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  1. Article ; Online: Impact of follow-up adherence on disease activity in childhood-onset systemic lupus erythematosus (cSLE).

    Nelson, Meghan Corrigan / Mosley, Colleen / Villacis-Nunez, D Sofia / Rouster-Stevens, Kelly / Thakral, Amit

    Lupus

    2023  Volume 32, Issue 6, Page(s) 799–803

    Abstract: Background/purpose: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, with a potential for significant disease damage, morbidity, and mortality. In comparison to the adult population, childhood-onset SLE (cSLE) tends to be more ... ...

    Abstract Background/purpose: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, with a potential for significant disease damage, morbidity, and mortality. In comparison to the adult population, childhood-onset SLE (cSLE) tends to be more aggressive given the higher preponderance of renal and neuropsychiatric disease and increased disease activity. There is a paucity of literature examining relationship between disease activity, rheumatology follow-up visits, and health care utilization. The objective of this study is to determine whether adherence with outpatient clinic visits would affect disease activity in patients with childhood-onset systemic lupus erythematosus (cSLE).
    Methods: 41 children <18 years of age at time of diagnosis with SLE who met Systemic Lupus International Collaborative Clinics (SLICC) criteria and not evaluated in clinic within the previous 120-day period were identified as eligible for inclusion. Patients were continuously searched between December 2021 and July 2022 for eligibility evaluation. Through retrospective chart review, we assessed disease activity (SLE Disease Activity Index) at the last clinic visit. The patients were stratified into two cohorts of lower and higher disease activity, with SLE disease activity index (SLEDAI) ≤ 3 and SLEDAI ≥ 4, respectively. Descriptive statistics and Willcox Rank Sum (numerical variables) and Fisher's test (categorical variables) were used to compare these two groups.
    Results: Clinical, epidemiological, and serological data were compared between the two groups, with observed statistically significant differences to include current use of high dose prednisone associated with higher SLEDAI scores (
    Conclusion: Our findings suggest that cSLE patients with higher disease activity are at risk for increased health care utilization with respect to ED visits as well as hospitalizations in the setting of follow-up nonadherence. While further studies are required to enhance our understanding of this association, this links the importance of disease-related outcome and routine outpatient visits in this particularly vulnerable patient population.
    MeSH term(s) Child ; Adult ; Humans ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Erythematosus, Systemic/epidemiology ; Lupus Erythematosus, Systemic/diagnosis ; Retrospective Studies ; Follow-Up Studies ; Age of Onset ; Prednisone ; Severity of Illness Index
    Chemical Substances Prednisone (VB0R961HZT)
    Language English
    Publishing date 2023-04-26
    Publishing country England
    Document type Journal Article
    ZDB-ID 1154407-7
    ISSN 1477-0962 ; 0961-2033
    ISSN (online) 1477-0962
    ISSN 0961-2033
    DOI 10.1177/09612033231173530
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Severe Immediate and Delayed Hypersensitivity Reactions to Biologics in a Toddler With Systemic Juvenile Idiopathic Arthritis.

    Villacis-Nunez, D Sofia / Bilcha, Kassahun / Spraker, Mary / Rouster-Stevens, Kelly / Cooley, Anthony

    Journal of investigative medicine high impact case reports

    2022  Volume 10, Page(s) 23247096221077836

    Abstract: Many pediatric rheumatic diseases can be safely managed with biologic therapy. Severe allergic reactions to these medications are uncommon. We report the case of a 2-year-old male with systemic-onset juvenile idiopathic arthritis and secondary macrophage ...

