Review
Medicine, General & Internal
Franck Zekre, Anita Duncan, Audrey Laurent, Maud Tusseau, Remi Pescarmona, Sophie Collardeau-Frachon, Camille Ohlmann, Sebastien Viel, Philippe Reix, Sarah Benezech, Alexandre Belot
Summary: Biological disease-modifying anti-rheumatic drugs (bDMARDs) targeting IL-6 and IL-1 beta are commonly used as a first-line therapy for systemic-onset juvenile idiopathic arthritis (sJIA), but they may increase the risk of pulmonary alveolar proteinosis (PAP). We report a new case of PAP-MAS successfully treated with Janus Kinase inhibitors (JAKi) and review previous clinical descriptions of this condition. Among the analyzed articles, the incidence of PAP-MAS among sJIA patients ranged from 1.28% to 12.9%. JAKi shows promise in treating lung disease associated with sJIA.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Immunology
Luna Dillemans, Youri Bekhuis, Albrecht Betrains, Karen Yu, Maarten van Hemelen, Noemie Portner, Lien De Somer, Patrick Matthys, Jeroen Breckpot, Thomas Tousseyn, Marijke Peetermans, Paul Proost, Carine Wouters, Steven Vanderschueren
Summary: We report a fatal case of a 20-year-old woman with refractory adult-onset Still's disease accompanied by fulminant macrophage activation syndrome and atypical hemolytic uremic syndrome. This case highlights the importance of prompt genetic assessment of complement-encoding alleles, in addition to HLH-related genes, in patients with severe adult-onset Still's disease with recurrent macrophage activation syndrome and features of thrombotic microangiopathy.
CLINICAL IMMUNOLOGY
(2023)
Review
Immunology
Mariame Mohamed Ahamada, Yang Jia, Xiaochuan Wu
Summary: Systemic lupus erythematosus (SLE) is an autoimmune disease that primarily affects adults, especially females and non-Caucasian individuals. Despite numerous studies on SLE, the exact pathogenesis remains unclear and there is no specific drug for effective treatment. The involvement of macrophages in the development of SLE is increasingly recognized, suggesting a potential for macrophage-centered therapeutic approaches.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Rheumatology
Guangbo Chen, Gail H. Deutsch, Grant S. Schulert, Hong Zheng, SoRi Jang, Bruce Trapnell, Pui Y. Lee, Claudia Macaubas, Katherine Ho, Corinne Schneider, Vivian E. Saper, Adriana Almeida de Jesus, Mark A. Krasnow, Alexei Grom, Raphaela Goldbach-Mansky, Purvesh Khatri, Elizabeth D. Mellins, Scott W. Canna
Summary: This study investigated the immunopathology and identified biomarkers in systemic JIA, MAS, and SJIA-LD by measuring over 1,300 analytes in sera. The proteome alterations in active systemic JIA and MAS overlapped, with novel elevations in heat-shock proteins and glycolytic enzymes.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Multidisciplinary Sciences
Jennifer L. Johnson, Elsa Meneses-Salas, Mahalakshmi Ramadass, Jlenia Monfregola, Farhana Rahman, Raquel Carvalho Gontijo, William B. Kiosses, Kersi Pestonjamasp, Dale Allen, Jinzhong Zhang, Douglas G. Osborne, Yanfang Peipei Zhu, Nathan Wineinger, Kasra Askari, Danni Chen, Juan Yu, Scott C. Henderson, Catherine C. Hedrick, Matilde Valeria Ursini, Sergio Grinstein, Daniel D. Billadeau, Sergio D. Catz
Summary: This study investigates the process of exocytosis in neutrophil leukocytes and shows that the actin nucleator protein WASH plays a critical role in this process. The authors find that WASH facilitates the release of gelatinase granules while inhibiting the release of pro-inflammatory granules. They also discover that a deficiency in WASH leads to dysregulated secretion and inflammation in neutrophil leukocytes.
NATURE COMMUNICATIONS
(2022)
Article
Rheumatology
Lu-Wei Huang, James Cheng-Chung Wei, Der-Yuan Chen, Yen-Ju Chen, Kuo-Tung Tang, Tai-Ming Ko, Hsin-Hua Chen
Summary: This study aimed to determine the bidirectional relationship between macrophage activation syndrome (MAS) and systemic lupus erythematosus (SLE). The results showed that there was no significant association between a history of MAS and the risk of SLE, but the risk of MAS was markedly increased in patients with SLE.
Article
Biochemistry & Molecular Biology
Karin Palmblad, Hanna Schierbeck, Erik Sundberg, Anna-Carin Horne, Helena Erlandsson Harris, Jan-Inge Henter, Ulf Andersson
Summary: This study examined the systemic release pattern of HMGB1 in children with critical MAS and found that HMGB1 may not only be a biomarker of inflammation, but also contribute to the pathogenesis of MAS. The use of etoposide in therapy could potentially ameliorate inflammation by promoting apoptotic cell death instead of proinflammatory lytic cell death.
