Mucosal Predominant Lymphoplasmacytic Inflammation May Be Associated with but Is Not Specific for Autoimmune and Distal Obstructive Biliary Pathology: An Analysis of 998 Cholecystectomies and Proposal for Diffuse Chronic Superficial Cholecystitis as the Category Designation.
Burcu Saka, Nevra Dursun, Olca Basturk, Leslie Ducato, Pelin Bagci, Oscar Tapia, Juan Carlos Roa, Sudeshna Bandyopadhyay, NVolkan Adsay. Emory, GA; MSKCC, NY; UFRO, Temuco, Chile; WSU, MI
Background: Diffuse mucosal-predominant lymphoplasmacytic inflammation (MPLI), once considered specific for primary sclerosing cholangitis (PSC) and termed “diffuse lymphoplasmacytic sclerosing cholecystitis”, has recently been proposed to be a marker of downstream obstruction, occurring also in “lymphoplasmacytic sclerosing” (autoimmune) pancreatitis (LPSP) and in patients with ampullo-pancreatic carcinomas (APC).
Design: Inflammation & other pathologic mucosal changes were evaluated in 998 cholecystectomies (34 PSC; 13 LPSP; 57 APC; and 894 ordinary chronic cholecystitis-OCC).
Results: Although MPLI appeared to be more closely associated with autoimmune conditions and distal biliary tract obstruction, detected in 16/34(47%) of PSC, 6/13(46%) of LPSP, and 28/57(47%) of APC, when all-comers were considered the most common cause of MPLI was non-specific CC (70/120, 58% of the MPLI cases) and this did not seem to be associated with lithiatic obstruction: 31/246 (12%) of non-obstructive calculous CC, 18/163(11%) of acalculous CC and 4/75(5%) of obstructive calculous CC showed MPLI. The mucosal changes accompanying MPLI had some clinical associations: 1) Atrophic form with flattened mucosa, lighter inflammation, less activity, mucous gland hyperplasia were features of PSC; 2) Active type with significant intraepithelial PMNs and mural thickening with inflammatory nodular-sclerosis were more in keeping with LPSP; 3) Hyperplastic changes with elongated back-to-back folds with edematous tips showing lymphangectasia was more typical of APC. However, overlaps were common, and any of these patterns were ultimately seen more commonly in cases with OCC. IgG4 immunostain was helpful in identifying LPSP but not entirely discriminatory, with 3/13 APC and 3/32 OCCs showing >10/HPF IgG4+ cells.
Conclusions: Although MPLI is one of the prominent patterns of injury in patients with distal biliary tract obstruction and autoimmunity, it is by no means specific; most MPLI occur in association with non-obstructive ordinary CC. Therefore, the term lymphoplasmacytic sclerosing cholecystitis with its autoimmune implications may not be appropriate for this entity, and thus we propose the term diffuse chronic superficial cholecystitis (DCSC). In reporting of DCSC, we recommend documentation of the mucosal changes so that clinical correlative determination of the disease process can be initiated.
Tuesday, March 1, 2011 1:00 PM
Poster Session IV # 71, Tuesday Afternoon