Article
Pediatrics
Tingyan He, Yu Xia, Ying Luo, Jun Yang
Summary: This study aimed to evaluate the efficacy and potential adverse effects of JAKi in patients with systemic juvenile idiopathic arthritis (SJIA). The results showed that JAKi may be an alternative or adjuvant agent for patients with persistently active disease, glucocorticoid-related adverse reactions, or SJIA-MAS.
FRONTIERS IN PEDIATRICS
(2023)
Article
Rheumatology
Rosemary G. Peterson, Rui Xiao, Karen E. James, Hannah Katcoff, Brian T. Fisher, Pamela F. Weiss
Summary: The study found significant variation in the use of biologics and glucocorticoids in children hospitalized with new-onset systemic JIA, with noticeable differences between hospitals. Despite increasing evidence supporting the use of biologics for improved outcomes, many children did not receive biologic treatment and a high rate of glucocorticoid exposure persisted.
ARTHRITIS CARE & RESEARCH
(2021)
Review
Rheumatology
Claas H. Hinze, Dirk Foell, Christoph Kessel
Summary: Systemic juvenile idiopathic arthritis (sJIA) is a disease characterized by severe systemic inflammation and arthritis. It poses challenges to rheumatologists treating pediatric and adult patients worldwide. Although treatment plans exist for classic sJIA, there is still a lack of clear treatment approaches for early sJIA without arthritis and complicated sJIA.
NATURE REVIEWS RHEUMATOLOGY
(2023)
Review
Medicine, General & Internal
Charlotte Girard-Guyonvarc'h, Mathilde Harel, Cem Gabay
Summary: This review summarizes the role and importance of IL-18 and IL-18BP in AOSD and sJIA. It is found that the imbalance of IL-18/IL-18BP is associated with the occurrence of these diseases, and treatment with recombinant IL-18BP shows potential therapeutic effects.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Immunology
Shuya Kaneko, Masaki Shimizu, Futaba Miyaoka, Asami Shimbo, Hitoshi Irabu, Mao Mizuta, Yasuo Nakagishi, Naomi Iwata, Junya Fujimura, Masaaki Mori, Tomohiro Morio
Summary: This study validates the correlation between laboratory markers reflecting disease activity of macrophage activation syndrome (MAS) and serum cytokine levels, and identifies valuable laboratory markers that change over time for a prompt MAS diagnosis.
CLINICAL IMMUNOLOGY
(2023)
Article
Pediatrics
Ellen Go, Mira van Veenendaal, Cedric Manlhiot, Rayfel Schneider, Brian W. McCrindle, Rae S. M. Yeung
Summary: This study describes the disease course of a small proportion of patients with both Kawasaki disease and systemic juvenile idiopathic arthritis, characterized by refractory Kawasaki disease, high prevalence of coronary artery dilatation, and shared immunopathology potentially linking the two conditions.
FRONTIERS IN PEDIATRICS
(2021)
Article
Rheumatology
R. Naveen, Avinash Jain, Hafis Muhammed, Latika Gupta, Durga P. Misra, Able Lawrence, Vikas Agarwal, Ramnath Misra, Amita Aggarwal
Summary: This study compared the clinical and laboratory parameters of MAS in SLE and sJIA, finding that the two diseases are more similar than dissimilar in clinical features and outcome. The criteria meant for MAS in sJIA or SLE-MAS performed equally well in diagnosing both diseases.
RHEUMATOLOGY INTERNATIONAL
(2021)
Review
Medicine, General & Internal
William G. Ambler, Kabita Nanda, Karen Brandt Onel, Susan Shenoi
Summary: This review focuses on the treatment options and potential future therapeutics for systemic juvenile idiopathic arthritis (SJIA). Recent advancements in targeted therapies have improved patient outcomes, but there are still subsets of patients with refractory disease and severe complications.
