population of industrialized countries, estimated at 0.016-0.15%. CONCLUSION: While the temporal disassociation between SpA and AOSD in most cases might suggest a coincidental finding, our work raises the possibility of an SpA/AOSD spectrum overlap that needs further study.

Peripherals manifestations of spondyloarthritis in Guinea.

INTRODUCTION: SpA is a group of chronic inflammatory rheumatic diseases that mainly affect the axial skeleton, but may also associate peripheral manifestations such as arthritis, enthesitis and dactylitis. AIM: Describe the peripheral manifestations of SpA. METHODS: We conducted a descriptive cross-sectional study of SpA patients received at the rheumatology department of CHU Ignace Deen in Conakry over a one-year period from June 1, 2021 to May 31, 2022. The diagnosis of SpA was retained in accordance with the ASAS (Assessment of spondyloarthritis internationnal society) classification and data on peripheral manifestations were collected. RESULTS: A total of 275 patients met the criteria and 82 had at least one peripheral involvement (29.8%). The mean age was 52.3 years, with females predominating in 58 (70.5%) cases. The mean time to diagnosis was 11 years. The most frequent peripheral manifestation was peripheral joint involvement (78%), followed by peripheral enthesitis (36.6%). The predominant clinical form was axial in 51.2%, with HLA-B27 antigen present in 63% and sacroiliitis in 51.2%. Treatment was based on NSAIDs (67.1%). CONCLUSION: Peripheral manifestations are becoming increasingly frequent in SpA, and are dominated by peripheral joint involvement. The predominant clinical form was axial, with a strong association with HLA-B27.

[Still's disease in children and acute lymphoblastic leukemia, an exceptional association: a case report]. / Maladie de Still de l'enfant et leucémie aigüe lymphoblastique, une association exceptionnelle: à propos d'un cas.

Still's disease, also known as systemic juvenile idiopathic arthritis (SJIA), and acute lymphoblastic leukemia have similar clinical and biological features posing diagnostic and treatment challenges. Indeed, while Still's disease is a diagnosis of exclusion in rheumatology, polyarthritis associated with hyperleukocytosis and fever, which is characteristic of this disease, are often detected in early stages of acute lymphocytic leukaemias. We here report the case of a 4-year-old girl, treated for Still's disease, in whom the diagnosis of acute lymphoblastic leukemia was made after 2 months, based on bone marrow biopsy.

[Profile and quality of life of patients with rheumatoid arthritis in Guinea Conakry and Cameroon]. / Profil et qualité de vie de patients atteints de polyarthrite rhumatoïde en Guinée Conakry et au Cameroun.

INTRODUCTION: rheumatoid arthritis (RA) dramatically affects the quality of life of patients. The objective of our study was to study the link between the activity of the disease and the quality of life of Guinean (Conakry) and Cameroonian patients with RA. METHODS: pilot multicentric cross-sectional study (Ignace Dean National Hospital of Conakry in Guinea and Efoulan Yaoundé District Hospital in Cameroon) for 15 months (1st October 2016 to 30th January 2018). The diagnosis of RA was based on the criteria of the ACR/EULAR. Disease activity was assessed by DAS 28. The EMIR questionnaire and the Steinbrocker score were used to assess quality of life. RESULTS: fifty-two patients, 82% of whom were women. The total EMIR score was 5.06±0.50 as a relatively impaired quality of life. Alteration of quality of life was more marked on psychic components (6.78±0.99) and pain (5.37±0.99). The work component was the least affected (4.03±0.98). DAS28 was significantly related to psychic components (p=0.036, R=0.29), pain (p=0.076, R=0.25), physical (p=0.0029, R=0.41), and at the overall quality of life (total EMIR) (p=0.027, R=0.31). CONCLUSION: the most significant of RA on quality of life was related to pain (EVA-pain) and RA activity (DAS 28). The results of this pilot study will have to be confirmed by a largest study.