Gallbladder Pathology in IgG4-Related Sclerosing Disease
Pelin Bagci, Burcu Saka, Nevra Dursun, Olca Basturk, Sudeshna Bandyopadhyay, Kee-Taek Jang, So Yeon Kong, Michael Goodman, Alton B Farris, Alyssa Krasinskas, Yoh Zen, Oscar E Tapia, Juan Carlos Roa, Alton B Farris, Volkan Adsay. Emory University, Atlanta; MSKCC, New York; WSU, Detroit; UPMC, Pittsburgh; King's College Hospital, London, United Kingdom; UFRO, Temuco, Chile
Background: Originally recognized as autoimmune pancreatitis (AIP), IgG4-related sclerosing disease (ISD) is now well established as a systemic autoimmune process that can involve various organs and is characterized by increased number of tissue IgG4 plasma cells (PCs). Gallbladder (GB) manifestations of this disease have not yet been fully elucidated.
Design: Patterns of inflammation in 29 GBs from patients with proven ISD were contrasted with those in 2394 cholecystectomies with various etiologies. IgG4 immunostaining was performed in 156 (Table). The number of IgG4+ PCs was graded as negative (<10/HPF), low (10-29), moderate (30-49), or high (≥50).
Results: 7/29 GBs from ISD patients revealed a distinctive pattern of inflammation associated with delicate fibrosis akin to that seen in AIP (Fig.1). IgG4+ PC grade in these patients was low in 1, moderate 1, high 5. Of the remaining 22, 10 revealed mucosal-predominant lymphoplasmacytic cholecystitis, 2 had eosinophilic chronic cholecystitis, and 10 had non-specific chronic inflammation. Overall, high numbers (≥50/HPF) of IgG4+ PCs were seen in 24% of ISD GBs. However, they were also seen in GBs with non-ISD etiologies, including 10% of GBs with obstructive tumor in the CBD (Table). Among the 10 non-ISD patients with moderate/high numbers of IgG4+ PCs, 5 had diabetes mellitus and 1 had hypothyroidism.
|ETIOLOGY (n,%)||NEGATIVE (<10)||LOW (10-29)||MODERATE (30-49)||HIGH (≥50)|
|Autoimmune Pancreatitis/ISD (29, 18%)||16 (55%)||4 (14%)||2 (7%)||7 (24%)|
|Primary sclerosing cholangitis (20, 13%)||14 (70%)||6 (30%)||0||0|
|Obstructive tumor in the CBD (31, 20%)||23 (74%)||4 (13%)||1 (3%)||3 (10%)|
|Ordinary cholecystitis with plasma cells (76, 49%)||66 (87%)||4 (5%)||5 (7%)||1 (1%)|