Registries in rheumatological and musculoskeletal conditions. Paediatric Behcet's disease: an international cohort study of 110 patients. One-year follow-up data

Koné‐Paut, Isabelle; Darce-Bello, Martha; Shahram, Farhad; Gattorno, Marco; Cimaz, Rolando; Özen, Seza; Cantarini, Luca; Tugal-Tutktun, I.; Assaad-Khalil, S.; Hofer, M; Kuemmerle‐Deschner, Jasmin; Benamour, S.; Mayouf, S. Al; Pajot, C.; Antón, J.; Faye, Albert; Bono, W.; Nielsen, Susanne Dam; Letierce, Alexia; Tran, Tú-Anh

doi:10.1093/rheumatology/keq324

Cited by 70 publications

(48 citation statements)

References 17 publications

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“…Behçet's syndrome (BS) is a systemic, chronic relapsing vasculitis, typically characterized by recurrent oro-genital ulcers, ocular inflammation and skin manifestations; articular, vascular, gastro-enteric and neurological involvement may also occur [1][2][3]. Even though the aetiology of BS is still unknown [4][5][6], recently, the combination of clinical features, lack of autoantibodies and beneficial effects derived from IL-1 inhibition has reinvigorated the concept of BS as an autoinflammatory disease [7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Development of de novo major involvement during follow-up in Behçet’s syndrome

et al. 2015

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The primary aim of the study was to evaluate the incidence of de novo major involvement during follow-up in a cohort of patients with Behçet's syndrome (BS); the secondary aim was to analyse the epidemiological profile and the long-term outcome of those patients who developed new major involvement. Among our cohort of 120 BS patients, we evaluated all subjects who had no major organ involvement during the early years of their disease; specifically, at disease onset, the 52% of the cohort presented a prevalent mucocutaneous involvement. The primary outcomes were represented by the following: Hatemi et al. (Rheum Dis Clin North Am 39(2):245-61, 2013) the incidence of de novo major involvement during the follow-up and Hatemi et al. (Clin Exp Rheumatol 32(4 Suppl 84):S112-22, 2014) the use of immunosuppressive drugs during the follow-up. We have defined the development of de novo major involvement during the follow-up as the occurrence of severe ocular, vascular or CNS involvement after a latency period from the diagnosis of at least 3 years. Among 62 patients characterized by a mild onset of disease, we observed that after at least 3 years from the diagnosis, 21 BS patients (34%) still developed serious morbidities. Specifically, three patients developed ocular involvement, nine patients developed neurological involvement and nine patients presented vascular involvement. Comparing main epidemiological and clinical findings of the two groups, we observed that patients who developed de novo major involvement were more frequently males and younger; furthermore, 95% of these patients were characterized by a young onset of disease (p < 0.001). Being free of major organ complication in the first years of BS is not necessary a sign of a favourable outcome. Globally, the development of de novo major involvement during the coursfce of BS suggests that a tight control is strongly recommended during the course of the disease.

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Section: Introductionmentioning

confidence: 99%

Development of de novo major involvement during follow-up in Behçet’s syndrome

et al. 2015

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“…Our study results suggest that more than one clinical phenotype is present in patients with MWS, complicating the effort of developing diagnostic criteria. Koné-Paut et al recently suggested more than one set of criteria is required for Behçet's disease, a polygenetic autoinflammatory disease (36), since adult criteria perform poorly in the pediatric population.…”

Section: Discussionmentioning

confidence: 99%

Challenges in Diagnosing Muckle‐Wells Syndrome: Identifying Two Distinct Phenotypes

