The Indian Journal of Pediatrics

, Volume 81, Issue 10, pp 1108–1110 | Cite as

IgG4-Related Orbital Inflammation Presenting as Unilateral Pseudotumor

  • Mehul Pravinchandra Jariwala
  • Manjari Agarwal
  • Kaustubh Mulay
  • Sujata Sawhney
Clinical Brief


IgG4 related systemic disease (IgG4-RSD) has been recognised in the last few years. Orbital pseudotumor as a presentation of IgG4-RSD is one of the rare complaints encountered in pediatric population. It is an inflammatory condition of unknown etiology characterized by tumorous swelling of the organs, characteristic histopathologic changes and elevated IgG4: IgG plasma cells ratio. The disease is also characterized by involvement of varied organ systems. The authors describe a seven-year-old boy with orbital pseudotumor after two years of initial onset with waxing and waning course, steroid responsive lesion and biopsy suggestive of IgG4-RSD involving the extraocular soft tissue. Treatment with oral corticosteroids and Azathioprine produced a significant decline in the pseudotumor size. It is important for pediatricians to be aware of this condition as appropriate recognition and management is important to prevent long-term damage of the tissue involved. This is the first case of IgG4 related orbital pseudotumor reported from India.


IgG4 related disease Orbital swelling Pseudotumor 


Conflict of Interest


Source of Funding



  1. 1.
    Carruthers MN, Stone JH, Khosroshahi A. The latest on IgG4-RD: a rapidly emerging disease. Curr Opin Rheumatol. 2012;24:60–9.PubMedCrossRefGoogle Scholar
  2. 2.
    Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539.PubMedCrossRefGoogle Scholar
  3. 3.
    Wallace ZS, Khosroshahi A, Jakobiec FA, Deshpande V, Hatton MP, Ritter J, et al. IgG4-related systemic disease as a cause of “idiopathic” orbital inflammation, including orbital myositis, and trigeminal nerve involvement. Surv Ophthalmol. 2011;57:26.PubMedCrossRefGoogle Scholar
  4. 4.
    Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17:303.PubMedCrossRefGoogle Scholar
  5. 5.
    Plaza JA, Garrity JA, Dogan A, Ananthamurthy A, Witzig TE, Salomão DR. Orbital inflammation with IgG4-positive plasma cells: manifestation of IgG4 systemic disease. Arch Ophthalmol. 2011;129:421–8.PubMedCrossRefGoogle Scholar
  6. 6.
    Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteriafor IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22:21–30.PubMedCrossRefGoogle Scholar
  7. 7.
    Deshpande V. The pathology of IgG4-related disease: critical issues and challenges. Semin Diagn Pathol. 2012;29:191–6.PubMedCrossRefGoogle Scholar
  8. 8.
    Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34:1812–9.PubMedCrossRefGoogle Scholar
  9. 9.
    Sah RP, Chari ST. Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol. 2011;23:108–13.PubMedCrossRefGoogle Scholar
  10. 10.
    Kawano M, Yamada K. Treatment of IgG4-related disease. Curr Immunol Rev. 2011;7:246–51.CrossRefGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2014

Authors and Affiliations

  • Mehul Pravinchandra Jariwala
    • 1
  • Manjari Agarwal
    • 1
  • Kaustubh Mulay
    • 2
  • Sujata Sawhney
    • 1
  1. 1.Department of Pediatric RheumatologySir Ganga Ram HospitalNew DelhiIndia
  2. 2.National Reporting Centre for Ophthalmic Pathology, Centre For SightHyderabadIndia

Personalised recommendations