Article
Urology & Nephrology
Els Van de Perre, Rachel B. Jones, David R. W. Jayne
Summary: The disease course of adult IgA vasculitis is distinct from that in children, with a higher frequency of persistent and relapsing disease. Renal disease plays a significant role in ongoing disease activity, but more than 50% of patients with chronic disease activity also exhibit extra-renal features.
CLINICAL KIDNEY JOURNAL
(2021)
Review
Biochemistry & Molecular Biology
Bryan Nguyen, Chirag Acharya, Supawit Tangpanithandee, Jing Miao, Pajaree Krisanapan, Charat Thongprayoon, Omar Amir, Michael A. Mao, Wisit Cheungpasitporn, Prakrati C. Acharya
Summary: This systematic review suggests that plasmapheresis/plasma exchange (PLEX) may have benefits in some patients with IgA nephropathy (IgAN) and Henoch-Schonlein purpura nephritis (HSP) who present with rapidly progressive glomerulonephritis (RPGN). However, more clinical studies are needed to confirm these findings.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Pediatrics
Zhijuan Kang, Wentao Wu, Mai Xun, Yunfeng Ding, Zhihui Li
Summary: Henoch-Schonlein purpura (HSP)/IgA vasculitis (IgAV) is the most common form of systemic vasculitis in children, mainly affecting the skin, gastrointestinal tract, joints, and kidneys, with rare involvement of the heart. This article presents a case of a 6-year-old male child with HSP/IgAV who had renal and cardiac involvement, including a rare coronary artery aneurysm. Reviewing other cases from the literature, a total of 24 cases of HSP/IgAV complicated with cardiac involvement have been reported, with majority of patients receiving glucocorticoid and/or immunosuppressant treatment and showing poor prognosis.
FRONTIERS IN PEDIATRICS
(2022)
Article
Medicine, General & Internal
Sung Kyun Yim, Seung Young Seo
Summary: This case report describes an elderly patient with IgA vasculitis (IgAV) initially mimicking drug-induced skin reaction. Despite initially being diagnosed with infectious colitis, the patient was eventually confirmed to have IgAV. Despite its rarity in elderly patients, IgAV should be highly suspected in patients presenting with rashes, proteinuria, and signs of colitis.
Article
Engineering, Biomedical
Souhila Ait-Tigrine, Lucas Liaudet, Sofia Bogiatzi, Patrick Yerly, Roger Hullin, Matthias Kirsch
Summary: The implantation of LVADs in patients with end-stage heart failure may lead to immune dysregulation and systemic inflammatory response. This study found that IgA vasculitis, clinically manifested as Henoch-Schonlein purpura, can occur after LVAD implantation and is associated with a significant increase in CXCL13 levels. The vasculitis spontaneously resolved over time as CXCL13 expression decreased.
Review
Pediatrics
Valentina M. L. Montorfani-Janett, Gabriele E. Montorfani, Camilla Lavagno, Gianluca Gualco, Mario G. Bianchetti, Gregorio P. Milani, Sebastiano A. G. Lava, Marirosa Cristallo Lacalamita
Summary: The involvement of external male genitalia in Henoch-Schonlein vasculitis is not well categorized. This systematic review analyzed 85 reports and found that the genital involvement mostly appeared concurrently with or after the cutaneous features of Henoch-Schonlein syndrome. Scrotal involvement can result from skin inflammation, epididymitis, orchitis, or testicular ischemia.
Article
Dermatology
Silvia Angelica Carmona-Cruz, Lourdes Carola Duran-McKinster, Maria Teresa Garcia-Romero
Summary: The prognosis of IgA vasculitis is determined by renal or other organ involvement. A retrospective study found that location of purpura above the waist and an altered urinalysis at diagnosis predicted a more aggressive course of the disease.