Kuemmerle‐Deschner

Samba

Tyrrell

et al. 2014

Arthritis Care & Research

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Objective. The diagnosis of Muckle-Wells syndrome (MWS) remains challenging due to the clinical heterogeneity and lack of diagnostic criteria. The aims of this study were to describe key elements of the diagnostic evaluation process in MWS and compare identified variables between patients diagnosed in childhood and adulthood. Methods. A cohort study of consecutive patients with a clinical and genetic diagnosis of MWS was conducted at 2 reference centers for autoinflammatory diseases. Demographic information, clinical presentation, access to care, and preclinical evaluation variables were captured. Presenting symptoms were compared between groups of patients diagnosed in childhood and adulthood. Prediction analysis explored variables associated with late diagnosis. Correspondence analysis identified clinical phenotypes. Results. A total of 34 MWS patients were included (16 males, 18 females) and median age at diagnosis was 31.5 years (range 0.5-75 years). Patients diagnosed during childhood reported musculoskeletal symptoms (62%), rash (62%), fever (54%), and abdominal pain (31%). Those diagnosed as adults described musculoskeletal symptoms (86%), rash (67%), hearing loss (52%), and fatigue (29%). Hearing loss was associated with late diagnosis, while access-to-care variables were not predictive. Correspondence analysis identified distinct clinical phenotypes as follows: an "inflammatory phenotype" (most commonly seen in patients diagnosed in childhood and characterized by relapsing fever and abdominal pain), an intermediate phenotype, and an "organ-disease" phenotype in patients diagnosed during adulthood and characterized by fatigue and hearing loss. Conclusion. Distinct clinical phenotypes were identified in patients with MWS. These are closely related to age at diagnosis. The presence of these phenotypes has to be considered when developing diagnostic criteria for MWS.

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“…The onset of disease occurs before 16 years in 4-26% of the patients with Behçet's disease. Although it is observed with a two-fold higher frequency in male patients in adults, gender difference is not observed in children (53,54). However, neurological involvement in Behçet's disease is observed with a 5.5-fold higher frequency in male patients (53).…”

Section: Behçet's Diseasementioning

confidence: 92%

“…Uveitis is mostly in the form of panuveitis and is observed more frequently and has a more severe prognosis in male patients. In addition, it has been reported that genital ulcers are observed more frequently in girls (54).…”

Section: Behçet's Diseasementioning

confidence: 99%

Diagnostic approach and current treatment options in childhood vasculitis

et al. 2015

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All inflammatory changes in the vessel wall are defined as vasculitis. Pediatric vasculitis may present with different clinical findings. Although Henoch-Schön-lein purpura which is the most common pediatric vasculitis generally recovers spontaneously, it should be monitorized closely because of the risk of renal failure. Although Kawasaki disease is easy to diagnose with its classical findings, the diagnosis may be delayed in case of incomplete Kawasaki disease. Kawasaki disease should be considered especially in infants in case of prolonged fever even if the criteria are not fully met and intravenous immunoglobulin treatment should be administered without delay in order to prevent development of coronary artery aneurism. Reaction at the site of administration of Bacillus Calmette-Guerin (BCG) vaccine may be observed as commonly as cervical lymphadenopathy in Kawasaki disease and may be used as a valuable finding in suspicious cases. Although anti-neutrophil cytoplasmic antibody-associated vasculitides are rare in children, renal involvement is more common and progression is more severe compared to adults. Hence, efficient and aggressive treatment is required. Takayasu's arteritis is observed commonly in young adult women and rarely in adolescent girls. Therefore, a careful physical examination and blood pressure measurement should be performed in addition to a detailed history in daily practice. In children with unexplained neurological findings, cerebral vasculitis should be considered in the absence of other systemic vasculitides and necessary radiological investigations should be performed in this regard. This review will provide an insight into the understanding of pediatric vasculitis, current diagnostic approaches and prognosis by the aid of new studies. (Turk Pediatri Ars 2015; 50: 194-205)

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Registries in rheumatological and musculoskeletal conditions. Paediatric Behcet's disease: an international cohort study of 110 patients. One-year follow-up data

Abstract: The expert committee has classified the majority of patients in the BD group although they presented with few symptoms independently of BD classification criteria.

Cited by 70 publications

References 17 publications

Development of de novo major involvement during follow-up in Behçet’s syndrome

Development of de novo major involvement during follow-up in Behçet’s syndrome

Challenges in Diagnosing Muckle‐Wells Syndrome: Identifying Two Distinct Phenotypes

Diagnostic approach and current treatment options in childhood vasculitis

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