HLA-B27 Predicts a More Chronic Disease Course in an 8-year Followup Cohort of Patients with Juvenile Idiopathic Arthritis

2013 ◽  
Vol 40 (5) ◽  
pp. 725-731 ◽  
Author(s):  
Lillemor Berntson ◽  
Ellen Nordal ◽  
Kristiina Aalto ◽  
Suvi Peltoniemi ◽  
Troels Herlin ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Population Based ◽  
Hla B27 ◽  
Disease Characteristics ◽  
Serological Data ◽  
Longterm Outcome

Objective.We investigated associations of HLA-B27 with clinical manifestations and longterm outcome in a near population-based setting among patients with juvenile idiopathic arthritis (JIA).Methods.We studied clinical and serological data from 410 patients with HLA-B27 results among 440 prospectively collected patients with JIA with 8-year followup data in a Nordic database. The study was structured to be as close to a population-based study as possible.Results.HLA-B27 was analyzed in 93% of patients, and was positive in 21% of the cohort, in 18.4% of the girls and in 25.9% of the boys. Boys who were HLA-B27-positive had significantly higher age at onset compared to HLA-B27-negative boys and compared to both HLA-B27-negative and positive girls. This difference in onset age in relation to HLA-B27 was not found in girls. HLA-B27 was associated with clinical signs of sacroiliitis, enthesitis, and tenosynovitis in boys, but not in girls. After 8 years of disease, 46 children (11.2%) were classified as having enthesitis-related arthritis (ERA). Boys with ERA had clinical signs of sacroiliitis more often than girls with ERA. HLA-B27-positive children, as well as children with clinical signs of sacroiliitis, enthesitis, and hip arthritis, had higher odds of not being in remission off medication after 8 years of disease.Conclusion.In this near population-based Nordic JIA cohort we found significant differences between HLA-B27-positive boys and girls in age at disease onset, clinical signs of sacroiliitis, and ERA classification. HLA-B27 was negatively associated with longterm remission status, possibly because of its association with clinical disease characteristics, such as sacroiliitis, rather than being a general marker of persistent disease.

Adult systemic lupus erythematosus in Egypt: The nation-wide spectrum of 3661 patients and world-wide standpoint

Lupus ◽  
2021 ◽  
pp. 096120332110142
Author(s):  
Tamer A Gheita ◽  
Rasha Abdel Noor ◽  
Esam Abualfadl ◽  
Osama S Abousehly ◽  
Iman I El-Gazzar ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Age Of Onset ◽  
Wide Spectrum ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Population Based ◽  
Systemic Lupus ◽  
Cross Sectional

Objective The aim of this study was to present the epidemiology, clinical manifestations and treatment pattern of systemic lupus erythematosus (SLE) in Egyptian patients over the country and compare the findings to large cohorts worldwide. Objectives were extended to focus on the age at onset and gender driven influence on the disease characteristics. Patients and method This population-based, multicenter, cross-sectional study included 3661 adult SLE patients from Egyptian rheumatology departments across the nation. Demographic, clinical, and therapeutic data were assessed for all patients. Results The study included 3661 patients; 3296 females and 365 males (9.03:1) and the median age was 30 years (17–79 years), disease duration 4 years (0–75 years) while the median age at disease onset was 25 years (4–75 years). The overall estimated prevalence of adult SLE in Egypt was 6.1/100,000 population (1.2/100,000 males and 11.3/100,000 females).There were 316 (8.6%) juvenile-onset (Jo-SLE) and 3345 adult-onset (Ao-SLE). Age at onset was highest in South and lowest in Cairo (p < 0.0001). Conclusion SLE in Egypt had a wide variety of clinical and immunological manifestations, with some similarities with that in other nations and differences within the same country. The clinical characteristics, autoantibodies and comorbidities are comparable between Ao-SLE and Jo-SLE. The frequency of various clinical and immunological manifestations varied between gender. Additional studies are needed to determine the underlying factors contributing to gender and age of onset differences.

