Skip to main content

Advertisement

Log in

Achromobacter xylosoxidans Sepsis Unveiling X-linked Agammaglobulinemia Masquerading as Systemic-onset Juvenile Idiopathic Arthritis

  • Case Report
  • Published:
Indian Pediatrics Aims and scope Submit manuscript

Abstract

Background

X-linked agammaglobulinemia, a primary immunodeficiency, can present with musculoskeletal manifestations.

Case characteristics

A 4-year-old boy, diagnosed as systemic juvenile idiopathic arthritis at the age of 3 years and treated with biological agents, presented with fever, dyspnea and chest pain. Blood culture and pericardial fluid culture revealed Achromobacter xylosoxidans.

Outcome

Investigation revealed normal serum ferritin but low levels of serum immunoglobulins. Further immunological work-up revealed diagnosis of X-linked agammaglobulinemia. Child improved on antibiotic therapy; treatment with steroids and biological was discontinued.

Message

Underlying immunodeficiency disease must be looked for in children suspected to have juvenile arthritis, more so if they develop unusual serious infection in response to immunomodulatory therapy.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Winkelstein JA, Marino MC, Lederman HM, Jones SM, Sullivan K, Burks AW, et al. X-linked agammaglobulinemia: Report on a United States registry of 201 patients. Medicine. 2006; 85:193–202.

    Article  PubMed  Google Scholar 

  2. Hernandez-Trujillo VP, Scalchunes C, Cunningham-Rundles C, Ochs HD, Bonilla FA, Paris K, et al. Autoimmunity and Inflammation in X-linked Agammaglobulinemia. J Clin Immunol. 2014;34:627–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Gharib A, Gupta S. Skeletal and joint manifestations of primary immunodeficiency diseases. SOJ Immunol. 2016;4:1–13.

    Article  Google Scholar 

  4. Hofer M, Southwood TR. Classification of childhood arthritis. Best Prac Res Clinic Rheumatol. 2002;16:379–96.

    Article  Google Scholar 

  5. Ravelli A, Minoia F, Davy S, Horne AC, Bovis F, Pistorio A, et al. 2016 classification criteria for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. Arthritis Rheumatol. 2016;68:566–76.

    Article  PubMed  Google Scholar 

  6. Igra-Siegman A, Chmel H, Cobbs C. Clinical and laboratory characteristics of achromobacter xylosoxidans infection. J Clin Microbiol. 1980;11:141–5.

    CAS  PubMed  PubMed Central  Google Scholar 

  7. Singh S, Rawat A, Suri D, Gupta A, Garg R, Saikia B, et al. X-linked agammaglobulinemia: Twenty years of singlecenter experience from North West India. Ann Allergy Asthma Immunol. 2016;4:405–11.

    Article  CAS  Google Scholar 

  8. Sheehan WJ, Delmonte OM, Miller DT, Roberts AE, Bonilla FA, Morra M, et al. Novel presentation of Omenn syndrome in association with aniridia. J Allergy Clin Immunol. 2010;4: 967–8.

    Google Scholar 

  9. Weitkamp J-H, Tang Y-W, Haas DW, Midha NK, Crowe JE, Jr. Recurrent achromobacter xylosoxidans bacteremia associated with persistent lymph node infection in a patient with hyper-immunoglobulin M syndrome. Clin Infect Dis. 2000;31:1183–7.

    Article  CAS  PubMed  Google Scholar 

  10. Yokota S, Tanaka T, Kishimoto T. Efficacy, safety and tolerability of tocilizumab in patients with systemic juvenile idiopathic arthritis. Ther Adv Musculoskelet Dis. 2012;4:387–97.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Mahesh Janarthanan.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Janarthanan, M., Gollapalli, S. & Sankaranarayanan, S. Achromobacter xylosoxidans Sepsis Unveiling X-linked Agammaglobulinemia Masquerading as Systemic-onset Juvenile Idiopathic Arthritis. Indian Pediatr 56, 423–425 (2019). https://doi.org/10.1007/s13312-019-1541-3

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13312-019-1541-3

Key messages

Navigation