    Abstract Many pediatric rheumatic diseases can be safely managed with biologic therapy. Severe allergic reactions to these medications are uncommon. We report the case of a 2-year-old male with systemic-onset juvenile idiopathic arthritis and secondary macrophage activation syndrome (MAS), whose treatment was complicated by severe allergic reactions to biologics, including drug reaction with eosinophilia and systemic symptoms (DRESS)/drug-induced hypersensitivity reaction (DIHR) likely due to anakinra, and anaphylactoid reaction to intravenous tocilizumab. These required transition to canakinumab, cyclosporine, and corticosteroids, with later development of interstitial lung disease and MAS flare needing transition from canakinumab to tofacitinib, which led to disease control. Whether lung disease is a manifestation of DRESS/DIHR to canakinumab remains unclear. High index of suspicion of hypersensitivity reactions for timely diagnosis and drug discontinuation is critical, especially in patients with active disease who might be at increased risk of these adverse events.
    MeSH term(s) Antirheumatic Agents/adverse effects ; Arthritis, Juvenile/complications ; Arthritis, Juvenile/drug therapy ; Biological Products/adverse effects ; Child, Preschool ; Humans ; Hypersensitivity/complications ; Hypersensitivity/drug therapy ; Hypersensitivity, Delayed/chemically induced ; Hypersensitivity, Delayed/complications ; Hypersensitivity, Delayed/drug therapy ; Macrophage Activation Syndrome/chemically induced ; Macrophage Activation Syndrome/complications ; Macrophage Activation Syndrome/drug therapy ; Male
    Chemical Substances Antirheumatic Agents ; Biological Products
    Language English
    Publishing date 2022-02-28
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 2710326-2
    ISSN 2324-7096 ; 2324-7096
    ISSN (online) 2324-7096
    ISSN 2324-7096
    DOI 10.1177/23247096221077836
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Longitudinal program evaluation of an inter-institutional mentorship network for pediatric rheumatology using a quality improvement framework.

    Hayward, Kristen / Grom, Alexi / Muscal, Eyal / Nigrovic, Peter A / Rouster-Stevens, Kelly A / Ardalan, Kaveh / Hiraki, Linda / Moorthy, L Nandini

    Research square

    2023  

    Abstract: Background: The American College of Rheumatology (ACR)/Childhood Arthritis and Rheumatology Research Alliance (CARRA) Mentoring Interest Group (AMIGO) is an inter-institutional mentorship program launched to target mentorship gaps within pediatric ... ...

    Abstract Background: The American College of Rheumatology (ACR)/Childhood Arthritis and Rheumatology Research Alliance (CARRA) Mentoring Interest Group (AMIGO) is an inter-institutional mentorship program launched to target mentorship gaps within pediatric rheumatology. Initial program evaluation indicated increased mentorship access. Given the small size of the pediatric rheumatology workforce, maintaining a consistent supply of mentors was a potential threat to the longevity of the network. Our aims were to: (i) describe the sustainability of AMIGO over the period 2011-2018, (ii) highlight ongoing benefits to participants, and (iii) describe challenges in the maintenance of a mentorship network.
    Methods: A mixed-methods approach centered on a quality improvement framework was used to report on process and outcomes measures associated with AMIGO annual cycles.
    Results: US and Canada Pediatric rheumatology workforce surveys identified 504 possible participants during the time period. As of fall 2018, 331 unique individuals had participated in AMIGO as a mentee, mentor or both for a program response rate of 66% (331/504). Survey of mentees indicated high satisfaction with impact on general career development, research/scholarship and work-life balance. Mentors indicated increased sense of connection to the community and satisfaction with helping mentees despite minimal perceived benefit to their academic portfolios. Based on AMIGO's success, a counterpart program, Creating Adult Rheumatology Mentorship in Academia (CARMA), was launched in 2018.
    Conclusions: Despite the challenges of a limited workforce, AMIGO continues to provide consistent access to mentorship opportunities for the pediatric rheumatology community. This experience can inform approaches to mentorship gaps in other academic subspecialties.
    Language English
    Publishing date 2023-12-12
    Publishing country United States
    Document type Preprint
    DOI 10.21203/rs.3.rs-3717708/v1
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: A single-center model for implementation of SLEDAI documentation adherence in childhood-onset systemic lupus erythematosus (cSLE).

    Nelson, Meghan Corrigan / Mosley, Colleen / Bennett, Tonya / Orenstein, Evan / Rouster-Stevens, Kelly

    Lupus

    2023  Volume 32, Issue 12, Page(s) 1447–1452

    Abstract: Background: Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune disease with variable disease expression but noted association with significant disease-related damage, morbidity, and mortality. The European Alliance of Associations for ... ...