Temporomandibular Joint Involvement in Children with Juvenile Idiopathic Arthritis

2010 ◽  
Vol 38 (3) ◽  
pp. 510-515 ◽  
Author(s):  
ELVIRA CANNIZZARO ◽  
SILKE SCHROEDER ◽  
LUKAS M. MÜLLER ◽  
CHRISTIAN J. KELLENBERGER ◽  
ROTRAUD K. SAURENMANN
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Temporomandibular Joint ◽  
Upper Extremity ◽  
Age At Onset ◽  
Disease Onset ◽  
Joint Involvement ◽  
Hla B27 ◽  
Radiological Findings ◽  
Rheumatology Clinic ◽  
Younger Age

Objective.To determine the rate of temporomandibular joint (TMJ) involvement and find factors associated with TMJ arthritis in a single-center cohort of patients with juvenile idiopathic arthritis (JIA).Methods.Retrospective analysis of all patients with JIA visiting the rheumatology clinic between January 1, 2005, and December 31, 2006. Followup information was included until August 2008. A diagnosis of TMJ arthritis was based on clinical rheumatological and/or radiological findings.Results.After a mean followup time for JIA of 4.6 years (range 0.08–14.17), 86/223 patients (38.6%) had developed TMJ arthritis. The rate of TMJ involvement differed significantly among JIA subtypes (p = 0.0016), with 61% in extended oligoarticular, 52% in polyarticular rheumatoid factor (RF)-negative, 50% in psoriatic, 36% in systemic, 33% in polyarticular RF-positive, 33% in persistent oligoarticular, 30% in unclassified JIA, and 11% in enthesitis-related arthritis. The rate of TMJ involvement in our cohort was statistically significantly lower for patients who were HLA-B27-positive (p = 0.0002). In a multivariate analysis, the association of the following factors was confirmed: JIA subtype (p = 0.0001), a higher erythrocyte sedimentation rate (ESR) at diagnosis (p = 0.0038), involvement of joints of the upper extremity (p = 0.011), the absence of HLA-B27 (p = 0.023), and younger age at onset of JIA (p = 0.050).Conclusion.In our cohort of children with JIA, the overall rate of TMJ involvement was 38.6%. Patients with certain JIA subtypes, a higher ESR at disease onset, involvement of upper extremity joints, and younger age at diagnosis were more likely to develop TMJ arthritis. The presence of HLA-B27 seemed to be protective.

scholarly journals SAT0495 LONG TERM OUTCOME OF JUVENILE IDIOPATHIC ARTHRITIS IN ADULTHOOD; THE MONOCENTRIC EXPERIENCE OF 520 PATIENTS FOLLOWED FOR 20 YEARS IN A TRANSITION TERTIARY CLINIC OF PEDIATRIC RHEUMATOLOGY.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1204.2-1204
Author(s):  
I. Pontikaki ◽  
S. Carbogno ◽  
F. Corona ◽  
A. Petaccia ◽  
R. Cimaz
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Educational Level ◽  
Disease Duration ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Term Outcome ◽  
Long Term Outcome

