scholarly journals Epidemiology of pediatric uveitis and associated systemic diseases

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Yoonkyeom Shin ◽  
Ji-Man Kang ◽  
Junwon Lee ◽  
Christopher Seungkyu Lee ◽  
Sung Chul Lee ◽  
...  
Keyword(s):  
Rheumatic Disease ◽  
Inflammatory Disease ◽  
Pediatric Patients ◽  
Age At Onset ◽  
Laboratory Data ◽  
Categorical Variables ◽  
Hla B27 ◽  
Female Ratio ◽  
Systemic Rheumatic Disease ◽  
Systemic Inflammatory Disease

Abstract Background The early detection of uveitis associated with systemic inflammatory disease in children is important for proper treatment and prognosis. However, the diagnosis may be delayed because of difficulties in childhood examinations and early minor systemic symptoms. The objective of our study was to identify the pattern of childhood uveitis and investigate the frequency and clinical features of rheumatic diseases in pediatric patients with uveitis. Methods This retrospective observational study reviewed the medical records of children (age ≤ 18 years) with uveitis at a Korean tertiary hospital between January 2005 and December 2018. Data collected included the age at onset of uveitis, sex, anatomic location of ocular inflammation, comorbid disease (including systemic inflammatory disease), ocular complications, relevant laboratory data, and treatment. Fisher’s exact test was used to compare categorical variables and the Mann–Whitney U test was used to compare continuous variables. A p-value of < 0.05 was considered statistically significant. Results A total of 155 pediatric patients with uveitis were included in this study. The median age at diagnosis was 13.0 years (interquartile range, 9.5–16.0 years). The male-to-female ratio was 1.09. The process was unilateral in 51.6% of children. Anterior uveitis, panuveitis, intermediate uveitis, and posterior uveitis represented 51.6, 26.5, 6.5, and 1.9% of the cases, respectively. Idiopathic uveitis (65.2%) was the most frequent type of uveitis. Systemic rheumatic disease associations were responsible for 28.4% of the cases, among which juvenile idiopathic arthritis (JIA) was the most frequent cause (14.8%). Human leukocyte antigen (HLA)-B27 and antinuclear antibody (ANA) positive rates were significantly higher in patients with JIA than in those with idiopathic uveitis (p = 0.006 and p = 0.007, respectively). Conclusions Approximately one-third of children with uveitis in Korea have a systemic rheumatic disease, of which JIA accounts for the majority of cases. HLA-B27 and ANA can serve as risk factors for JIA-associated uveitis.

scholarly journals Epidemiology of pediatric uveitis and associated systemic diseases

2020 ◽  
Author(s):  
Yoonkyeom Shin ◽  
Ji-Man Kang ◽  
Junwon Lee ◽  
Christopher Seungkyu Lee ◽  
Sung Chul Lee ◽  
...  
Keyword(s):  
Rheumatic Disease ◽  
Inflammatory Disease ◽  
Pediatric Patients ◽  
Age At Onset ◽  
Laboratory Data ◽  
Categorical Variables ◽  
Hla B27 ◽  
Female Ratio ◽  
Systemic Rheumatic Disease ◽  
Systemic Inflammatory Disease

