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Article: Autoimmune Congenital Complete Heart Block: How Late Can It Occur?

Makadia, Luv / Izmirly, Peter / Buyon, Jill P / Phoon, Colin K L

2023 Volume 13, Issue 2, Page(s) e29–e34

Abstract: ... ...

Abstract	Objective
Language	English
Publishing date	2023-05-08
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2628074-7
ISSN	2157-7005 ; 2157-6998
ISSN (online)	2157-7005
ISSN	2157-6998
DOI	10.1055/s-0043-1768708
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Article ; Online: To Be or Not to Be Treated: That Is the Question in Managing a Fetus With Cardiac Injury Exposed to Anti-SSA/Ro.

Buyon, Jill / Saxena, Amit / Friedman, Deborah / Izmirly, Peter

Journal of the American Heart Association

2022 Volume 11, Issue 13, Page(s) e025580

MeSH term(s)	Antibodies, Antinuclear ; Autoantibodies ; Fetus ; Humans ; Lupus Erythematosus, Systemic
Chemical Substances	Antibodies, Antinuclear ; Autoantibodies
Language	English
Publishing date	2022-06-22
Publishing country	England
Document type	Letter ; Research Support, N.I.H., Extramural ; Comment
ZDB-ID	2653953-6
ISSN	2047-9980 ; 2047-9980
ISSN (online)	2047-9980
ISSN	2047-9980
DOI	10.1161/JAHA.122.025580
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Article ; Online: Reply.

Buyon, Jill / Izmirly, Peter / Masson, Mala / Carlucci, Philip / Izmirly, Caroline G / Clancy, Robert / Cuneo, Bettina

Arthritis & rheumatology (Hoboken, N.J.)

2024

Language	English
Publishing date	2024-01-17
Publishing country	United States
Document type	Letter
ZDB-ID	2756371-6
ISSN	2326-5205 ; 2326-5191
ISSN (online)	2326-5205
ISSN	2326-5191
DOI	10.1002/art.42806
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Article: Clinical implications of discordance between anti-dsDNA antibodies by multiplex flow immunoassay and

Zaminski, Devyn / Saxena, Amit / Izmirly, Peter / Buyon, Jill P / Belmont, H Michael

Lupus science & medicine

2023 Volume 10, Issue 2

Abstract: Objective: Anti-dsDNA antibodies (anti-dsDNA) are a component of all classification schemes in SLE and comprise one of the domains in validated activity indices. Anti-dsDNA is frequently measured commercially by an enzyme immunoassay (EIA) or : ... ...

Abstract	Objective: Anti-dsDNA antibodies (anti-dsDNA) are a component of all classification schemes in SLE and comprise one of the domains in validated activity indices. Anti-dsDNA is frequently measured commercially by an enzyme immunoassay (EIA) or Methods: All patients fulfilled the classification criteria for SLE by at least one of the validated schemes: American College of Rheumatology, Systemic Lupus Erythematosus International Collaborating Clinics and/or American College of Rheumatology/European League Against Rheumatism classification criteria. Patients with one or more simultaneously paired anti-dsDNA by multiplex EIA and CLIFT were identified. Analysis of concordance or discordance, titre comparability of assays and association with hybrid SLE Disease Activity Index score, prevalence of lupus nephritis (LN), ability to predict a flare and classification criteria was performed. Results: 207 patients were simultaneously tested by EIA and CLIFT at least once for anti-dsDNA, generating 586 paired results. 377 pairs were concordant and 209 were discordant. 41 of 207 patients always had discordant paired results and 39 patients always had results with titre discordance. In 100 patients with LN, 60 were positive by EIA and 72 by CLIFT. Sensitivities and specificities for patients with LN versus patients without LN were EIA 60% and 47%, and CLIFT 72% and 37%, respectively. 42 patients had flare assessment within 90 days of their paired result. Six of seven patients with mild flares and all four patients with severe flares had concordant positive results. Conclusion: Our data demonstrate that discordance of positivity between both assays for anti-dsDNA is relatively common, occurring in a fifth of patients overall and a third of visits. EIA positivity is associated with LN less often than CLIFT positivity. With the significant discordance of results between anti-dsDNA assays, obtaining both CLIFT and EIA assays may be beneficial for classification and routine monitoring of SLE.
MeSH term(s)	Humans ; Fluorescent Antibody Technique ; Crithidia ; Lupus Erythematosus, Systemic/diagnosis ; Immunoassay/methods ; Lupus Nephritis/diagnosis ; DNA
Chemical Substances	anti-dsDNA autoantibody ; DNA (9007-49-2)
Language	English
Publishing date	2023-11-14
Publishing country	England
Document type	Journal Article
ZDB-ID	2779620-6
ISSN	2053-8790
ISSN	2053-8790
DOI	10.1136/lupus-2023-001012
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Article: Autoimmune Congenital Complete Heart Block: How Late Can It Occur?