Background:Juvenile Idiopathic Arthritis (JIA) is a chronic pediatric inflammatory disease that shows many differences compared to adult-onset arthritis. The different clinical manifestations, the assessment and the management of JIA is the reason that the transition from childhood to adulthood is an important multidimensional process that emphasizes a lot of aspects.Objectives:To describe the long-term outcome of JIA.Methods:Five-hundred and twenty patients affected by JIA and referred to a transition care rheumatology tertiary centre were considered between 1999 and 2019. The outcome assessment included remission, disease duration, medications, number of prosthesis implantation, pregnancies, mortality and social integration (employment status and educational level).Results:A hundred and thirty-eight (26%) males and 382 (73%) females were included; 157 (30%) patients were lost to follow up. The mean age of the patients was 27 (18-57) years, with a mean age at onset of 8 years and an average disease duration of 19 years. Subtypes of JIA at disease onset included 252 (48%) oligoarthritis, 134 (26%) polyarthritis, 64 (12%) systemic arthritis, 22 (4%) psoriatic arthritis, 43 (8%) enthesitis related arthritis and 1 (0.1%) undifferentiated arthritis. Ninty-three (18%) patients suffered of uveitis. Ninty-five implant prosthesis and 16 arthrodesis were recorded. At follow up 198 (38%) patients were on remission of which 107 (20%) off medication. Among the 322 patients still on medication, 84 (16%) were under treatment with oral steroids, 226 (43%) with sDMARDs and 249 (40%) with bDMARDs. Five deaths (1%) occurred in this cohort. Two hundred and thirty-five subjects had a higher educational level, 327 had an employment. We have data of twenty-nine pregnancies. The transition age was considered after the age of 16 years old. The key word for the management of this cohort was the multidisciplinary approach towards each patient, with the collaboration of other specialists (ophthalmologist, orthopedic, dermatologist, gastroenterologist, obstetric and psychologist).Conclusion:In the era of biologic therapy the long-term outcome of JIA underwent an outstanding improvement regarding a lot of variables. Two hundred and thirty-two patients were still followed, not only because of the continuation of the biological therapy, but also for a multidisciplinary care even during remission. JIA often persists over the adulthood, therefore the long term follow-up and care of these patients needs to be conducted by a rheumatologist expertized in JIA in collaboration with other specialists.Disclosure of Interests:None declared

scholarly journals Intravascular large cell lymphoma: Clinicopathological and immunohistochemical description of a case

2003 ◽  
Vol 11 (1) ◽  
pp. 21-24 ◽  
Author(s):  
Branko Dozic ◽  
Dubravka Cvetkovic ◽  
Marko Dozic ◽  
Ljiljana Bumbasirevic
Keyword(s):  
Blood Vessels ◽  
Laboratory Tests ◽  
Viral Encephalitis ◽  
Disease Onset ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Lymphoid Cells ◽  
Clinical Feature ◽  
Low Grade ◽  
X Rays

A 51-year-old female patient was admitted to the Neurological clinic because of motor seizures with myoclonus of the right hand and right side of the face. The results of initial brain CT scan, chest X rays, EEG ultrasonography of the great blood vessels and laboratory tests made in another hospital were unremarkable. Because of repeated partial seizures transient aphasic disturbances, urinary sphincter disturbances and periodic low-grade fever the patient was transferred to our hospital four months after the disease onset. Laboratory tests and NMR suggested a nonspecific disseminated viral encephalitis. After administration of Endoxan she was ambulatory for several weeks and then became increasingly exhausted confused, febrile, dyspneic, tachypneic and developed a shock status with hepatorenal insufficiency. She died after 7 months of disease duration. Postmortem examination revealed intravascular collections of large atypical lymphoid cells of B cell line. Blood vessels changed in this way were common in the brain and rare in other organs including skin, lungs, heart, liver spleen and digestive system. They were not found in the lymph nodes and bone marrow. A biopsy was not done because of absence of symptomatic and swollen tissues. However, correlation of clinical feature and postmortem findings shows that absence of clinical manifestations in an organ does not mean lack of microscopic pathological changes and biopsy should be done regardless of absence of clinical signs. This case shows that intravascular lymphoma may mimic vasculitis or disseminated nonspecific viral encephalitis.

scholarly journals Baseline disease characteristics in Brazilian patients enrolled in Transthyretin Amyloidosis Outcome Survey (THAOS)