ANNALS OF MEDICINE
(2022)
Article
Rheumatology
Stephane Mitrovic, Nolan Hassold, Aly Kamissoko, Nicolas Rosine, Alexis Mathian, Guillaume Mercy, Edouard Pertuiset, Gaetane Nocturne, Bruno Fautrel, Isabelle Kone-Paut
Summary: A retrospective national survey revealed features of SpA in patients with SJIA/AOSD, with a prevalence of 6.58% in AOSD patients and 10% in SJIA patients. This suggests a possible overlap between SpA and AOSD that requires further study.
Review
Rheumatology
Eduardo Liquidano-Perez, Gibert Maza-Ramos, Jose Luis Salazar-Bailon, Marco Antonio Yamazaki-Nakashimada, Francisco Rivas-Larrauri
Summary: This study presents a refractory case of soJIA complicated with MAS successfully treated with plasma exchange, resulting in significant improvement.
RHEUMATOLOGY INTERNATIONAL
(2022)
Article
Rheumatology
Masaki Shimizu, Kenichi Nishimura, Naomi Iwata, Takahiro Yasumi, Hiroaki Umebayashi, Yasuo Nakagishi, Yuka Okura, Nami Okamoto, Noriko Kinjo, Mao Mizuta, Masato Yashiro, Junko Yasumura, Hiroyuki Wakiguchi, Tomohiro Kubota, Mariko Mouri, Utako Kaneko, Masaaki Mori
Summary: A multicenter, retrospective study in Japan revealed that the first-line treatment for MAS in pediatric rheumatology institutes is methylprednisolone pulse therapy and/or cyclosporine A. Dexamethasone palmitate (DEX-P) can be considered as an effective and safe therapeutic option for patients with corticosteroid-resistant MAS.
INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES
(2023)
Article
Pediatrics
Mikhail M. Kostik, Eugenia A. Isupova, Konstantin Belozerov, Tatyana S. Likhacheva, Evgeny N. Suspitsin, Rinat Raupov, Vera V. Masalova, Irina A. Chikova, Margarita F. Dubko, Olga V. Kalashnikova, Vyacheslav G. Chasnyk, Randy Q. Cron
Summary: This study provides evidence for the efficacy and safety of short-term increased doses (2-3 times normal) of canakinumab in treating MAS associated with sJIA. Further research on the efficacy and safety of increased doses of canakinumab for the treatment of MAS in children with sJIA is warranted.
FRONTIERS IN PEDIATRICS
(2022)
Review
Rheumatology
Emely L. Verweyen, Grant S. Schulert
Summary: Systemic JIA (SJIA) is characterized by severe complications, including macrophage activation syndrome (SJIA-MAS) and lung disease (SJIA-LD), which are driven by interferons (IFNs) and involve the JAK-STAT signaling pathway. New therapeutic advances, such as JAK inhibitors and antibodies targeting IFNs, are urgently needed to address the divergent disease pathogenesis observed in SJIA.
Review
Biochemistry & Molecular Biology
Chao-Yi Wu, Huang-Yu Yang, Jing-Long Huang, Jenn-Haung Lai
Summary: Monocytes and macrophages play a crucial role in the pathogenesis of juvenile idiopathic arthritis (JIA), with their activation being linked to inflammation regulation and tissue repair mechanisms in the disease process.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Rheumatology
Piero Ruscitti, Valentina Natoli, Alessandro Consolaro, Roberta Caorsi, Silvia Rosina, Gabriella Giancane, Roberta Naddei, Ilenia Di Cola, Claudia Di Muzio, Onorina Berardicurti, Daniela Iacono, Ilenia Pantano, Gelsomina Rozza, Silvia Rossi, Ludovico De Stefano, Silvia Balduzzi, Antonio Vitale, Francesco Caso, Luisa Costa, Marcella Prete, Luca Navarini, Annamaria Iagnocco, Fabiola Atzeni, Giuliana Guggino, Federico Perosa, Luca Cantarini, Bruno Frediani, Carlomaurizio Montecucco, Francesco Ciccia, Paola Cipriani, Marco Gattorno, Roberto Giacomelli, Angelo Ravelli
Summary: This study compares the clinical features and treatments of systemic JIA and adult-onset Still's disease. The results reveal remarkable disparities in the prevalence of clinical manifestations between the two illnesses, which may partly depend on their classification by different criteria.