PEDIATRIC DERMATOLOGY
(2022)
Article
Pediatrics
Dan Wu, Lei Lei, Hejia Zhang, Xingfeng Yao, Zhi Chen, Nan Zhang, Jie Ni, Chen Ling, Xiaorong Liu, Xiangmei Chen
Summary: This study found that glomerular C4d deposition is associated with mesangial hypercellularity and glomerular IgM deposition in children with IgA nephropathy (IgAN) and Henoch-Schonlein purpura nephropathy (HSPN). It may be a risk factor for decline in estimated glomerular filtration rate (eGFR) in children with IgAN.
PEDIATRIC NEPHROLOGY
(2023)
Review
Urology & Nephrology
Xinyao Luo, Jiaxing Tan, Dingyuan Wan, Junda Chen, Yahong Hu
Summary: The Oxford classification, especially for E, S, T, and C lesions, may serve as predictors for adverse renal outcomes in patients with HSPN, regardless of age group.
INTERNATIONAL UROLOGY AND NEPHROLOGY
(2022)
Article
Immunology
Maria Maddalena Sirufo, Martina Raggiunti, Lina Maria Magnanimi, Lia Ginaldi, Massimo De Martinis
Summary: This case report describes a female who developed HSP after receiving the COVID-19 vaccine ChAdOx1 nCoV-19 AZD1222, which is the first report of its kind. No similar cases were reported in the ChAdOx1 nCoV-19 AZD1222 trial with 23,848 participants.
Article
Urology & Nephrology
Fengmei Wang, Huizi Zhu, Shougang Bao, Hengtao Qi, Liang Xu, Xiang Liu, Chunjuan Zhai, Xiaowei Yang, Rong Wang
Summary: This study aimed to investigate the prevalence of left renal vein (LRV) entrapment in different types of renal diseases and its association with glomerular incidental IgA and galactose-deficient-IgA1 (Gd-IgA1) deposition. The study found that the prevalence of LRV entrapment was significantly higher in patients with idiopathic IgAN and HSPN compared to patients without LRV entrapment. Patients with LRV entrapment also showed a higher occurrence of glomerular IgA and Gd-IgA1 deposition compared to patients without LRV entrapment.
Article
Pediatrics
Ling Hou, Zhou Zhang, Yue Du
Summary: This study successfully treated 5 pediatric patients with IgA vasculitis and nephritis using LEF combined with a corticosteroid, resulting in complete remission of proteinuria in four patients and significantly reduced proteinuria in one patient. No severe adverse events were reported during the 6 to 12 months treatment period.
Article
Rheumatology
Kinanah Yaseen, Leal C. Herlitz, Alexandra Villa-Forte
Summary: IgA vasculitis in adults presents differently from pediatric cases, with ongoing controversies in treatment approaches. Research suggests various immunosuppressive therapies may be beneficial for severe IgAV, but there is a lack of high-quality evidence or guidelines for the treatment of severe cases, emphasizing the need for further controlled trials.
CURRENT RHEUMATOLOGY REPORTS
(2021)
Article
Pediatrics
Marina Avramescu, Annie Lahoche, Julien Hogan, Remi Salomon, Gwenaelle Roussey, Justine Bacchetta, Stephane Decramer, Tim Ulinski, Coralie Barbe, Christine Pietrement
Summary: The prognosis of Henoch-Schonlein purpura (HSP), IgA vasculitis, depends on kidney involvement. There is no consensus on the initiation of treatment for HSP nephritis (HSPN). Early kidney biopsy at diagnosis seems to be associated with a better renal outcome at 5 years compared to treatment without a biopsy.
PEDIATRIC NEPHROLOGY
(2022)
Article
Pediatrics
Hillarey K. Stone, Mark Mitsnefes, Kimberley Dickinson, Evanette K. Burrows, Hanieh Razzaghi, Ingrid Y. Luna, Caroline A. Gluck, Bradley P. Dixon, Vikas R. Dharnidharka, William E. Smoyer, Michael J. Somers, Joseph T. Flynn, Susan L. Furth, Charles Bailey, Christopher B. Forrest, Michelle Denburg, Edward Nehus
Summary: This study aims to investigate the clinical management and kidney outcomes of a large cohort of children with IgAVN. The results show that conservative management is the predominant practice pattern, while immunosuppressive medications are used in patients with more severe presentations. Kidney outcomes are favorable in this group of children with IgAVN.
