ABOUT THE RESEARCHER

OVERVIEW

Dr. Pui Lee is a pediatric rheumatologist with research interests in autoinflammatory diseases and myeloid cell biology. His lab studies the role of immunometabolism in the development and differentiation of monocytes, a type of white blood cells that incites inflammation, with the goal of identifying treatment targets for inflammatory diseases. In addition, Dr. Lee is interested in the mechanism of immune activation in autoinflammatory diseases. He has ongoing projects focused on macrophage activation syndrome and a rare disease called deficiency of adenosine deaminase 2 (DADA2).

BACKGROUND

Dr. Pui Lee is an Attending Physician in Rheumatology at Boston Children’s Hospital and an Assistant Professor in Pediatrics at Harvard Medical School. He completed the MD-PhD program at the University of Florida and subsequently finished residency and fellowship training at Boston Children's Hospital.

PUBLICATIONS

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  1. Socioeconomic and Racial/Ethnic Disparities in Multisystem Inflammatory Syndrome. Pediatrics. 2021 Feb 18. View abstract
  2. Deficiency of Adenosine Deaminase 2 in Adults and Children: Experience From India. Arthritis Rheumatol. 2021 02; 73(2):276-285. View abstract
  3. Reply. Arthritis Rheumatol. 2021 01; 73(1):176-178. View abstract
  4. Distinct clinical and immunological features of SARS-CoV-2-induced multisystem inflammatory syndrome in children. J Clin Invest. 2020 11 02; 130(11):5942-5950. View abstract
  5. Monogenic autoinflammatory disorders: Conceptual overview, phenotype, and clinical approach. J Allergy Clin Immunol. 2020 11; 146(5):925-937. View abstract
  6. Atrioventricular Block in Children With Multisystem Inflammatory Syndrome. Pediatrics. 2020 11; 146(5). View abstract
  7. Immune dysregulation and multisystem inflammatory syndrome in children (MIS-C) in individuals with haploinsufficiency of SOCS1. J Allergy Clin Immunol. 2020 11; 146(5):1194-1200.e1. View abstract
  8. NF-E2-Related Factor 2 Regulates Interferon Receptor Expression and Alters Macrophage Polarization in Lupus. Arthritis Rheumatol. 2020 10; 72(10):1707-1720. View abstract
  9. Interstitial Lung Disease and Psoriasis in a Child With Aicardi-Goutières Syndrome. Front Immunol. 2020; 11:985. View abstract
  10. On the Alert for Cytokine Storm: Immunopathology in COVID-19. Arthritis Rheumatol. 2020 07; 72(7):1059-1063. View abstract
  11. Polyarteritis nodosa and deficiency of adenosine deaminase 2 - Shared genealogy, generations apart. Clin Immunol. 2020 06; 215:108411. View abstract
  12. Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases. J Clin Invest. 2020 04 01; 130(4):1669-1682. View abstract
  13. Th17 reprogramming of T cells in systemic juvenile idiopathic arthritis. JCI Insight. 2020 03 26; 5(6). View abstract
  14. Analysis of peripheral blood ADA1 and ADA2 levels in children and adults. Response to: 'Total adenosine deaminase highly correlated with adenosine deaminase 2 activity in serum' by Gao et al. Ann Rheum Dis. 2020 Feb 13. View abstract
  15. Genotype and functional correlates of disease phenotype in deficiency of adenosine deaminase 2 (DADA2). J Allergy Clin Immunol. 2020 06; 145(6):1664-1672.e10. View abstract
  16. A dominant autoinflammatory disease caused by non-cleavable variants of RIPK1. Nature. 2020 01; 577(7788):109-114. View abstract
  17. Differential Responsiveness of Monocyte and Macrophage Subsets to Interferon. Arthritis Rheumatol. 2020 01; 72(1):100-113. View abstract
  18. Giant Cell Tumor of Tendon Sheath and Tendinopathy as Early Features of Early Onset Sarcoidosis. Front Pediatr. 2019; 7:480. View abstract
  19. Adenosine deaminase 2 as a biomarker of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Ann Rheum Dis. 2020 02; 79(2):225-231. View abstract
  20. High-dimensional analysis reveals a pathogenic role of inflammatory monocytes in experimental diffuse alveolar hemorrhage. JCI Insight. 2019 08 08; 4(15). View abstract
  21. NETing the mechanism of inflammation in DADA2. Blood. 2019 07 25; 134(4):338-339. View abstract
  22. Megakaryocyte emperipolesis mediates membrane transfer from intracytoplasmic neutrophils to platelets. Elife. 2019 05 01; 8. View abstract
  23. Tofacitinib Treatment of Refractory Systemic Juvenile Idiopathic Arthritis. Pediatrics. 2019 05; 143(5). View abstract
  24. Differential attenuation of ß2 integrin-dependent and -independent neutrophil migration by Ly6G ligation. Blood Adv. 2019 02 12; 3(3):256-267. View abstract
  25. Vasculopathy, Immunodeficiency, and Bone Marrow Failure: The Intriguing Syndrome Caused by Deficiency of Adenosine Deaminase 2. Front Pediatr. 2018; 6:282. View abstract
  26. Disrupted N-linked glycosylation as a disease mechanism in deficiency of ADA2. J Allergy Clin Immunol. 2018 10; 142(4):1363-1365.e8. View abstract