    Abstract Background: Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune disease with variable disease expression but noted association with significant disease-related damage, morbidity, and mortality. The European Alliance of Associations for Rheumatology (EULAR) recommends routine monitoring of SLE through validated disease activity and chronicity indices, including the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Despite this, physician adherence with SLEDAI documentation remains elusive at various academic institutions. The aim of our study was to determine baseline SLEDAI documentation rates at our center and assess the change in adherence in SLEDAI documentation rate with electronic clinical decision support (CDS) reminders facilitated through the electronic medical record (EMR) over a 2-year period.
    Methods: All SLE encounters over a 24-month period at a pediatric academic center were reviewed in order to obtain baseline SLEDAI documentation percentages. Physicians subsequently received monthly email reminders, initiated at month 4 of project initiation, with subsequent CDS reminder 13 months after project initiation prompted by anti-dsDNA lab result. Chart review was repeated continuously for each provider, and SLEDAI documentation rates were emailed to each provider monthly. Physicians completed a post-intervention survey regarding barriers to SLEDAI documentation at the end of the study.
    Results: A total of 1980 SLE encounters were reviewed for this study. Baseline SLEDAI documentation rates were 10%. Following the introduction of monthly emails reminding physicians to document SLEDAI, rates increased to 55%. After the initiation of electronic in-basket reminders prompted by lab results, rates increased to 60%. Noted barriers to documentation were cited to be forgetfulness (67%) and lack of time (33%).
    Conclusion: Our study demonstrates that monthly email reminders as well as EMR-mediated electronic in-basket reminders increased SLEDAI documentation rates at an academic center. Noted barriers to documentation were reported to be forgetfulness (67%) and lack of time (33%).
    MeSH term(s) Child ; Humans ; Lupus Erythematosus, Systemic/diagnosis ; Age of Onset ; Severity of Illness Index
    Language English
    Publishing date 2023-10-09
    Publishing country England
    Document type Journal Article
    ZDB-ID 1154407-7
    ISSN 1477-0962 ; 0961-2033
    ISSN (online) 1477-0962
    ISSN 0961-2033
    DOI 10.1177/09612033231206451
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article: Improving Hepatitis B Screening Prior to Rituximab: A Quality Improvement Project.

    Villacis-Nunez, D Sofia / Orenstein, Evan / Selvaggio, Phyllis / Rouster-Stevens, Kelly / Wang, Chia-Shi / Thakral, Amit

    Children (Basel, Switzerland)

    2023  Volume 10, Issue 7

    Abstract: Rituximab, used in the treatment of some rheumatic and kidney diseases, can lead to hepatitis B virus (HBV) reactivation; HBV screening is recommended for those starting this medication. We aimed to improve by 50% the proportion of patients undergoing ... ...

    Abstract Rituximab, used in the treatment of some rheumatic and kidney diseases, can lead to hepatitis B virus (HBV) reactivation; HBV screening is recommended for those starting this medication. We aimed to improve by 50% the proportion of patients undergoing HBV screening by implementing multimodal interventions to support clinicians in this evidence-based practice. We conducted a quality improvement project from November 2020 to June 2022 at a tertiary care pediatric hospital system, including patients with rheumatic and/or kidney diseases starting rituximab. Multimodal interventions targeting clinicians included electronic health tools (dot phrase, display of screening recommendations and screening results in rituximab order sets/therapy plans), educational meetings, and e-mail/paper reminders. The primary outcome was the proportion of patients with complete HBV screening, while the secondary outcome was utilization of each laboratory component, tracked using statistical process control charts. Pre- and post-intervention data were compared using Fisher's test. One hundred eighty-two patients who had been prescribed rituximab were included, of which 98 (54%) were post-intervention. The proportions of patients undergoing complete HBV screening (6% vs. 44%;
    Language English
    Publishing date 2023-06-30
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2732685-8
    ISSN 2227-9067
    ISSN 2227-9067
    DOI 10.3390/children10071142
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Predictors for early readmission in patients hospitalized with new onset pediatric lupus nephritis.

    Ah Guerra, Angel / Garro, Rouba / McCracken, Courtney / Rouster-Stevens, Kelly / Prahalad, Sampath

    Lupus

    2021  Volume 30, Issue 12, Page(s) 1991–1997

    Abstract: Objective: The objective is to determine the 30-day hospital readmission rate following a hospitalization due to pediatric lupus nephritis of recent onset and characterize the risk factors associated with these early readmissions.: Methods: The study ...

