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IgG4-Related Orbital Inflammation Presenting as Unilateral Pseudotumor

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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.

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  1. Carruthers MN, Stone JH, Khosroshahi A. The latest on IgG4-RD: a rapidly emerging disease. Curr Opin Rheumatol. 2012;24:60–9.

    Article  PubMed  CAS  Google Scholar 

  2. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  4. Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17:303.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  7. Deshpande V. The pathology of IgG4-related disease: critical issues and challenges. Semin Diagn Pathol. 2012;29:191–6.

    Article  PubMed  Google Scholar 

  8. Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34:1812–9.

    Article  PubMed  Google Scholar 

  9. Sah RP, Chari ST. Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol. 2011;23:108–13.

    Article  PubMed  CAS  Google Scholar 

  10. Kawano M, Yamada K. Treatment of IgG4-related disease. Curr Immunol Rev. 2011;7:246–51.

    Article  CAS  Google Scholar 

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Correspondence to Mehul Pravinchandra Jariwala.

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Jariwala, M.P., Agarwal, M., Mulay, K. et al. IgG4-Related Orbital Inflammation Presenting as Unilateral Pseudotumor. Indian J Pediatr 81, 1108–1110 (2014).

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