Article
Dermatology
Rahul Mahajan, Seema Manjunath, Manoj Gopal Madakshira, Debajyoti Chatterjee, Anuradha Bishnoi, Dipankar De, Sanjeev Handa, Bishan Dass Radotra, Manu Jamwal, Reena Das
Summary: In diagnosing EB, CDM showed the highest concordance with NGS, followed by TEM and IFM. The study suggests that CDM can be a feasible diagnostic tool.
JOURNAL OF CUTANEOUS PATHOLOGY
(2022)
Letter
Oncology
Sreejesh Sreedharanunni, Manu Jamwal, Anand Balakrishnan, Arun Vijayalakshmi Aravindan, Ritika Sharma, Namrata Singh, Sweta Rajpal, Shelly Singla, Alka Rani Khadwal, Jasmina Ahluwalia, Pankaj Malhotra, Reena Das
Article
Dermatology
Mohan H. Kumar, Prashant Sharma, Shivaprakash M. Rudramurthy, Inderpaul Singh Sehgal, Kuruswamy Thurai Prasad, Ashok Kumar Pannu, Reena Das, Naresh K. Panda, Navneet Sharma, Arunaloke Chakrabarti, Ritesh Agarwal, Valliappan Muthu
Summary: This study compared the serum iron indices in COVID-19 patients with and without COVID-19-associated mucormycosis (CAM), finding that CAM cases had lower TIBC levels than COVID-19 controls, suggesting a potential association with dysregulated iron metabolism in the pathogenesis of CAM.
Article
Hematology
Madhu Chopra, Arihant Jain, Sanjeev Chhabra, Shaweta Kaundal, Charanpreet Singh, Aditya Jandial, Gaurav Prakash, Alka Khadwal, Chandan Das, Mini P. Singh, Reena Das, Pankaj Malhotra, Deepesh P. Lad
Summary: This study reports the antibody response to the COVISHIELD (TM) vaccine in patients with hematological malignancies. The results confirm the safety and efficacy of the COVISHIELD (TM) vaccine in this population.
INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION
(2022)
Letter
Hematology
Niranjan Shiwaji Khaire, Manu Jamwal, Prashant Sharma, Jasbir Kaur Hira, Sanjeev Chhabra, Pankaj Malhotra, Reena Das
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
(2022)
Letter
Hematology
Ritika Sharma, Manu Jamwal, Hari Kishan Senee, Narender Kumar, Deepak Bansal, Amita Trehan, Jasmina Ahluwalia, Reena Das
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
(2022)
Article
Gastroenterology & Hepatology
Ujjal Poddar, Amita Aggarwal, Krishnegowda Jayalakshmi, Moinak Sen Sarma, Anshu Srivastava, Amit Rawat, Surender Kumar Yachha
Summary: This study analyzed 48 cases of very early-onset inflammatory bowel disease (VEO-IBD) among 200 children, and found that 32% of them had monogenic VEO-IBD. The clinical features that differentiated monogenic from nonmonogenic VEO-IBD included neonatal onset, perianal disease, consanguinity and sibling death, wasting, stunting, and unclassified phenotype of IBD.