2019 ◽  
Vol 77 (2) ◽  
pp. 96-100 ◽  
Author(s):  
Márcia Waddington Cruz ◽  
Marcus Vinicius Pinto ◽  
Luiz Felipe Pinto ◽  
Renata Gervais ◽  
Moisés Dias ◽  
...  
Keyword(s):  
Family History ◽  
Symptom Onset ◽  
Correct Diagnosis ◽  
Age At Onset ◽  
Positive Family History ◽  
Clinical Manifestations ◽  
Familial Amyloid Polyneuropathy ◽  
Transthyretin Amyloidosis ◽  
Disease Characteristics ◽  
Amyloid Polyneuropathy

ABSTRACT Transthyretin amyloidosis (ATTR) is characterized by the deposit of mutant or wild-type transthyretin that forms amyloid fibrils, which are extracellularly deposited within tissues and organs. Clinical manifestations of familial amyloid polyneuropathy vary according to the mutation, age at onset and geographical location. This study aimed to describe baseline disease characteristics of Brazilian patients with transthyretin familial amyloid polyneuropathy (ATTR-FAP) enrolled in the Transthyretin Amyloidosis Outcome Survey (THAOS). Methods: The THAOS is an international, noninterventional, longitudinal, observational, web-based registry designed to characterize ATTR. The outcome measures included demographics (age at symptom onset, gender, time from onset of symptoms to diagnosis, family history), genotype, and clinical characteristics (presence of amyloid deposit, frequency of misdiagnosis, presenting symptomatology). The analysis was conducted in a dataset from Brazilian patients (from November 2008 to January 2016). Results: One hundred and sixty participants (52.5% male) were included in the analysis. The majority of participants (90.6%) reported a positive family history of ATTR-FAP Median age at symptom onset was 32.5 years. Val30Met mutation was found in 91.9%. Misdiagnosis was observed in 26.6% of symptomatic patients. Over one-third (35.3%) of the misdiagnosed patients experienced a delay of more than one year before receiving a correct diagnosis. At presentation, 79.7% of the patients had motor, 87.5% sensory and 93.8% autonomic symptoms. Conclusion: ATTR-FAP in Brazil starts early, has a strong family history and the majority has Val30Met mutation. Misdiagnosis is common and the most common presentation is of a sensorimotor and autonomic neuropathy.

Age at onset as a determinant of presenting phenotype and initial relapse recovery in multiple sclerosis

2011 ◽  
Vol 18 (1) ◽  
pp. 45-54 ◽  
Author(s):  
M Cossburn ◽  
G Ingram ◽  
C Hirst ◽  
Y Ben-Shlomo ◽  
TP Pickersgill ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Late Onset ◽  
Age At Onset ◽  
Disease Onset ◽  
Complete Recovery ◽  
Population Based ◽  
Disease Course ◽  
Index Event ◽  
Relapsing Remitting ◽  
Age Dependent

Background: Age at onset modifies prognosis in multiple sclerosis (MS) and may also exert an effect on the characteristics of disease ignition. Understanding how age influences presentation informs disease management and may allow differentiation of distinct clinical sub-groups. Objectives: To determine the nature of age-specific presentations of relapsing–remitting MS (RRMS) with respect to onset symptoms, gender ratios and index event outcomes. Methods: In a prospective, population-based sample of 1424 patients in South-East Wales we examined associations between age at onset, clinical features and outcome of the onset event, making specific comparisons between paediatric, adolescent and late-onset MS. Results: Age at onset varied significantly between sexes (Male 31.2, Female 29.3, p = 0.002), 0.7% had paediatric onset, 2.7% adolescent onset and 2.8% late-onset MS (>50 years). Optic neuritis was common in younger patients and declined after age 30. Lower limb motor, facial sensory, sexual and sphincteric symptoms rose with age independent of sex and disease course. F:M ratios were highest <16 years of age and declined with increasing age, with a male excess in those over 50. Probability of complete recovery from index event declined with age from 87.4% in the youngest group to 68% in the eldest ( p = 0.009). Conclusions: Age at disease onset in RRMS exerts a significant effect on gender ratios and presenting phenotype, and allows identification of specific clinical sub-groups. In addition, ability to recover from initial relapse declines with age, suggesting accumulation of disability in MS is an age-dependent response to relapse.

