Esophageal Dysmotility: A Manifestation of IgG4-Related Sclerosing Disease.
Krystal B Johnson. Duke University Medical Center, Durham, NC
Background: Esophageal motility disorders have a wide range of primary and secondary causes; yet, many cases remain idiopathic in etiology. When medical treatment modalities fail to control symptoms, surgical myotomy or partial esophagogastrectomy can be the only alternative. Following a sentinel case of end-stage dysmotility with markedly increased IgG4-positive plasma cells, this study was undertaken to identify at what frequency additional cases might exist.
Design: A retrospective review was performed of all esophagogastrectomies undertaken for a primary diagnosis of either dysmotility or achalasia between 2000-2010. Including the sentinel case, 10 cases were identified (excluding 4 cases of pseudoachalasia due to malignancy or fistulas). IgG4-positive and IgG-positive plasma cells were each counted in 3 high power fields (hpf) in the most concentrated areas, the average calculated and an IgG4:IgG ratio determined. A cutoff IgG4:IgG ratio of 0.3 was used to define significant elevation in the IgG4-positive plasma cells. These results were correlated with the histologic and clinical findings.
Results: Four of the 10 cases had an IgG4:IgG ratio >0.3 and differed significantly histologically from the remaining 6 cases. The IgG4-positive patients had an average age of 64 and were 25% male (vs. 54 and 50%; differences not significant). The average IgG4:IgG ratios were 0.48 (0.35-0.72) vs. 0.15 (0-0.23; p=0.005). The total average IgG4 counts were also significantly different: 207/hpf (151-287/hpf) vs. 18/hpf (0-48/hpf; p=0.004). These 4 IgG4-positive cases showed inflammation predominantly in the submucosa, with increased follicular hyperplasia (100% vs. 17%; p=0.002) and periductal inflammation (100% vs. 33%; p=0.01), but did not differ significantly with respect to the observed fibrosis, lymphocytic ductulitis, or phlebitis. One patient demonstrated phlebitis, elevated IgG level (1710, normal 588-1573) and ANA titer (1:640). None of the patients had known IgG4-related lesions elsewhere.
Conclusions: Significantly elevated IgG4 counts, IgG4:IgG ratios, periductal inflammation and lymphoid hyperplasia were identified in 40% of our esophageal dysmotility cases over 10 years. These results suggest that some cases of esophageal dysmotility may be part of the IgG4-related sclerosing disease spectrum. If so, such patients would be expected to respond favorably to immunosuppression, obviating the need for invasive management.
Wednesday, March 2, 2011 9:30 AM
Poster Session V # 218, Wednesday Morning