PEDIATRIC NEPHROLOGY
(2023)
Letter
Rheumatology
Ovgu Kul Cinar, Matko Marlais, Muthana Al Obaidi, Iek Leng Cheng, Kjell Tullus, Paul Brogan, Elena Moraitis
Article
Rheumatology
Charalampia Papadopoulou, Muthana Al Obaidi, Elena Moraitis, Sandrine Compeyrot-Lacassagne, Despina Eleftheriou, Paul Brogan
Summary: This study described the clinical presentation, treatment, and outcome of paediatric inflammatory multisystem syndrome temporally related to Sars-CoV-2 (PIMS-TS), proposed a framework for multidisciplinary team management, and highlighted the role of the pediatric rheumatologist. The study found significant diagnostic challenges with the current definition of PIMS-TS, emphasizing the importance of an MDT approach and the role of pediatric rheumatologists in managing hyperinflammation in young patients.
Article
Rheumatology
Paul Brogan, Rae S. M. Yeung, Gavin Cleary, Satyapal Rangaraj, Ozgur Kasapcopur, Aimee O. Hersh, Suzanne Li, Dusan Paripovic, Kenneth Schikler, Andrew Zeft, Claudia Bracaglia, Despina Eleftheriou, Pooneh Pordeli, Simone Melega, Candice Jamois, Jacques Gaudreault, Margaret Michalska, Paul Brunetta, Jennifer C. Cooper, Patricia B. Lehane
Summary: In this study, 25 pediatric patients with new-onset or relapsing GPA or MPA completed the remission-induction phase with RTX treatment. Most patients experienced mild to moderate infusion-related adverse events, and the study results showed that RTX is well tolerated and effective in pediatric patients with GPA or MPA.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Rheumatology
Marta Bustaffa, Isabelle Kone-Paut, Seza Ozen, Gayane Amaryan, Efimia Papadopoulou-Alataki, Romina Gallizzi, Maria Carrabba, Yonatan Butbul Aviel, Luca Cantarini, Maria Alessio, Jordi Anton, Laura Obici, Faysal Gok, Ezgi Deniz Batu, Estefania Moreno, Paul Brogan, Maria Trachana, Gabriele Simonini, Donato Rigante, Yosef Uziel, Antonella Insalaco, Maria Cristina Maggio, Nicolino Ruperto, Marco Gattorno, L. Rossi Semerano
Summary: The new EPCC and INSAID classification of MEFV variants can identify two distinct groups of FMF patients, which differ in clinical characteristics, therapeutic approach, and response to treatment.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2022)
Article
Rheumatology
Kader Cetin Gedik, Lovro Lamot, Micol Romano, Erkan Demirkaya, David Piskin, Sofia Torreggiani, Laura A. Adang, Thais Armangue, Kathe Barchus, Devon R. Cordova, Yanick J. Crow, Russell C. Dale, Karen L. Durrant, Despina Eleftheriou, Elisa M. Fazzi, Marco Gattorno, Francesco Gavazzi, Eric P. Hanson, Min Ae Lee-Kirsch, Gina A. Montealegre Sanchez, Benedicte Neven, Simona Orcesi, Seza Ozen, M. Cecilia Poli, Elliot Schumacher, Davide Tonduti, Katsiaryna Uss, Daniel Aletaha, Brian M. Feldman, Adeline Vanderver, Paul A. Brogan, Raphaela Goldbach-Mansky
Summary: This study aims to improve the diagnosis, treatment, and long-term monitoring of patients with autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI, and AGS. The research team developed guidelines and considerations to enhance care quality and disease outcomes.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Immunology
Ying Hong, Marina Casimir, Benjamin C. Houghton, Fang Zhang, Barbara Jensen, Ebun Omoyinmi, Robert Torrance, Charalampia Papadopoulou, Michelle Cummins, Marion Roderick, Adrian J. Thrasher, Paul A. Brogan, Despina Eleftheriou
Summary: Lentivirus-mediated ADA2 gene correction can ameliorate the immune cell damage and vasculitis in DADA2 patients, and restore the function of hematopoietic stem cells in patients with pure red cell aplasia.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Pediatrics
Ovgu Kul Cinar, Amber Putland, Karen Wynne, Despina Eleftheriou, Paul A. Brogan
Summary: Hereditary systemic autoinflammatory diseases (SAIDs) are rare and severe conditions characterized by mutations in key regulators of the immune system. Through translational research, we have gained a better understanding of these diseases and developed targeted treatments.