P035 Hip involvement in Tunisian Juvenile Idiopathic Arthritis patients

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Makhlouf Yasmine ◽  
Wafa Triki ◽  
Kaouther Maatallah ◽  
Hanene Ferjani ◽  
Dorra Ben Nessib ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Rheumatoid Factor ◽  
Age At Onset ◽  
Joint Damage ◽  
Joint Effusion ◽  
Male Predominance ◽  
Disease Characteristics ◽  
Tunisian Patients ◽  
The Mean ◽  
Magnetic Resonance Imaging Mri

Abstract Background Juvenile idiopathic arthritis (JIA) is characterized by a widely variable clinical course and outcome. If uncontrolled, joint damage may occur. In this context, coxitis is a feared complication. The aim of our study was to determine the prevalence and patterns of hip involvement in Tunisian JIA patients. Methods A retrospective study including children with JIA according to the International League of Associations for Rheumatology (ILAR)) was conducted between 2012and 2021. Sociodemographic data as well as disease characteristics were collected. Laboratory markers including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were recorded. Hip involvement was assessed clinical exam and imaging (standard radiograph, ultrasound or magnetic resonance imaging (MRI)). We compared these parameters between the two groups: G1: presence of coxitis and G2: absence of coxitis. Results The study included 40 patients with a male predominance: sex ratio was 1.6. The mean age was 11.1 years-old [3–16]. The mean age at onset of the disease was 18.1 years old [8–30]. The distribution of the different subsets was as follows: polyarticular with rheumatoid factor (n = 1), polyarticular without rheumatoid factor (n = 2), enthesitis-related arthritis (n = 29), oligoarthritis (n = 7), psoriatic arthritis (n = 1). Extra-articular manifestations were found in 21.2% of cases: ocular (n = 4), pulmonary (n = 2) and cardiovascular (n = 1). The mean ESR and CRP was 30.9 mm/h [2–90] and 15.8 mg/l [1–70] respectively. A high ESR or CRP were found in 67% of cases. Hip involvement concerned 70% of the patients and was bilateral in 67.9% of them. Hip radiographs were normal in 50% of cases. Ultrasound was performed in 9 patients and revealed a positive Doppler synovitis (n = 2), a negative Doppler synovitis (n = 7) and joint effusion (n = 2). MRI was performed in 20% of cases and revealed synovitis (67%) and joint effusion (33%). Overall, 79.3% of patients had medical treatment combining NSAIDs and rehabilitation, 39% of the patients had had local infiltration with Hexatrione and only two patients had hip replacement. Hip involvement was not correlated with age at onset (P = 0.2), subtype (P = 0.8), sex (P = 0.7), extraarticular manifestations (P = 0.4). Similarly, there was no correlation between the presence of coxitis and ESR (P = 0.07) as well as CRP (P = 0.5). Conclusion Our study showed that hip involvement is frequent among Tunisian patients with JIA. Although not correlated with disease characteristics, hip involvement should be assessed frequently and carefully.

P036 Juvenile Idiopathic Arthritis in adulthood: outcomes of oligo and polyarticular forms?

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Miladi Saoussen ◽  
Makhlouf Yasmine ◽  
Fazaa Alia ◽  
Sellami Mariem ◽  
Ouenniche Kmar ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Transition To Adulthood ◽  
Disease Onset ◽  
Adult Life ◽  
Treatment Modalities ◽  
Disease Characteristics ◽  
Female Predominance ◽  
Tunnel Syndrome ◽  
The Mean ◽  
International League

