Adult-Onset Asthma and Periocular Xanthogranulomas Associated with Systemic IgG4-Related Disease.

PubMed ID: 27152364

Author(s): Burris CK, Rodriguez ME, Raven ML, Burkat CN, Albert DM. Adult-onset asthma and periocular xanthogranulomas associated with systemic IgG4-related disease. Am J Ophthalmol Case Rep. 2016 Apr;1:34-37. PMID 27152364

Journal: American Journal Of Ophthalmology Case Reports, Volume 1, Apr 2016

PURPOSE The aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD).

OBSERVATIONS A 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Additional stains revealed CD68 positivity, and S100 negativity. The IgG and IgG4 stained slides showed increased IgG4 positive plasma cells but did not meet the criteria for IgG4-related orbital disease. His IgG4 serology was elevated, and IgG4 staining of his tissue previously diagnosed as autoimmune sclerosing pancreatitis was found to meet the criteria for IgG4-RD.

CONCLUSIONS AND IMPORTANCE AAPOX can be associated with systemic IgG4-RD.