Hypertrophic pachymeningitis can lead to clinical brainstem and cervical spinal cord compression leading to neurologic deficits. IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is one recently recognized etiology of previously idiopathic cases. A 34-year-old right-handed female presented with slowly pro-gressive neurologic symptoms and worsening radiographic syringomyelia. She successfully underwent Chiari decompression and excision of her pachymeningitis with improvement in her radiographic find-ings. Extensive clinical workup has led to a diagnosis of IgG4-RHP and treatment with steroids. IgG4-RHP is a rare cause of spinal cord compression and on our review of the literature this is the first description of significant syringomyelia associated with this condition. This remains a challenging entity to treat and neurology and rheumatology referrals should be placed early to investigate IgG4-RHP as an etiology for idiopathic cases. Treatment of this disease is likely to evolve with further research.(c) 2022 Elsevier Masson SAS. All rights reserved.
机构:
Univ Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USAUniv Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USA
Bledsoe, Jacob R.
Della-Torre, Emanuel
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机构:
Univ Vita Salute San Raffaele, San Raffaele Sci Inst, Unit Immunol Rheumatol Allergy & Rare Dis UnIRAR, Milan, Italy
Ragon Inst MGH MIT & Harvard Univ, Cambridge, MA USAUniv Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USA
Della-Torre, Emanuel
Rovati, Lucrezia
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机构:
Univ Vita Salute San Raffaele, San Raffaele Sci Inst, Unit Immunol Rheumatol Allergy & Rare Dis UnIRAR, Milan, Italy
Ragon Inst MGH MIT & Harvard Univ, Cambridge, MA USAUniv Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USA
Rovati, Lucrezia
Deshpande, Vikram
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机构:
Massachusetts Gen Hosp, James Homer Wright Pathol Labs, Boston, MA 02114 USAUniv Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USA
机构:
Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Fei Yunyun
Peng Yu
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Peng Yu
Zhang Panpan
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Panpan
Zhang Xia
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Xia
Peng Linyi
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Peng Linyi
Zhou Jiaxin
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhou Jiaxin
Zhang Li
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Li
Zhang Shangzhu
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Shangzhu
Liu Jinjing
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Liu Jinjing
Wu Di
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Wu Di
Lai Yamin
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Peking Union Med Coll Hosp, Dept Gastroenterol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Lai Yamin
Liu Xiaowei
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Peking Union Med Coll Hosp, Dept Ophthalmol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Liu Xiaowei
Xue Huadan
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Peking Union Med Coll Hosp, Dept Radiol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Xue Huadan
Zhang Xuan
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Xuan
Zeng Xiaofeng
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zeng Xiaofeng
Zhang Fengchun
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Fengchun
Zhao Yan
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhao Yan
Zhang Wen
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
机构:Chinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R China
Fei Yunyun
Chen Yu
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Chen Yu
Zhang Panpan
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Zhang Panpan
Chen Hua
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Chen Hua
Wu Di
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Wu Di
Zhao Lidan
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Zhao Lidan
Peng Linyi
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Peng Linyi
Wang Li
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Wang Li
Wu Qingjun
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Zhang Xuan
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Zhang Xuan
Zhao Yan
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Zhao Yan
Zeng Xiaofeng
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Zeng Xiaofeng
Zhang Fengchun
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Zhang Fengchun
Zhang Wen
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Chinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R ChinaChinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R China
机构:
Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Matza, Mark A.
Perugino, Cory A.
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Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Ragon Inst MGH MIT & Harvard, Cambridge, MA USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Perugino, Cory A.
Harvey, Liam
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Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Harvey, Liam
Fernandes, Ana D.
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Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Fernandes, Ana D.
Wallace, Zachary S.
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Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Wallace, Zachary S.
Liu, Hang
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Ragon Inst MGH MIT & Harvard, Cambridge, MA USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Liu, Hang
Allard-Chamard, Hugues
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Univ Sherbrooke, Div Rheumatol, Fac Med & Sci Sante, Sherbrooke, PQ, Canada
Ctr Rech Clin Etienne Le Bel, Sherbrooke, PQ, CanadaHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Allard-Chamard, Hugues
Pillai, Shiv
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Ragon Inst MGH MIT & Harvard, Cambridge, MA USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Pillai, Shiv
Stone, John H.
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Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
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Massachusetts Gen Hosp, Div Rheumatol Allergy & Immunol, Boston, MA 02114 USA
Ragon Inst MGH MIT & Harvard, Cambridge, MA USAMassachusetts Gen Hosp, Div Rheumatol Allergy & Immunol, Boston, MA 02114 USA
Perugino, Cory A.
Stone, John H.
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h-index: 0
机构:
Massachusetts Gen Hosp, Div Rheumatol Allergy & Immunol, Boston, MA 02114 USAMassachusetts Gen Hosp, Div Rheumatol Allergy & Immunol, Boston, MA 02114 USA
机构:
Univ Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USAUniv Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USA
Bledsoe, Jacob R.