INFLAMMATORY BOWEL DISEASES
(2023)
Article
Hematology
Nisha Duggal, Namrata Singh, Suchet Sachdev, Avinash Kumar Singh, Jasbir Kaur Hira, Sanjeev Chhabra, Deepak Bansal, Pankaj Malhotra, Neelam Varma, Reena Das, Prashant Sharma
Summary: Genetic testing for inherited erythrocytosis often involves laborious exon-by-exon gene panel testing or expensive next-generation sequencing. A study in north Indian erythrocytosis patients found a high prevalence (61%) of the Chuvash polycythemia mutation. This study assessed the use of PCR-RFLP for VHL c.598C > T mutation as a first-line test in 99 individuals with JAK2 V617F-negative, unexplained erythrocytosis. The results suggest that PCR-RFLP for VHL c.598C > T mutation may be an effective initial genetic screening method for unexplained congenital erythrocytosis in regions where Chuvash polycythemia is common.
INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION
(2023)
Letter
Hematology
Prashant Sharma, Prateek Bhatia, Reena Das, Sanjeev Chhabra, Jasbir Kaur Hira
INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION
(2023)
Letter
Pathology
Manu Jamwal, Namrata Singh, Prashant Sharma, Ajay Duseja, Deepesh P. Lad, Pankaj Malhotra, Reena Das
Article
Transplantation
Joyita Bharati, Jhumki Das, Pandiarajan Vignesh, Kenar D. Jhaveri, Arun Prabhahar, Chandan Krushna Das, Anita Singh Parihar, Ritambhra Nada, Raja Ramachandran, Amit Rawat, Harbir Singh Kohli
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2023)
Review
Medicine, General & Internal
Pallavi L. Nadig, Vibhu Joshi, Rakesh Kumar Pilania, Rajni Kumrah, Jayakanthan Kabeerdoss, Saniya Sharma, Deepti Suri, Amit Rawat, Surjit Singh
Summary: Kawasaki disease (KD) is a childhood vasculitis that primarily affects medium vessels, particularly the coronary arteries. Coronary artery aneurysm occurs in about 25-30% of untreated cases, leading to significant morbidity. Intravenous immunoglobulin (IVIg) has been consistently shown to reduce the risk of coronary artery aneurysms to less than 5%, but the mechanism of immunomodulation is still unclear. Studies suggest that IVIg may modulate the innate immune system by affecting toll-like receptor pathways, autophagy, apoptosis, neutrophil extracellular trap, dendritic cell modulation, T-cell differentiation, cytokine release, and regulatory T-cell function. This review discusses the potential mechanisms underlying the immunomodulatory actions of IVIg in KD patients and summarizes the evidence regarding infusion protocols and dosages used in treatment.
Review
Medicine, General & Internal
Saniya Sharma, Pallavi L. Nadig, Rakesh Kumar Pilania, Kaushal Sharma, Manpreet Dhaliwal, Amit Rawat, Surjit Singh
Summary: The exact cause of Kawasaki disease (KD), a common childhood vasculitis, is still unknown, but it is believed to involve an abnormal immune response triggered by an infectious or environmental factor in genetically predisposed children. Patients with inborn errors of immunity (IEI) are more susceptible to infections that can lead to immune dysregulation due to imbalances in the immune system. KD can develop as a complication in both primary and secondary immunodeficiencies. The association between KD and IEI is currently based on specific case reports and case series, and more research is needed to fully understand the underlying mechanisms.
Review
Pediatrics
Aaqib Zaffar Banday, Alisha Babbar, Pratap Kumar Patra, Ankur Kumar Jindal, Deepti Suri, Vignesh Pandiarajan, Amit Rawat, Anju Gupta, Surjit Singh
Summary: In this study, the occurrence of Kawasaki disease (KD) in relation to neoplasms was investigated. The results suggest a possible association between neoplasms and the triggering of KD, as well as an increased risk of coronary artery abnormalities (CAAs) in neoplasm-related KD.
JOURNAL OF TROPICAL PEDIATRICS
(2023)
Letter
Pathology
Tushar Sehgal, Neelam Varma, Reena Das, Subhash Varma
INDIAN JOURNAL OF PATHOLOGY AND MICROBIOLOGY
(2022)