Abstract Background: The early detection of uveitis associated with systemic inflammatory disease in children is important for proper treatment and prognosis. However, the diagnosis may be delayed because of difficulties in childhood examinations and early minor systemic symptoms. The objective of our study was to identify the pattern of childhood uveitis and investigate the frequency and clinical features of rheumatic diseases in pediatric patients with uveitis.Methods: This retrospective observational study reviewed the medical records of children (age ≤18 years) with uveitis at a Korean tertiary hospital between January 2005 and December 2018. Data collected included the age at onset of uveitis, sex, anatomic location of ocular inflammation, comorbid disease (including systemic inflammatory disease), ocular complications, relevant laboratory data, and treatment. Fisher’s exact test was used to compare categorical variables and the Mann–Whitney U test was used to compare continuous variables. A p-value of <0.05 was considered statistically significant.Results: A total of 155 pediatric patients with uveitis were included in this study. The median age at diagnosis was 13.0 years (interquartile range, 9.5–16.0 years). The male-to-female ratio was 1.09. The process was unilateral in 51.6% of children. Anterior uveitis, panuveitis, intermediate uveitis, and posterior uveitis represented 51.6%, 26.5%, 6.5%, and 1.9% of the cases, respectively. Idiopathic uveitis (65.2%) was the most frequent type of uveitis. Systemic rheumatic disease associations were responsible for 28.4% of the cases, among which juvenile idiopathic arthritis (JIA) was the most frequent cause (14.8%). Human leukocyte antigen (HLA)-B27 and antinuclear antibody (ANA) positive rates were significantly higher in patients with JIA than in those with idiopathic uveitis (p=0.006 and p=0.007, respectively).Conclusions: Approximately one-third of children with uveitis in Korea have a systemic rheumatic disease, of which JIA accounts for the majority of cases. HLA-B27 and ANA can serve as risk factors for JIA-associated uveitis.

scholarly journals Epidemiology of pediatric uveitis and associated systemic diseases

2020 ◽  
Author(s):  
Yoonkyeom Shin ◽  
Ji-Man Kang ◽  
Junwon Lee ◽  
Christopher Seungkyu Lee ◽  
Sung Chul Lee ◽  
...  
Keyword(s):  
Rheumatic Disease ◽  
Inflammatory Disease ◽  
Pediatric Patients ◽  
Laboratory Data ◽  
Screening Tools ◽  
Categorical Variables ◽  
Hla B27 ◽  
Female Ratio ◽  
Systemic Rheumatic Disease ◽  
Systemic Inflammatory Disease

Abstract Background: The early detection of uveitis associated with systemic inflammatory disease in children is important for proper treatment and prognosis. However, the diagnosis may be delayed because of difficulties in childhood examinations and early minor systemic symptoms. The objective of our study was to identify the pattern of childhood uveitis and investigate the frequency and clinical features of rheumatic diseases in pediatric patients with uveitis. Methods: This retrospective observational study reviewed the medical records of children (age ≤18 years) with uveitis at a Korean tertiary hospital between January 2005 and December 2018. Data collected included the age at onset of uveitis, sex, anatomic location of ocular inflammation, comorbid disease (including systemic inflammatory disease), ocular complications, relevant laboratory data, and treatment. Fisher’s exact test was used to compare categorical variables and the Mann–Whitney U test was used to compare continuous variables. A p-value of <0.05 was considered statistically significant. Results: A total of 155 pediatric patients with uveitis were included in this study. The median age at diagnosis was 13.0 years (interquartile range, 9.5–16.0 years). The male-to-female ratio was 1.09. The process was unilateral in 51.6% of children. Anterior uveitis, panuveitis, intermediate uveitis, and posterior uveitis represented 51.6%, 26.5%, 6.5%, and 1.9% of the cases, respectively. Idiopathic uveitis (65.2%) was the most frequent type of uveitis. Systemic rheumatic disease associations were responsible for 28.4% of the cases, among which juvenile idiopathic arthritis (JIA) was the most frequent cause (14.8%). Human leukocyte antigen (HLA)-B27 and antinuclear antibody (ANA) positive rates were significantly higher in patients with JIA than in those with idiopathic uveitis (p=0.006 and p=0.007, respectively). Conclusions: Approximately one-third of children with uveitis in Korea have a systemic rheumatic disease, of which JIA accounts for the majority of cases. HLA-B27 and ANA can be useful screening tools in children with JIA-associated uveitis.

scholarly journals Enthesitis Related Arthritis: A Single Center Experience

Acta Medica ◽  
2021 ◽  
pp. 1-6
Author(s):  
Selcan Demir ◽  
Müşerref Kasap Cüceoğlu ◽  
Yelda Bilginer
Keyword(s):  
Laboratory Data ◽  
Inflammatory Back Pain ◽  
Hla B27 ◽  
Female Ratio ◽  
Enthesitis Related Arthritis ◽  
Spinal Involvement ◽  
Therapeutic Decisions ◽  
Number Of Patients ◽  
Spinal Mri