Della-Torre, Emanuel
论文数: 0引用数: 0
h-index: 0
机构:
Univ Vita Salute San Raffaele, San Raffaele Sci Inst, Unit Immunol Rheumatol Allergy & Rare Dis UnIRAR, Milan, Italy
Ragon Inst MGH MIT & Harvard Univ, Cambridge, MA USAUniv Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USA
Della-Torre, Emanuel
Rovati, Lucrezia
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h-index: 0
机构:
Univ Vita Salute San Raffaele, San Raffaele Sci Inst, Unit Immunol Rheumatol Allergy & Rare Dis UnIRAR, Milan, Italy
Ragon Inst MGH MIT & Harvard Univ, Cambridge, MA USAUniv Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USA
Rovati, Lucrezia
Deshpande, Vikram
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h-index: 0
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Massachusetts Gen Hosp, James Homer Wright Pathol Labs, Boston, MA 02114 USAUniv Massachusetts, UMass Mem Med Ctr, Dept Pathol, Worcester, MA 01605 USA
机构:
Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Fei Yunyun
Peng Yu
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Peng Yu
Zhang Panpan
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Panpan
Zhang Xia
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Xia
Peng Linyi
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Peng Linyi
Zhou Jiaxin
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhou Jiaxin
Zhang Li
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Li
Zhang Shangzhu
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Shangzhu
Liu Jinjing
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Liu Jinjing
Wu Di
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Wu Di
Lai Yamin
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Peking Union Med Coll Hosp, Dept Gastroenterol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Lai Yamin
Liu Xiaowei
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Peking Union Med Coll Hosp, Dept Ophthalmol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Liu Xiaowei
Xue Huadan
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Peking Union Med Coll Hosp, Dept Radiol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Xue Huadan
Zhang Xuan
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Xuan
Zeng Xiaofeng
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zeng Xiaofeng
Zhang Fengchun
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhang Fengchun
Zhao Yan
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
Zhao Yan
Zhang Wen
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Peking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R ChinaPeking Union Med Coll Hosp, Clin Immunol Ctr, Dept Rheumatol, Beijing, Peoples R China
机构:Chinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R China
Fei Yunyun
Chen Yu
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Chen Yu
Zhang Panpan
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Zhang Panpan
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Wu Di
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Wu Di
Zhao Lidan
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Zhao Lidan
Peng Linyi
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Peng Linyi
Wang Li
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Wang Li
Wu Qingjun
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Wu Qingjun
Zhang Xuan
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机构:Chinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R China
Zhang Xuan
Zhao Yan
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h-index: 0
机构:Chinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R China
Zhao Yan
Zeng Xiaofeng
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h-index: 0
机构:Chinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R China
Zeng Xiaofeng
Zhang Fengchun
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h-index: 0
机构:Chinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R China
Zhang Fengchun
Zhang Wen
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h-index: 0
机构:
Chinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R ChinaChinese Acad Med Sci, Dept Rheumatol, Peking Union Med Coll Hosp, Clin Immunol Ctr, Beijing, Peoples R China
机构:
Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Matza, Mark A.
Perugino, Cory A.
论文数: 0引用数: 0
h-index: 0
机构:
Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Ragon Inst MGH MIT & Harvard, Cambridge, MA USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Perugino, Cory A.
Harvey, Liam
论文数: 0引用数: 0
h-index: 0
机构:
Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Harvey, Liam
Fernandes, Ana D.
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h-index: 0
机构:
Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Fernandes, Ana D.
Wallace, Zachary S.
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h-index: 0
机构:
Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Wallace, Zachary S.
Liu, Hang
论文数: 0引用数: 0
h-index: 0
机构:
Ragon Inst MGH MIT & Harvard, Cambridge, MA USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Liu, Hang
Allard-Chamard, Hugues
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sherbrooke, Div Rheumatol, Fac Med & Sci Sante, Sherbrooke, PQ, Canada
Ctr Rech Clin Etienne Le Bel, Sherbrooke, PQ, CanadaHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Allard-Chamard, Hugues
Pillai, Shiv
论文数: 0引用数: 0
h-index: 0
机构:
Ragon Inst MGH MIT & Harvard, Cambridge, MA USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
Pillai, Shiv
Stone, John H.
论文数: 0引用数: 0
h-index: 0
机构:
Harvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USAHarvard Med Sch, Div Rheumatol Allergy & Immunol, Massachusetts Gen Hosp, Boston, MA 02114 USA
机构:
Massachusetts Gen Hosp, Div Rheumatol Allergy & Immunol, Boston, MA 02114 USA
Ragon Inst MGH MIT & Harvard, Cambridge, MA USAMassachusetts Gen Hosp, Div Rheumatol Allergy & Immunol, Boston, MA 02114 USA
Perugino, Cory A.
Stone, John H.
论文数: 0引用数: 0
h-index: 0
机构:
Massachusetts Gen Hosp, Div Rheumatol Allergy & Immunol, Boston, MA 02114 USAMassachusetts Gen Hosp, Div Rheumatol Allergy & Immunol, Boston, MA 02114 USA