MOLECULAR MEDICINE
(2021)
Article
Rheumatology
Sonali Dey, Rashmi Roongta, Sumantro Mondal, Subhankar Haldar, Geetabali Sircar, Biswadip Ghosh, Alakendu Ghosh
Summary: This article presents a case of recurrent MAS in pediatric SLE and emphasizes the importance of early detection and prompt treatment for reducing morbidity in these patients.
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.
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
Roberto Caricchio, Marcello Gallucci, Chandra Dass, Xinyan Zhang, Stefania Gallucci, David Fleece, Michael Bromberg, Gerard J. Criner
Summary: Researchers developed predictive criteria for COVID-19-associated cytokine storm (CS) and proposed new early predictive criteria to identify CS occurring in patients with COVID-19, which can determine the need for early therapeutic regimen and potentially reduce mortality.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Article
Pediatrics
Christopher Towe, Alexei A. Grom, Grant S. Schulert
Summary: Chronic lung disease in children with systemic juvenile idiopathic arthritis (SJIA-LD) is a potentially life-threatening complication. The optimal approaches to diagnosis and management of SJIA-LD are still unclear. This review discusses the current clinical understanding of SJIA-LD, including its potential pathogenesis and treatment options, and highlights the urgent unmet needs in this area.
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)
Review
Immunology
Adam Al-Hakim, Anoop Mistry, Sinisa Savic
Summary: This review discusses new developments in acquired/late onset SAID, including various diseases such as phenocopies, Schnitzler's syndrome, Adult onset Still's disease, VEXAS syndrome, and autoinflammatory complications associated with myelo-dysplastic syndrome.
JOURNAL OF INFLAMMATION RESEARCH
(2022)
Article
Medicine, Research & Experimental
Jian Qiu, Chang Shu, Xin Li, Wei-Chang Zhang
Summary: PAQR3 silencing accelerates diabetic wound healing by promoting M2 macrophage polarization and angiogenesis, which is mediated by the inhibition of STUB1-mediated PPAR gamma protein ubiquitination and degradation.
LABORATORY INVESTIGATION
(2022)
Review
Rheumatology
Shima Yasin, Grant S. Schulert
CURRENT OPINION IN RHEUMATOLOGY
(2018)
Review
Pediatrics
Onengiya Harry, Shima Yasin, Hermine Brunner
JOURNAL OF PEDIATRICS
(2018)
Article
Dermatology
Richard H. Schwartz, Shima K. Yasin, Jinny K. Yoo
PEDIATRIC DERMATOLOGY
(2014)
Letter
Rheumatology
Shima Yasin, Krista Solomon, Scott W. Canna, Charlotte Girard-Guyonvarc'h, Cem Gabay, Eduardo Schiffrin, Andrew Sleight, Alexei A. Grom, Grant S. Schulert
Article
Rheumatology
Shima Yasin, Ndate Fall, Rachel A. Brown, Maggie Henderlight, Scott W. Canna, Charlotte Girard-Guyonvarc'h, Cem Gabay, Alexei A. Grom, Grant S. Schulert
Article
Urology & Nephrology
Qassim Abid, Alejandro Best Rocha, Christopher P. Larsen, Grant Schulert, Rebecca Marsh, Shima Yasin, Cathy Patty-Resk, Rudolph P. Valentini, Matthew Adams, Rossana Baracco
AMERICAN JOURNAL OF KIDNEY DISEASES
(2020)
Article
Rheumatology
Grant S. Schulert, Shima Yasin, Brenna Carey, Claudia Chalk, Thuy Do, Andrew H. Schapiro, Ammar Husami, Allen Watts, Hermine I. Brunner, Jennifer Huggins, Elizabeth D. Mellins, Esi M. Morgan, Tracy Ting, Bruce C. Trapnell, Kathryn A. Wikenheiser-Brokamp, Christopher Towe, Alexei A. Grom
ARTHRITIS & RHEUMATOLOGY
(2019)
Review
Rheumatology
Shima Yasin, T. Shawn Sato, Polly Ferguson
Summary: Advances in understanding the pathogenesis of chronic recurrent multifocal osteomyelitis (CRMO) in children have influenced therapeutic strategies. The use of whole-body MRI (WBMRI) and increased awareness of CRMO have led to higher rates and timeliness of CRMO diagnoses. This review highlights recent findings from CRMO cohorts and discusses the course, complications, and long-term sequelae of the disease. It is important for clinicians to be aware of the potential for long-term sequelae in order to optimize therapy and prevent complications.
CURRENT OPINION IN RHEUMATOLOGY
(2022)