Clinical Rheumatology

, Volume 34, Issue 2, pp 397–401 | Cite as

X-linked agammaglobulinemia combined with juvenile idiopathic arthritis and invasive Klebsiella pneumoniae polyarticular septic arthritis

  • Zaihua Zhu
  • Yuli Kang
  • Zhenlang Lin
  • Yanjing Huang
  • Huoyang Lv
  • Yasong LiEmail author
Case Based Review


X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the Bruton’s tyrosine kinase (BTK) gene. XLA can also present in combination with juvenile idiopathic arthritis (JIA), the major chronic rheumatologic disease in children. We report herein the first known case of a juvenile patient diagnosed with XLA combined with JIA that later developed into invasive Klebsiella pneumoniae polyarticular septic polyarthritis. An additional comprehensive review of XLA combined with JIA and invasive K. pneumoniae septic arthritis is also presented. XLA was identified by the detection of BTK mutations while the diagnosis of JIA was established by clinical and laboratory assessments. Septic arthritis caused by invasive K. pneumoniae was confirmed by culturing of the synovia and gene detection of the isolates. Invasive K. pneumoniae infections can not only result in liver abscesses but also septic arthritis, although this is rare. XLA combined with JIA may contribute to invasive K. pneumoniae infection.


Juvenile idiopathic arthritis Klebsiella pneumoniae Septic arthritis X-linked agammaglobulinemia 



All authors declare no conflict of interest.


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Copyright information

© Clinical Rheumatology 2014

Authors and Affiliations

  • Zaihua Zhu
    • 1
  • Yuli Kang
    • 2
  • Zhenlang Lin
    • 3
  • Yanjing Huang
    • 1
  • Huoyang Lv
    • 4
  • Yasong Li
    • 1
    Email author
  1. 1.Department of RheumatologyZhejiang Provincial People’s HospitalHangzhouChina
  2. 2.Department of Dermatology, Huashan HospitalFudan UniversityShanghaiChina
  3. 3.Department of PediatricsSecond Affiliated Hospital of Wenzhou Medical UniversityWenzhouChina
  4. 4.Clinical laboratoryZhejiang Provincial People’s HospitalHangzhouChina

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