Makadia, Luv / Izmirly, Peter / Buyon, Jill P. / Phoon, Colin K. L.

American Journal of Perinatology Reports

2023 Volume 13, Issue 02, Page(s) e29–e34

Abstract: Objective: Maternal anti-Ro (SSA) and/or anti-La (SSB) antibodies are a risk factor for congenital complete heart block (CHB). Because detailed analysis of the incidence of CHB after 24 weeks of gestational age (GA) is lacking, we aimed to ascertain the ...

Abstract	Objective: Maternal anti-Ro (SSA) and/or anti-La (SSB) antibodies are a risk factor for congenital complete heart block (CHB). Because detailed analysis of the incidence of CHB after 24 weeks of gestational age (GA) is lacking, we aimed to ascertain the risk of “later-onset” CHB among offspring of SSA/SSB-positive mothers in the published literature. Study Design: Using search terms “neonatal lupus heart block” and “autoimmune congenital heart block” on PubMed and Ovid, we gathered prospective studies of SSA/SSB-positive mothers with fetal echo surveillance starting from before CHB diagnosis and retrospective cases of fetal CHB diagnosis after 24 weeks of GA (if there was prior normal heart rate) or after birth. Results: Ten prospective studies included 1,248 SSA/SSB-positive pregnancies with 24 cases of CHB diagnosed during pregnancy (1.9%). Among these, three (12.5%) were after 24 weeks—at weeks 25, 26, and 28. Our retrospective studies revealed 50 patients with CHB diagnosis in late fetal life and neonatal period and 34 in the nonneonatal childhood period. An additional four cases were diagnosed after age 18 years. Conclusion: Later-onset autoimmune CHB in offspring of SSA/SSB-positive mothers does occur. Our analysis suggests that prenatal surveillance should continue beyond 24 weeks of GA but is limited by inconsistent published surveillance data.
Keywords	neonatal lupus ; fetal echocardiography ; congenital heart block ; complete heart block
Language	English
Publishing date	2023-04-01
Publisher	Thieme Medical Publishers, Inc.
Publishing place	Stuttgart ; New York
Document type	Article
ZDB-ID	2628074-7
ISSN	2157-7005 ; 2157-6998
ISSN (online)	2157-7005
ISSN	2157-6998
DOI	10.1055/s-0043-1768708
Database	Thieme publisher's database

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Article: Thoughts on COVID-19 and autoimmune diseases.

Askanase, Anca D / Khalili, Leila / Buyon, Jill P

Lupus science & medicine

2020 Volume 7, Issue 1, Page(s) e000396

Abstract: Over the 2 months since coronavirus first appeared in China, cases have emerged on every continent, and it is clear that patients with autoimmune diseases might also be affected. Coronavirus disease 2019 (COVID-19) is a highly contagious viral illness ... ...

Abstract	Over the 2 months since coronavirus first appeared in China, cases have emerged on every continent, and it is clear that patients with autoimmune diseases might also be affected. Coronavirus disease 2019 (COVID-19) is a highly contagious viral illness with a mortality rate approaching 2%. Here we discuss the challenges that patients with autoimmune diseases might face and the information on using immunomodulatory therapies like chloroquine, tocilizumab and baricitinib to quench the cytokine storm in patients with very severe COVID-19 pneumonia.
MeSH term(s)	Antibodies, Monoclonal, Humanized/therapeutic use ; Autoimmune Diseases/complications ; Autoimmune Diseases/therapy ; Azetidines/therapeutic use ; Betacoronavirus ; COVID-19 ; Chloroquine/therapeutic use ; Coronavirus Infections/complications ; Coronavirus Infections/drug therapy ; Cytokine Release Syndrome ; Humans ; Hydroxychloroquine/therapeutic use ; Immunomodulation ; Immunosuppression ; Pandemics ; Pneumonia, Viral/complications ; Pneumonia, Viral/drug therapy ; Rheumatology ; SARS-CoV-2 ; Sulfonamides/therapeutic use
Chemical Substances	Antibodies, Monoclonal, Humanized ; Azetidines ; Sulfonamides ; Hydroxychloroquine (4QWG6N8QKH) ; Chloroquine (886U3H6UFF) ; tocilizumab (I031V2H011) ; baricitinib (ISP4442I3Y)
Keywords	covid19
Language	English
Publishing date	2020-04-03
Publishing country	England
Document type	Journal Article
ZDB-ID	2779620-6
ISSN	2053-8790
ISSN	2053-8790
DOI	10.1136/lupus-2020-000396
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Article ; Online: Gerald Weissmann: Inflammation in rheumatic disease.