FRONTIERS IN PEDIATRICS
(2022)
Article
Rheumatology
Simone Melega, Paul Brogan, Gavin Cleary, Aimee O. Hersh, Ozgur Kasapcopur, Satyapal Rangaraj, Rae S. M. Yeung, Andrew Zeft, Jennifer Cooper, Pooneh Pordeli, Petra Kirchner, Patricia B. Lehane
Summary: This study assessed the impact of prolonged low immunoglobulin levels on the risk of serious infection in pediatric patients receiving rituximab treatment. The results showed that the majority of patients did not experience serious infections, and the infections that did occur were manageable.
RHEUMATOLOGY AND THERAPY
(2022)
Meeting Abstract
Rheumatology
F. De Benedetti, A. Grom, P. Brogan, C. Bracaglia, M. Pardeo, G. Marucci, D. Eleftheriou, C. Papadopoulou, P. Quartier, J. Anton, R. Frederiksen, V. Asnaghi, C. de Min
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Rheumatology
Paul Brogan, Ray Naden, Stacy P. Ardoin, Jennifer C. Cooper, Fabrizio De Benedetti, Jean-Francois Dicaire, Despina Eleftheriou, Brian Feldman, Jon Goldin, Seth E. Karol, Fiona Price-Kuehne, David Skuse, Constantine A. Stratakis, Nicholas Webb, John H. Stone
Summary: The study developed a Pediatric Glucocorticoid Toxicity Index (pGTI) as a weighted clinical assessment tool to measure changes in GC toxicity over time. It includes toxicity assessment and a Damage Checklist to comprehensively measure GC toxicity, providing an accurate and easy-to-use tool for clinical and research purposes.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2022)
Article
Medicine, General & Internal
Pui Y. Lee, Brad A. Davidson, Roshini S. Abraham, Blanche Alter, Juan I. Arostegui, Katherine Bell, Alexandre Belot, Jenna R. E. Bergerson, Timothy J. Bernard, Paul A. Brogan, Yackov Berkun, Natalie T. Deuitch, Dimana Dimitrova, Sophie A. Georgin-Lavialle, Marco Gattorno, Bodo Grimbacher, Hasan Hashem, Michael S. Hershfield, Rebecca N. Ichord, Kazushi Izawa, Jennifer A. Kanakry, Raju P. Khubchandani, Femke C. C. Klouwer, Evan A. Luton, Ada W. Man, Isabelle Meyts, Joris M. Van Montfrans, Seza Ozen, Janna Saarela, Gustavo C. Santo, Aman Sharma, Ariane Soldatos, Rachel Sparks, Troy R. Torgerson, Ignacio Leandro Uriarte, Taryn A. B. Youngstein, Qing Zhou, Ivona Aksentijevich, Daniel L. Kastner, Eugene P. Chambers, Amanda K. Ombrello
Summary: Deficiency of adenosine deaminase 2 (DADA2) is a recessively inherited disease characterized by systemic vasculitis, early-onset stroke, bone marrow failure, and/or immunodeficiency affecting both children and adults. This study provides consensus statements for the evaluation and management of DADA2, a potentially fatal disease that requires early diagnosis and treatment.
