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X-linked agammaglobulinemia combined with juvenile idiopathic arthritis and invasive Klebsiella pneumoniae polyarticular septic arthritis. 2015

Zaihua Zhu, and Yuli Kang, and Zhenlang Lin, and Yanjing Huang, and Huoyang Lv, and Yasong Li
Department of Rheumatology, Zhejiang Provincial People's Hospital, 158 Shangtang Rd, Hangzhou, 310014, Zhejiang, China.

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.

UI MeSH Term Description Entries
D007710 Klebsiella Infections Infections with bacteria of the genus KLEBSIELLA. Infections, Klebsiella,Infection, Klebsiella,Klebsiella Infection
D007711 Klebsiella pneumoniae Gram-negative, non-motile, capsulated, gas-producing rods found widely in nature and associated with urinary and respiratory infections in humans. Bacillus pneumoniae,Bacterium pneumoniae crouposae,Hyalococcus pneumoniae,Klebsiella pneumoniae aerogenes,Klebsiella rhinoscleromatis
D008297 Male Males
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000361 Agammaglobulinemia An immunologic deficiency state characterized by an extremely low level of generally all classes of gamma-globulin in the blood. Hypogammaglobulinemia,Agammaglobulinemias,Hypogammaglobulinemias
D001170 Arthritis, Infectious Arthritis caused by BACTERIA; RICKETTSIA; MYCOPLASMA; VIRUSES; FUNGI; or PARASITES. Arthritis, Bacterial,Arthritis, Septic,Arthritis, Viral,Arthritides, Bacterial,Arthritis, Suppurative,Bacterial Arthritides,Bacterial Arthritis,Infectious Arthritis,Suppurative Arthritis,Septic Arthritis,Viral Arthritis
D001171 Arthritis, Juvenile Arthritis in children, with onset before 16 years of age. The terms juvenile rheumatoid arthritis (JRA) and juvenile idiopathic arthritis (JIA) refer to classification systems for chronic arthritis in children. Only one subtype of juvenile arthritis (polyarticular-onset, rheumatoid factor-positive) clinically resembles adult rheumatoid arthritis and is considered its childhood equivalent. Arthritis, Juvenile Chronic,Arthritis, Juvenile Idiopathic,Arthritis, Juvenile Rheumatoid,Childhood Arthritis,PCJIA,PJIA Polyarticular Juvenile Idiopathic Arthritis,Polyarticular Juvenile Idiopathic Arthritis,Polyarticular-Course Juvenile Idiopathic Arthritis,Still's Disease, Juvenile-Onset,Enthesitis-Related Arthritis, Juvenile,Juvenile Chronic Arthritis,Juvenile Idiopathic Arthritis,Juvenile Rheumatoid Arthritis,Juvenile-Onset Still Disease,Juvenile-Onset Stills Disease,Oligoarthritis, Juvenile,Polyarthritis, Juvenile, Rheumatoid Factor Negative,Polyarthritis, Juvenile, Rheumatoid Factor Positive,Psoriatic Arthritis, Juvenile,Still Disease, Juvenile-Onset,Systemic Arthritis, Juvenile,Arthritides, Childhood,Arthritis, Childhood,Arthritis, Juvenile Enthesitis-Related,Arthritis, Juvenile Psoriatic,Arthritis, Juvenile Systemic,Childhood Arthritides,Chronic Arthritis, Juvenile,Enthesitis Related Arthritis, Juvenile,Idiopathic Arthritis, Juvenile,Juvenile Arthritis,Juvenile Enthesitis-Related Arthritis,Juvenile Oligoarthritis,Juvenile Onset Still Disease,Juvenile Onset Stills Disease,Juvenile Psoriatic Arthritis,Juvenile Systemic Arthritis,Juvenile-Onset Still's Disease,Rheumatoid Arthritis, Juvenile,Still Disease, Juvenile Onset,Still's Disease, Juvenile Onset,Stills Disease, Juvenile-Onset
D040181 Genetic Diseases, X-Linked Genetic diseases that are linked to gene mutations on the X CHROMOSOME in humans (X CHROMOSOME, HUMAN) or the X CHROMOSOME in other species. Included here are animal models of human X-linked diseases. X-Linked Genetic Diseases,Genetic Diseases, X-Chromosome Linked,Disease, X-Linked Genetic,Diseases, X-Linked Genetic,Genetic Disease, X-Linked,Genetic Diseases, X Chromosome Linked,Genetic Diseases, X Linked,X Linked Genetic Diseases,X-Linked Genetic Disease

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