Cronstein, Bruce N / Buyon, Jill P / Abramson, Steven B

Annals of the rheumatic diseases

2020 Volume 79, Issue 4, Page(s) 435–436

MeSH term(s)	History, 20th Century ; History, 21st Century ; Inflammation/immunology ; Liposomes ; Neutrophils/immunology ; Rheumatic Diseases/immunology ; Rheumatology/history
Chemical Substances	Liposomes
Language	English
Publishing date	2020-01-22
Publishing country	England
Document type	Biography ; Historical Article ; Journal Article
ZDB-ID	7090-7
ISSN	1468-2060 ; 0003-4967
ISSN (online)	1468-2060
ISSN	0003-4967
DOI	10.1136/annrheumdis-2019-216770
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Article ; Online: Risk Assessment Model for Postpartum Venous Thromboembolism Prevention in Patients with Systemic Lupus Erythematosus.

Griffin, Myah M / Engel, Alexis / Mehta-Lee, Shilpi S / Nusbaum, Julie / Golpanian, Michael / Izmirly, Peter / Belmont, H Michael / Buyon, Jill P

American journal of perinatology

2023 Volume 40, Issue 16, Page(s) 1732–1737

Abstract: Objective: This article assesses the application of the Royal College of Obstetricians and Gynaecologists (RCOG) venous thromboembolism (VTE) risk model on a cohort of postpartum patients with a history of systemic lupus erythematosus (SLE).: Study ... ...

Abstract	Objective: This article assesses the application of the Royal College of Obstetricians and Gynaecologists (RCOG) venous thromboembolism (VTE) risk model on a cohort of postpartum patients with a history of systemic lupus erythematosus (SLE). Study design: This is a secondary analysis of an ongoing patient registry of women with SLE from 2016 to 2022. There were 49 SLE patients with 55 pregnancies using the Definitions of Remission in SLE (DORIS) criteria to determine SLE disease activity. RCOG risk assessment model scoring was calculated for each patient prior to and after delivery. The primary outcome was the qualification of "active SLE" by standard rheumatologic criteria and assessment of recommendations for VTE prophylaxis based on RCOG VTE risk assessment scoring. Data were analyzed using Fisher's exact test, chi-square test, and Mann-Whitney Results: In the study cohort, 34 pregnancies (61.8%) were in DORIS remission at delivery. Twenty-one pregnancies (38.2%) were not and scored 3 points on the RCOG VTE risk model. Of these pregnancies, only 19% ( Conclusion: These data reveal a need to improve upon providing postpartum VTE prophylaxis to SLE patients not in remission while also recognizing a diagnosis of SLE alone should not equate with active disease. Moreover, SLE patients in remission may still warrant VTE prophylaxis if other non-SLE-related risk factors are present. Key points: · Those with SLE are at increased risk for VTE postpartum.. · VTE prophylaxis should be instituted when clinically appropriate.. · Caution should be exercised in broadly assigning disease activity for SLE diagnosis only.. · This study supports VTE prophylaxis use in postpartum patients with SLE..
MeSH term(s)	Pregnancy ; Humans ; Female ; Venous Thromboembolism/etiology ; Venous Thromboembolism/prevention & control ; Anticoagulants/therapeutic use ; Risk Assessment ; Venous Thrombosis ; Postpartum Period ; Risk Factors ; Puerperal Disorders/etiology ; Puerperal Disorders/prevention & control ; Pregnancy Complications, Cardiovascular/prevention & control ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/drug therapy
Chemical Substances	Anticoagulants
Language	English
Publishing date	2023-07-20
Publishing country	United States
Document type	Journal Article
ZDB-ID	605671-4
ISSN	1098-8785 ; 0735-1631
ISSN (online)	1098-8785
ISSN	0735-1631
DOI	10.1055/s-0043-1771049
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Article: Erythrocyte complement receptor 1 (ECR1) and erythrocyte-bound C4d (EC4d) in the prediction of poor pregnancy outcomes in systemic lupus erythematosus (SLE).

Conklin, John / Golpanian, Michael / Engel, Alexis / Izmirly, Peter / Belmont, H Michael / Dervieux, Thierry / Buyon, Jill P / Alexander, Roberta Vezza

Lupus science & medicine

2023 Volume 9, Issue 1

Abstract: Background: Complement activation has been associated with adverse pregnancy outcomes (APO) in SLE. Pregnant women with SLE were studied to evaluate whether complement dysregulation within the first two pregnancy trimesters predicts APO.: Methods: ... ...