    Abstract Objective: The objective is to determine the 30-day hospital readmission rate following a hospitalization due to pediatric lupus nephritis of recent onset and characterize the risk factors associated with these early readmissions.
    Methods: The study included 76 children hospitalized from 01/01/2008 to 4/30/2017 due to a new diagnosis of lupus nephritis. We calculated the 30-day hospital readmission rate and compared the characteristics of the patients that were readmitted to patients that were not readmitted using univariable and multivariable analysis.
    Results: The 30-day readmission rate was 17.1%. Factors that predicted hospital readmission in unavailable analysis were male gender (38.5 vs 14.3%,
    Conclusion: In all, 17% of children hospitalized due to new onset lupus nephritis were readmitted within 30 days of discharge. Absence of IVMP and receiving intravenous albumin assisted diuresis during initial hospitalization increase the risk of early readmission in new onset pediatric lupus nephritis.
    MeSH term(s) Adolescent ; Albumins/administration & dosage ; Albumins/adverse effects ; Child ; Female ; Hospitalization/statistics & numerical data ; Humans ; Infusions, Intravenous ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/epidemiology ; Lupus Nephritis/diagnosis ; Lupus Nephritis/drug therapy ; Lupus Nephritis/epidemiology ; Male ; Patient Readmission/statistics & numerical data ; Retrospective Studies ; Risk Factors ; Time Factors
    Chemical Substances Albumins
    Language English
    Publishing date 2021-09-16
    Publishing country England
    Document type Journal Article
    ZDB-ID 1154407-7
    ISSN 1477-0962 ; 0961-2033
    ISSN (online) 1477-0962
    ISSN 0961-2033
    DOI 10.1177/09612033211044648
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Clinical Determinants of Childhood Onset Systemic Lupus Erythematosus among Early and Peri-Adolescent Age Groups.

    Nelson, Meghan Corrigan / Chandrakasan, Shanmuganathan / Ponder, Lori / Sanz, Ignacio / Goldberg, Baruch / Ogbu, Ekemini A / Rouster-Stevens, Kelly / Prahalad, Sampath

    Children (Basel, Switzerland)

    2022  Volume 9, Issue 12

    Abstract: Introduction: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that is associated with significant morbidity and mortality. SLE disproportionately affects women and minorities. Childhood-onset SLE (cSLE) in particular tends to be ... ...

    Abstract Introduction: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that is associated with significant morbidity and mortality. SLE disproportionately affects women and minorities. Childhood-onset SLE (cSLE) in particular tends to be more aggressive than adult-onset SLE. Despite substantial improvements in the treatment of cSLE, there is significant variability in treatment responses and long-term outcomes. Furthermore, there is a paucity of studies involving cSLE, and in particular, cSLE among different age groups. The aim of this study was to test the hypothesis that an early-onset cSLE cohort would demonstrate unique characteristics with distinctive clinical and laboratory features at disease onset. We specifically investigated whether clinical, epidemiological, or serological factors are differentially associated with early- and late-onset cSLE. This could have direct impact on clinical management with the goal of improving outcomes and quality of life for children with SLE. Methods: Our study was conducted at a large tertiary center. We included 213 subjects seen at our pediatric rheumatology clinic aged 4−17 years. Epidemiologic, clinical phenotype, disease severity, serology, treatment, and outcome data were compared between subjects with cSLE onset prior to 10 years of age (early-onset disease, n = 43) and those with cSLE onset greater than 10 years of age (peri-adolescent disease, n = 170). We compared clinical features between early- and peri-adolescent onset cSLE in order to investigate the association between age at disease onset of cSLE and clinical disease expression and outcomes. Results: Of the 213 subjects with cSLE in our study, 43 subjects had early-onset disease (age 2 to ≤9 years) and 170 patients had peri-adolescent onset disease. We found that early-onset cSLE was associated with a higher prevalence of positive anti-dsDNA antibody at cSLE diagnosis, higher anti-dsDNA antibody titer at cSLE diagnosis, rash, and azathioprine use (p < 0.001, p = 0.004, p = 0.011, and p = 0.008, respectively). In contrast, we found that peri-adolescent onset cSLE (≥10 years of age) was associated with worse disease activity (SLEDAI range 0−24) (p < 0.001), higher SLICC at diagnosis (p < 0.001), as well as a higher rate of mycophenolate mofetil and hydroxychloroquine use (p = 0.003 and p < 0.001, respectively). There were no significant differences in the prevalence of neuropsychiatric symptoms or the development of Class IV/Class V lupus nephritis between the early-onset and peri-adolescent groups.
    Language English
    Publishing date 2022-11-30
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2732685-8
    ISSN 2227-9067
    ISSN 2227-9067
    DOI 10.3390/children9121865
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Lung involvement in juvenile idiopathic inflammatory myopathy: A systematic review.