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory rheumatic disease of childhood that can cause major physical disability, impairing patients' ability to lead a normal adult life. The objective of this study was to assess the outcomes of oligo- and polyarticular JIA in adulthood as well as the progression to authentic rheumatoid arthritis (RA). Methods We conducted a retrospective study including adult patients with JIA (diagnosed according to the International League of Associations for Rheumatology [ILAR]). Data collected included age, gender, disease characteristics (JIA subtype, disease duration). The progression to an authentic RA was determined by checking the response to the ACR/EULAR 2010 classification criteria. Results Twenty-eight patients were included in this study. There was a female predominance with a sex ratio of 1.5. The mean age of disease onset was 6 years and 2 months [2–17]. The mean age at inclusion was 29.5 years [18–64]. The frequency of each JIA subtype was as follows: polyarticular with RF + (n = 14), polyarticular with RF- (n = 9), oligoarthritis (n = 5). The polyarticular forms: RF+ and RF- evolved into genuine seropositive and seronegative RA in 71.4% and 66.7% of cases, respectively. Among the oligoarticular JIA subtype, an extension of the disease to a seronegative RA was noted in one patient (20%). Hip involvement was noted in 20% of patients. Carpal tunnel syndrome and elbow arthritis were found in 28.5% and 14.3% of patients, respectively. Regarding treatment modalities and at the time of recruitment, 36.3% of RAs were treated with Methotrexate, and 33% were on biological treatment. Ten percent of the patients dropped out of school because of disease flares and deformities. Conclusion Our study showed oligo- and polyarticular JIA evolves in most of the cases towards an authentic RA causing deformities and handicaps. Earlier management is necessary to allow a better transition to adulthood.

scholarly journals HLA-B27 predicts a more extended disease with increasing age at onset in boys with juvenile idiopathic arthritis

2008 ◽  
Vol 6 (S1) ◽  
Author(s):  
L Berntson ◽  
M Damgård ◽  
B Andersson-Gäre ◽  
T Herlin ◽  
S Nielsen ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Age At Onset ◽  
Hla B27

scholarly journals Patterns of pain over time among children with juvenile idiopathic arthritis

2017 ◽  
Vol 103 (5) ◽  
pp. 437-443 ◽  
Author(s):  
Amir Rashid ◽  
Lis Cordingley ◽  
Roberto Carrasco ◽  
Helen E Foster ◽  
Eileen M Baildam ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Multinomial Logistic Regression ◽  
Age At Onset ◽  
Management Strategies ◽  
Disease Onset ◽  
Common Symptom ◽  
Pain Outcomes ◽  
First Pain ◽  
Over Time

ObjectivesPain is a very common symptom of juvenile idiopathic arthritis (JIA). Disease activity alone cannot explain symptoms of pain in all children, suggesting other factors may be relevant. The objectives of this study were to describe the different patterns of pain experienced over time in children with JIA and to identify predictors of which children are likely to experience ongoing pain.MethodsThis study used longitudinal-data from patients (aged 1–16 years) with new-onset JIA. Baseline and up to 5-year follow-up pain data from the Childhood Arthritis Prospective Study (CAPS) were used. A two-step approach was adopted. First, pain trajectories were modelled using a discrete mixture model. Second, multinomial logistic regression was used to determine the association between variables and trajectories.ResultsData from 851 individuals were included (4 years, median follow-up). A three-group trajectory model was identified: consistently low pain (n=453), improved pain (n=254) and consistently high pain (n=144). Children with improved pain or consistently high pain differed on average at baseline from consistently low pain. Older age at onset, poor function/disability and longer disease duration at baseline were associated with consistently high pain compared with consistently low pain. Early increases in pain and poor function/disability were also associated with consistently high pain compared with consistently low pain.ConclusionsThis study has identified routinely collected clinical factors, which may indicate those individuals with JIA at risk of poor pain outcomes earlier in disease. Identifying those at highest risk of poor pain outcomes at disease onset may enable targeted pain management strategies to be implemented early in disease thus reducing the risk of poor pain outcomes.

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