Objective: Enthesitis-related arthritis is a subtype of juvenile idiopathic arthritis category, characterized by enthesitis, arthritis, and the risk of axial involvement. We aimed to summarize the demographics, clinical, and laboratory findings of enthesitis-related arthritis patients and to identify the distinguishing features of enthesitis-related arthritis patients with HLA B27 positive compared to the patients who were HLA B27 negative. Materials and Methods: This retrospective study included patients with Enthesitis-related arthritis who followed up between 2015 and 2018. Demographical, clinical, and laboratory data were retrospectively reviewed from the patient files and computerized medical charts. Results: A total of 72 patients diagnosed with enthesitis-related arthritis were included in the study. The male/female ratio was 2.1/1. Fifty-three (73%) of them presented with peripheral arthritis. The most commonly affected joint was knee (81.1%), followed by the ankle (43%), hips (32%), and wrist (5%). HLA B27 was positive in 36 (50%) patients. During follow-up, the number of patients who developed enthesitis-related arthritis -associated uveitis was 8 (11.1%). During follow-up, 56 patients with inflammatory back pain and/or sacroiliac tenderness underwent spinal MRI. Ten (17.8%) patients had only thoracal and/or lumbar involvement, 18 (32%) had only sacroiliitis, and 9 (16%) patients had both of them on spinal MRI. In comparison with HLA-B27-negative children, HLA-B27-positive patients were more likely to have enthesitis (16 (44.4%) vs 8 (22.2%), p=0.046), MRI proven sacroiliitis (19 (52.7%) vs 8 (22.2%), p=0.031), MRI proven spinal involvement (13 (36.1%) vs 6 (16.6%), p=0.031), and uveitis (8 (100%) vs 0(0%), p=0.014). During follow up, 65/72 (90.2 %) of them needed disease-modifying antirheumatic drugs (DMARD), and 51/72 (70.8%) needed anti-tumor necrosis factor-α (TNF-α) therapy. Conclusion: We found that patients who were HLA-B27- positive had significantly more enthesitis, MRI-proven sacroiliitis, MRI-proven spinal involvement, and acute anterior uveitis, in comparison to patients who were HLA B27 negative. It is crucial to carefully assess those patients with concern for enthesitis-related arthritis to determine the expected prognosis and make therapeutic decisions appropriately.

scholarly journals Younger age of onset and uveitis associated with HLA-B27 and delayed diagnosis in Thai patients with axial spondyloarthritis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Naphruet Limsakul ◽  
Praveena Chiowchanwisawakit ◽  
Parichart Permpikul ◽  
Yubolrat Thanaketpaisarn
Keyword(s):  
Regression Analysis ◽  
Multiple Regression Analysis ◽  
Axial Spondyloarthritis ◽  
Age At Onset ◽  
Delayed Diagnosis ◽  
Musculoskeletal Symptom ◽  
Hla B27 ◽  
Delay In Diagnosis ◽  
Factors Associated ◽  
Younger Age

AbstractTo identify characteristics associated with HLA-B27, and to identify factors associated with delayed diagnosis in Thai patients with axial spondyloarthritis (axSpA). This cross-sectional study included Thai patients were diagnosed with axSpA by a rheumatologist at Siriraj Hospital. Clinical data were collected. Regression analyses were employed to identify factors associated with study outcomes. Of total 177 patients, 127 (72%) were positive HLA-B27. Uveitis [Odds ratio (OR) 4.0], age at onset of the first musculoskeletal symptom of ≤ 28 years [OR 3.5], female [OR 0.4], and psoriasis [OR 0.4] were significantly associated with HLA-B27 in multiple regression analysis. Those with positive HLA-B27 had less spinal flexibility. Elevated C-reactive protein (p = 0.012) was associated with shorter delay in diagnosis, while uveitis (p < 0.001) and younger age at onset of the first symptom (p = 0.002) were associated with longer delay in diagnosis in multiple regression analysis. Younger age at onset of the first musculoskeletal symptom and uveitis were associated with HLA-B27 and delayed diagnosis in axSpA patients. Young people with musculoskeletal symptom and uveitis should be referred to a rheumatologist to rule out or make a timely diagnosis of axSpA.