  27. Stromal cell cadherin-11 regulates adipose tissue inflammation and diabetes. J Clin Invest. 2017 Sep 01; 127(9):3300-3312. View abstract
  28. The metabolic regulator mTORC1 controls terminal myeloid differentiation. Sci Immunol. 2017 May 26; 2(11). View abstract
  29. Pathogenesis of Diffuse Alveolar Hemorrhage in Murine Lupus. Arthritis Rheumatol. 2017 06; 69(6):1280-1293. View abstract
  30. The presentation and management of granulomatosis with polyangiitis (Wegener's Granulomatosis) in the pediatric airway. Laryngoscope. 2017 01; 127(1):233-240. View abstract
  31. Renal involvement in the immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) disorder. Pediatr Nephrol. 2015 Jul; 30(7):1197-202. View abstract
  32. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome associated with neonatal epidermolysis bullosa acquisita. Pediatr Dermatol. 2015 May-Jun; 32(3):e74-7. View abstract
  33. Hypercalcemia and miliary sarcoidosis in a 15-year-old boy. Arthritis Rheum. 2013 Aug; 65(8):2112. View abstract
  34. Ly6 family proteins in neutrophil biology. J Leukoc Biol. 2013 Oct; 94(4):585-94. View abstract
  35. Maintenance of anti-Sm/RNP autoantibody production by plasma cells residing in ectopic lymphoid tissue and bone marrow memory B cells. J Immunol. 2013 Apr 15; 190(8):3916-27. View abstract
  36. Pleiotropic IFN-dependent and -independent effects of IRF5 on the pathogenesis of experimental lupus. J Immunol. 2012 Apr 15; 188(8):4113-21. View abstract
  37. Pathogenic role of B cells in the development of diffuse alveolar hemorrhage induced by pristane. Lab Invest. 2011 Oct; 91(10):1540-50. View abstract
  38. B cells enhance early innate immune responses during bacterial sepsis. J Exp Med. 2011 Aug 01; 208(8):1673-82. View abstract
  39. IL-1a modulates neutrophil recruitment in chronic inflammation induced by hydrocarbon oil. J Immunol. 2011 Feb 01; 186(3):1747-54. View abstract
  40. Monocyte and macrophage abnormalities in systemic lupus erythematosus. Arch Immunol Ther Exp (Warsz). 2010 Oct; 58(5):355-64. View abstract
  41. Monocyte surface expression of Fcgamma receptor RI (CD64), a biomarker reflecting type-I interferon levels in systemic lupus erythematosus. Arthritis Res Ther. 2010; 12(3):R90. View abstract
  42. Endogenous type-I interferon activity is not associated with depression or fatigue in systemic lupus erythematosus. J Neuroimmunol. 2010 Jun; 223(1-2):13-9. View abstract
  43. Type I interferon modulates monocyte recruitment and maturation in chronic inflammation. Am J Pathol. 2009 Nov; 175(5):2023-33. View abstract
  44. Induction of autoimmunity by pristane and other naturally occurring hydrocarbons. Trends Immunol. 2009 Sep; 30(9):455-64. View abstract
  45. Idiopathic nephrotic syndrome and atopy: is there a common link? Am J Kidney Dis. 2009 Nov; 54(5):945-53. View abstract
  46. B cell proliferation, somatic hypermutation, class switch recombination, and autoantibody production in ectopic lymphoid tissue in murine lupus. J Immunol. 2009 Apr 01; 182(7):4226-36. View abstract
  47. Increased expression of FcgammaRI/CD64 on circulating monocytes parallels ongoing inflammation and nephritis in lupus. Arthritis Res Ther. 2009; 11(1):R6. View abstract
  48. TLR7-dependent and FcgammaR-independent production of type I interferon in experimental mouse lupus. J Exp Med. 2008 Dec 22; 205(13):2995-3006. View abstract
  49. Colocalization of antigen-specific B and T cells within ectopic lymphoid tissue following immunization with exogenous antigen. J Immunol. 2008 Sep 01; 181(5):3259-67. View abstract
  50. A novel type I IFN-producing cell subset in murine lupus. J Immunol. 2008 Apr 01; 180(7):5101-8. View abstract
  51. Deficiency of the type I interferon receptor protects mice from experimental lupus. Arthritis Rheum. 2007 Nov; 56(11):3770-83. View abstract
  52. Type I interferon as a novel risk factor for endothelial progenitor cell depletion and endothelial dysfunction in systemic lupus erythematosus. Arthritis Rheum. 2007 Nov; 56(11):3759-69. View abstract
  53. Type I interferon production by tertiary lymphoid tissue developing in response to 2,6,10,14-tetramethyl-pentadecane (pristane). Am J Pathol. 2006 Apr; 168(4):1227-40. View abstract
  54. Short-term atorvastatin treatment enhances specific antibody production following tetanus toxoid vaccination in healthy volunteers. Vaccine. 2006 May 08; 24(19):4035-40. View abstract
  55. Association of anti-nucleoprotein autoantibodies with upregulation of Type I interferon-inducible gene transcripts and dendritic cell maturation in systemic lupus erythematosus. Clin Immunol. 2005 Dec; 117(3):238-50. View abstract
  56. The nuclear IkappaB protein IkappaBNS selectively inhibits lipopolysaccharide-induced IL-6 production in macrophages of the colonic lamina propria. J Immunol. 2005 Mar 15; 174(6):3650-7. View abstract