    Abu-Rumeileh, Sarah / Marrani, Edoardo / Maniscalco, Valerio / Maccora, Ilaria / Pagnini, Ilaria / Mastrolia, Maria Vincenza / Rouster-Stevens, Kelly / Simonini, Gabriele

    Autoimmunity reviews

    2023  Volume 22, Issue 10, Page(s) 103416

    Abstract: Objective: Juvenile idiopathic inflammatory myopathies (JIIM) are a group of connective tissue disorders characterized by muscle inflammation and variable systemic involvement, including interstitial lung disease (ILD). Available data on JIIM-associated ...

    Abstract Objective: Juvenile idiopathic inflammatory myopathies (JIIM) are a group of connective tissue disorders characterized by muscle inflammation and variable systemic involvement, including interstitial lung disease (ILD). Available data on JIIM-associated ILD are very limited. We performed a systematic review of the available clinical, laboratory, and radiological features of JIIM-associated ILD.
    Methods: A systematic literature review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement.
    Results: A total of 90 patients were identified, of whom 77.8% had JDM, 10% amyopathic JDM, 7.8% anti-synthetase syndrome, 3.3% overlap syndrome, and 1.1% juvenile polymyositis. Anti-melanoma differentiation-associated gene 5 (MDA-5/CADM-140) was the most frequently reported myositis-specific antibody (32.2%). At diagnosis of ILD, 55.5% of patients had respiratory symptoms. Ground glass opacity was the most reported radiological feature (52.9%). Thirty-three % of patients developed rapidly progressive (RP) lung disease; 26.7% were admitted to the intensive care unit (ICU); 28.9% died; all deaths were due to ILD, with a median interval of 2 months (IQR 1.5-4.7) between the onset of respiratory symptoms and death. Patients admitted to the ICU and who died of ILD were more likely to be male, to have a rapidly progressive pattern, progression of radiological features, and a higher level of KL-6.
    Conclusions: MDA-5/CADM-14 is associated with RP-ILD. ILD is a rare but severe manifestation among the spectrum of systemic involvement associated with JIIM, with a high rate of ICU admission and mortality. Early recognition and aggressive treatment are needed to prevent a severe outcome.
    MeSH term(s) Humans ; Male ; Female ; Dermatomyositis/diagnosis ; Myositis/complications ; Polymyositis ; Lung Diseases, Interstitial/complications ; Lung Diseases, Interstitial/diagnosis ; Lung ; Autoantibodies ; Retrospective Studies
    Chemical Substances Autoantibodies
    Language English
    Publishing date 2023-08-22
    Publishing country Netherlands
    Document type Systematic Review ; Journal Article ; Review
    ZDB-ID 2144145-5
    ISSN 1873-0183 ; 1568-9972
    ISSN (online) 1873-0183
    ISSN 1568-9972
    DOI 10.1016/j.autrev.2023.103416
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Prevalence of tissue transglutaminase antibodies and IgA deficiency are not increased in juvenile idiopathic arthritis: a case-control study.

    Kohli, Angela Taneja / Hersh, Aimee O / Ponder, Lori / Chan, Lai Hin Kimi / Rouster-Stevens, Kelly A / Tebo, Anne E / Kugathasan, Subra / Guthery, Stephen L / Bohnsack, John F / Prahalad, Sampath

    Pediatric rheumatology online journal

    2023  Volume 21, Issue 1, Page(s) 110

    Abstract: Background: The prevalence of Celiac Disease (CD) in Juvenile Idiopathic Arthritis (JIA) has been reported to be 0.1-7% in various small studies. As a result of the limited number of research and their inconclusive results there are no clear ... ...