Abstract	Background: Complement activation has been associated with adverse pregnancy outcomes (APO) in SLE. Pregnant women with SLE were studied to evaluate whether complement dysregulation within the first two pregnancy trimesters predicts APO. Methods: Pregnant women fulfilled classification criteria for SLE. APO included neonatal death, preterm delivery before 36 weeks and small for gestational age newborn. Pre-eclampsia was also evaluated. Erythrocyte complement receptor 1 (ECR1) and erythrocyte-bound C4d (EC4d) were measured by flow cytometry. Complement proteins C3 and C4 were measured by immunoturbidimetry and anti-double-stranded DNA by ELISA in serum. Statistical analysis consisted of t-test, confusion matrix-derived diagnostic analysis, and multivariate logistic regression. Results: Fifty-one women had 57 pregnancies and 169 visits during the study. Baseline visits occurred mainly in the first (n=32) and second trimester (n=21). Fourteen (24.6%) pregnancies resulted in 21 APO with preterm delivery being the most common (n=10). ECR1 <5.5 net mean fluorescence intensity in the first trimester predicted APO with a diagnostic OR (DOR) of 18.33 (95% CI: 2.39 to 140.4; t-test p=0.04). Other individual biomarkers did not reach statistical significance. To estimate the likelihood of APO, we developed an algorithm that included the week of pregnancy, ECR1 and EC4d. From this algorithm, a Pregnancy Adversity Index (PAI) was calculated, and a PAI >0 indicated an elevated likelihood of pregnancy complications (DOR: 20.0 (95% CI: 3.64 to 109.97)). Conclusions: Low levels of ECR1 in early or mid-pregnancy are predictive of an APO. Incorporating the weeks of gestation and both ECR1 and EC4d generated a PAI, which further predicted serious pregnancy complications.
MeSH term(s)	Infant, Newborn ; Humans ; Female ; Pregnancy ; Pregnancy Outcome ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/diagnosis ; Premature Birth ; Erythrocytes ; Pregnancy Complications ; Complement C3 ; Receptors, Complement
Chemical Substances	Complement C3 ; Receptors, Complement
Language	English
Publishing date	2023-02-09
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2779620-6
ISSN	2053-8790
ISSN	2053-8790
DOI	10.1136/lupus-2022-000754
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Article ; Online: Contribution of S100A4-expressing fibroblasts to anti-SSA/Ro-associated atrioventricular nodal calcification and soluble S100A4 as a biomarker of clinical severity.

Firl, Christina E M / Halushka, Marc / Fraser, Nicola / Masson, Mala / Cuneo, Bettina F / Saxena, Amit / Clancy, Robert / Buyon, Jill

Frontiers in immunology

2023 Volume 14, Page(s) 1114808

Abstract: Background: Fibrosis and dystrophic calcification disrupting conduction tissue architecture are histopathological lesions characterizing cardiac manifestations of neonatal lupus (cardiac-NL) associated with maternal anti-SSA/Ro antibodies.: Objectives! ...

Abstract	Background: Fibrosis and dystrophic calcification disrupting conduction tissue architecture are histopathological lesions characterizing cardiac manifestations of neonatal lupus (cardiac-NL) associated with maternal anti-SSA/Ro antibodies. Objectives: Increased appreciation of heterogeneity in fibroblasts encourages re-examination of existing models with the consideration of multiple fibroblast subtypes (and their unique functional differences) in mind. This study addressed fibroblast heterogeneity by examining expression of α-Smooth Muscle Actin (myofibroblasts) and of S100 Calcium-Binding Protein A4 (S100A4). Methods: Using a previously established model of rheumatic scarring/fibrosis Results: S100A4 immunostaining was observed in 4 cardiac-NL hearts with positional identity in the conduction system at regions of dystrophic calcification but not fibrotic zones, the latter containing only myofibroblasts. Conclusions: These findings position the S100A4
MeSH term(s)	Infant, Newborn ; Humans ; Heart Block/etiology ; Heart Block/pathology ; Heart ; Biomarkers ; Calcinosis ; Fibrosis ; Fibroblasts/metabolism ; S100 Calcium-Binding Protein A4/metabolism
Chemical Substances	Biomarkers ; S100A4 protein, human (142662-27-9) ; S100 Calcium-Binding Protein A4
Language	English
Publishing date	2023-04-06
Publishing country	Switzerland
Document type	Journal Article ; Research Support, N.I.H., Extramural
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2023.1114808
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