scholarly journals Association of inflammatory disease and long-term outcomes among young adults with myocardial infarction: the Partners YOUNG-MI registry

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Weber ◽  
D.W Biery ◽  
A Singh ◽  
S Divakaran ◽  
A.N Berman ◽  
...  
Keyword(s):  
Risk Factors ◽  
Inflammatory Disease ◽  
Inflammatory Arthritis ◽  
Young Age ◽  
Funding Source ◽  
Long Term Outcomes ◽  
Increased Risk ◽  
Systemic Inflammatory Disease ◽  
All Cause Mortality

Abstract Background Autoimmune systemic inflammatory diseases are associated with an increased risk of cardiovascular disease, particularly myocardial infarction (MI). However, there are limited data on the prevalence and effects of inflammatory disease among U.S. adults who experience an MI at a young age. Purpose We sought to determine the prevalence and prognostic value of inflammatory disease in U.S. adults who experience an MI at a young age. Methods The YOUNG-MI registry is a retrospective cohort study of consecutive patients who experienced a Type 1 MI at or below the age of 50 years from 2000 to 2016 at two large medical centers. A diagnosis of rheumatoid arthritis (RA), psoriasis (PsO), systemic lupus erythematosus (SLE), or inflammatory arthritis was determined through physician review of electronic medical records (EMR). Demographic information, presence of cardiovascular (CV) risk-factors, medical procedures, and medications upon discharge were also ascertained from the EMR. Incidence of death was determined using a combination of EMR and national databases. Cox proportional hazard modeling was performed on a sub-sample following Mahalanobis Distance matching on age, sex, and CV risk factors. Results The cohort consisted of 2097 individuals (median age 45 years, 19% female, 53% ST-elevation MI). Among these, 53 (2.5%) individuals possessed a diagnosis of systemic inflammatory disease at or before their index MI (23% SLE, 9% RA, 64% PsO, 4% inflammatory arthritis). When compared to the remainder of the cohort, patients with a diagnosis of systemic inflammatory disease were more likely to be female (36% vs 19%, p=0.004) and be diagnosed with hypertension (62% vs 46%, p=0.025). There was, however, no significant difference in the prevalence of other CV risk factors – diabetes, smoking, dyslipidemia – or a family history of premature coronary artery disease. Despite these similarities, patients with inflammatory disease were less likely to be prescribed aspirin (88% vs 95%, p=0.049) or a statin (76% vs 89%, p=0.008) upon discharge. Over a median follow-up of 11.2 years, patients with inflammatory disease experienced an increased risk of all-cause mortality when compared with the full-cohort (Figure). Compared to the matched sample (n=138), patients with systemic inflammatory disease exhibited an increased risk of all-cause mortality (HR=2.68, CI [1.18 to 6.07], p=0.018), which remained significant after multivariable adjustment for length of stay and GFR (HR=2.38, CI [1.02 to 5.54], p=0.045). Conclusions Among individuals who experienced an MI at a young age, approximately 2.5% had evidence of a systemic inflammatory disease at or before their MI. When compared with a population of individuals with similar cardiovascular risk profiles, those with inflammatory disease had higher rates of all-cause mortality. Our findings suggest that the presence of a systemic inflammatory disorder is independently associated with worse long-term outcomes. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): 1. 5T32 HL094301 NIH T32 Training Grant, “Noninvasive Cardiovascular Imaging Research Training Program”

scholarly journals Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case–control study based on 450 patients

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Qiaozhu Zeng ◽  
Jingyuan Gao ◽  
Xinyu Zhang ◽  
Aichun Liu ◽  
Zhenfan Wang ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Clinical Laboratory ◽  
Imaging Finding ◽  
Age At Onset ◽  
Female Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Peking University ◽  
Chinese Cohort ◽  
Male To Female