Article
Immunology
Samantha Cooray, Fiona Price-Kuehne, Ying Hong, Ebun Omoyinmi, Alice Burleigh, Kimberly C. Gilmour, Bilal Ahmad, Sangdun Choi, Mohammad W. Bahar, Paul Torpiano, Andrey Gagunashvili, Barbara Jensen, Evangelos Bellos, Vanessa Sancho-Shimizu, Jethro A. Herberg, Kshitij Mankad, Atul Kumar, Marios Kaliakatsos, Austen J. J. Worth, Despina Eleftheriou, Elizabeth Whittaker, Paul A. Brogan
Summary: We describe a novel, severe autoinflammatory syndrome characterized by neuroinflammation, systemic autoinflammation, splenomegaly, and anemia (NASA) caused by bi-allelic mutations in IRAK4. This study describes 5 affected patients from 2 unrelated families with compound heterozygous mutations in IRAK4 resulting in severe systemic autoinflammation, massive splenomegaly and severe transfusion dependent anemia and, in 3/5 cases, severe neuroinflammation and seizures. Immunological analysis demonstrated elevated levels of pro-inflammatory cytokines in affected patients.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Pediatrics
C. M. Foley, D. Mckenna, K. Gallagher, K. Mclellan, H. Alkhdher, S. Lacassagne, E. Moraitis, C. Papadopoulou, C. Pilkington, M. Al Obaidi, D. Eleftheriou, P. Brogan
Summary: Systemic juvenile idiopathic arthritis (sJIA) is a complex inflammatory disorder involving innate and adaptive immunity. Research shows that early use of IL-1 or IL-6 blockade as first-line treatment is effective. This study reviewed the medical records of sJIA patients over a 16-year period and examined clinical presentation, treatment interventions, and remission rates. The results indicate that patients who received early biologic treatment achieved higher rates of clinical remission.
FRONTIERS IN PEDIATRICS
(2023)
Article
Rheumatology
Fabrizio De Benedetti, Alexei A. Grom, Paul A. Brogan, Claudia Bracaglia, Manuela Pardeo, Giulia Marucci, Despina Eleftheriou, Charalampia Papadopoulou, Grant S. Schulert, Pierre Quartier, Jordi Anton, Christian Laveille, Rikke Frederiksen, Veronica Asnaghi, Maria Ballabio, Philippe Jacqmin, Cristina de Min
Summary: The objective of this study was to confirm the adequacy of an emapalumab dosing regimen in relation to interferon-γ (IFN-γ) activity and assess its efficacy and safety in treating MAS secondary to sJIA or AOSD. The results showed that emapalumab was efficacious in inducing remission of MAS and viral infections were observed.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Meeting Abstract
Rheumatology
Adriana Almeida de Jesus, Guibin Chen, Dan Yang, Tomas Brdicka, Natasha Ruth, David Bennin, Dita Cebecauerova, Hana Malcova, Helen Freeman, Neil Martin, Karel Svojgr, Murray Passo, Farzana Bhuyan, Sara Alehashemi, Andre Rastegar, Kat Uss, Lela Kardova, Iris Duric, Ebun Omoyinmi, Petra Peldova, Chyi-Chia Richard Lee, David Kleiner, Colleen Hadigan, Stephen Hewitt, Stefania Pittaluga, Carmelo Carmona-Rivera, Katherine R. Calvo, Nirali Shah, Miroslava Balascakova, Danielle Fink, Radana Kotalova, Zuzana Parackova, Lucie Peterkova, Daniela Kuzilkova, Vit Campr, Lucie Sramkova, Stephen Brooks, Eric Meffre, Rebecca Harper, Hyesun Kuehn, Mariana Kaplan, Paul Brogan, Sergio Rosenzweig, Zuoming Deng, Anna Huttenlocher, Susan Moir, Douglas Kuhns, Manfred Boehm, Karolina Skvarova Kramarzova, Raphaela Goldbach-Mansky
ARTHRITIS & RHEUMATOLOGY
(2022)