IgG4-Related Orbital Inflammation Presenting as Unilateral Pseudotumor

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 organ...

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Bibliographic Details
Published in:Indian journal of pediatrics 2014-10, Vol.81 (10), p.1108-1110
Main Authors: Jariwala, Mehul Pravinchandra, Agarwal, Manjari, Mulay, Kaustubh, Sawhney, Sujata
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child
Clinical Brief
General aspects
Gynecology
Humans
Immunoglobulin G - immunology
Inflammation - complications
Inflammation - diagnosis
Inflammation - immunology
Male
Medical sciences
Medicine
Medicine & Public Health
Orbital Diseases - complications
Orbital Diseases - diagnosis
Orbital Diseases - immunology
Orbital Pseudotumor - etiology
Pediatrics
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Summary: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.
ISSN:0019-5456
0973-7693
DOI:10.1007/s12098-014-1471-y