AbstractWe aimed to compare the demographic, clinical and laboratory characteristics between IgG4-related kidney disease (IgG4-RKD+) and extrarenal IgG4-related disease (IgG4-RKD−) in a large Chinese cohort, as well as describing the radiological and pathological features of IgG4-RKD+. We retrospectively analyzed the medical records of 470 IgG4-related disease (IgG4-RD) patients at Peking University People’s Hospital from January 2004 to January 2020. The demographic, clinical, laboratory, radiological and pathological characteristics between IgG4-RKD+ and IgG4-RKD− were compared. Twenty IgG4-RD patients who had definite etiology of renal impairment including diabetes, hypertension and etc. were excluded. Among the remained 450 IgG4-RD patients, 53 were diagnosed with IgG4-RKD+ . IgG4-RKD+ patients had older age at onset and at diagnosis. Male to female ratio of IgG4-RKD+ patients is significantly higher. In the IgG4-RKD+ group, the most commonly involved organs were salivary gland, lymph nodes and pancreas. It was found that renal function was impaired in approximately 40% of IgG4-RKD+ patients. The most common imaging finding is multiple, often bilateral, hypodense lesions. Male sex, more than three organs involved, and low serum C3 level were risk factors for IgG4-RKD+ in IgG4-RD patients. These findings indicate potential differences in pathogenesis of these two phenotypes.

scholarly journals Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Marco Cattalini ◽  
◽  
Sara Della Paolera ◽  
Fiammetta Zunica ◽  
Claudia Bracaglia ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Clinical Laboratory ◽  
Troponin T ◽  
Inflammatory Diseases ◽  
Age At Onset ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Chi Square ◽  
Pediatric Society ◽  
Multicenter Survey

Abstract Background There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome-temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities. Methods The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group – KDG) or KD-like (Kawacovid Group - KCG) disease between February 1st 2020, and May 31st 2020. Demographic, clinical, laboratory data, treatment information, and patients’ outcome were collected in an online anonymized database (RedCAP®). Relationship between clinical presentation and SARS-CoV-2 infection was also taken into account. Moreover, clinical characteristics of KDG during SARS-CoV-2 epidemic (KDG-CoV2) were compared to Kawasaki Disease patients (KDG-Historical) seen in three different Italian tertiary pediatric hospitals (Institute for Maternal and Child Health, IRCCS “Burlo Garofolo”, Trieste; AOU Meyer, Florence; IRCCS Istituto Giannina Gaslini, Genoa) from January 1st 2000 to December 31st 2019. Chi square test or exact Fisher test and non-parametric Wilcoxon Mann-Whitney test were used to study differences between two groups. Results One-hundred-forty-nine cases were enrolled, (96 KDG and 53 KCG). KCG children were significantly older and presented more frequently from gastrointestinal and respiratory involvement. Cardiac involvement was more common in KCG, with 60,4% of patients with myocarditis. 37,8% of patients among KCG presented hypotension/non-cardiogenic shock. Coronary artery abnormalities (CAA) were more common in the KDG. The risk of ICU admission were higher in KCG. Lymphopenia, higher CRP levels, elevated ferritin and troponin-T characterized KCG. KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%; p = 0.04 and 71,9% vs 43,4%; p = 0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%; p < 0.0001). SARS-CoV-2 assay more often resulted positive in KCG than in KDG (75,5% vs 20%; p < 0.0001). Short-term follow data showed minor complications. Comparing KDG with a KD-Historical Italian cohort (598 patients), no statistical difference was found in terms of clinical manifestations and laboratory data. Conclusion Our study suggests that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD and PIMS-TS. Older age at onset and clinical peculiarities like the occurrence of myocarditis characterize this multi-inflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths.

ANAs in systemic rheumatic disease

1985 ◽  
Vol 78 (3) ◽  
pp. 141-148 ◽  
Author(s):  
Eng M. Tan ◽  
Charles A. Robinson ◽  
Robert M. Nakamura
Keyword(s):  
Rheumatic Disease ◽  
Systemic Rheumatic Disease

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.

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