    Abstract Background: The prevalence of Celiac Disease (CD) in Juvenile Idiopathic Arthritis (JIA) has been reported to be 0.1-7% in various small studies. As a result of the limited number of research and their inconclusive results there are no clear recommendations for routine CD screening in asymptomatic patients with JIA. Our aim is to estimate the prevalence of IgA deficiency and tissue transglutaminase (tTG) IgA in a cohort of JIA followed in two large academic medical centers.
    Methods: Serum was collected and stored from all subjects and analyzed in a reference laboratory for total IgA (Quantitative Nephelometry) and tTG IgA antibody levels (Semi-Quantitative Enzyme-Linked Immunosorbent Assay). Fisher's exact tests were performed for statistical significance. Risk estimates (odds ratios) with 95% confidence intervals were calculated.
    Results: 808 JIA cases and 140 controls were analyzed. Majority were non-Hispanic whites (72% vs. 68% p = 0.309). A total of 1.2% of cases were IgA deficient compared to none of the controls (p = 0.373). After excluding IgA deficient subjects, 2% of cases had tTG IgA ≥ 4u/mL compared to 3.6% of controls (p = 0.216) (OR = 0.5; 95% C.I = 0.1-1.4); and 0.8% of cases had tTG IgA > 10u/mL compared to 1.4% of controls (p = 0.627) (OR = 0.5; 95%C.I = 0.1-2.9).
    Conclusions: Using the largest JIA cohort to date to investigate prevalence of celiac antibodies, the prevalence of positive tTG IgA was 0.8% and of IgA deficiency was 1.2%. The results did not demonstrate a higher prevalence of abnormal tTG IgA in JIA. The study did not support the routine screening of asymptomatic JIA patients for CD.
    MeSH term(s) Humans ; Protein Glutamine gamma Glutamyltransferase 2 ; Arthritis, Juvenile/epidemiology ; Case-Control Studies ; Transglutaminases ; Prevalence ; IgA Deficiency/diagnosis ; IgA Deficiency/epidemiology ; Immunoglobulin A ; Autoantibodies ; Celiac Disease/diagnosis ; Celiac Disease/epidemiology
    Chemical Substances Protein Glutamine gamma Glutamyltransferase 2 (EC 2.3.2.13) ; Transglutaminases (EC 2.3.2.13) ; Immunoglobulin A ; Autoantibodies
    Language English
    Publishing date 2023-10-05
    Publishing country England
    Document type Journal Article
    ZDB-ID 2279468-2
    ISSN 1546-0096 ; 1546-0096
    ISSN (online) 1546-0096
    ISSN 1546-0096
    DOI 10.1186/s12969-023-00890-z
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Simultaneous Presentation of Crohn's Disease and Takayasu Arteritis in a Teenage Patient.

    Polyakova, Inna / Iannucci, Glen / George, Roshan / Gill, Anne / Patel, Dinesh Govind / Rouster-Stevens, Kelly

    Journal of investigative medicine high impact case reports

    2020  Volume 8, Page(s) 2324709620977317

    Abstract: A 14-year-old female with no significant medical history presented with hypertensive urgency, in the setting of 4 to 6 weeks of diarrhea, abdominal pain, headaches, anemia, weight loss, and high blood pressures. Her evaluation revealed signs of a ... ...

    Abstract A 14-year-old female with no significant medical history presented with hypertensive urgency, in the setting of 4 to 6 weeks of diarrhea, abdominal pain, headaches, anemia, weight loss, and high blood pressures. Her evaluation revealed signs of a systemic inflammatory process that was most suspicious for inflammatory bowel disease. However, when her hypertension was evaluated with a renal Doppler ultrasound, there were signs of narrowing, stenosis, and hypoplasia that led to a diagnostic angiogram of the abdominal aorta. Full body positron emission tomography scan revealed multiple areas of stenosis and aortic thickening with enhancement compatible with Takayasu arteritis. She received prednisone, methotrexate, and infliximab with marked improvement in her clinical symptoms and inflammatory markers.
    MeSH term(s) Adolescent ; Angiography ; Crohn Disease/complications ; Crohn Disease/diagnostic imaging ; Crohn Disease/drug therapy ; Female ; Humans ; Infliximab/therapeutic use ; Methotrexate/therapeutic use ; Positron Emission Tomography Computed Tomography ; Prednisone/therapeutic use ; Takayasu Arteritis/complications ; Takayasu Arteritis/diagnostic imaging ; Takayasu Arteritis/drug therapy ; Ultrasonography, Doppler
    Chemical Substances Infliximab (B72HH48FLU) ; Prednisone (VB0R961HZT) ; Methotrexate (YL5FZ2Y5U1)
    Language English
    Publishing date 2020-11-25
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 2710326-2
    ISSN 2324-7096 ; 2324-7096
    ISSN (online) 2324-7096
    ISSN 2324-7096
    DOI 10.1177/2324709620977317
    Database MEDical Literature Analysis and Retrieval System OnLINE

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