The American Journal of Surgical Pathology. 33(12):1886-1893, DEC 2009
DOI: 10.1097/PAS.0b013e3181bd535b
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PMID: 19898222
Issn Print: 0147-5185
Publication Date: 2009/12/01
IgG4-related Lung and Pleural Disease: A Clinicopathologic Study of 21 Cases
Yoh Zen;Dai Inoue;Azusa Kitao;Manabu Onodera;Hitoshi Abo;Shiro Miyayama;Toshifumi Gabata;Osamu Matsui;Yasuni Nakanuma;
+ Author Information
*Institute of Liver Studies, King's College Hospital, London, UKDepartments of †Pathology‡Radiology, Kanazawa University Graduate School of Medicine, Kanazawa§Department of Radiology, Toyama Prefectural Central Hospital, Toyama∥Department of Radiology, Fukui Saiseikai Hospital, Fukui, Japan
Abstract
Immunoglobulin G4 (IgG4)-related disorders can occur in the respiratory system. However, the clinicopathologic characteristics have not been well clarified. In this study, we examined clinical and pathologic features of, and follow-up data on, IgG4-related lung and pleural lesions. The patients group consisted of 17 males and 4 females with an average age of 69 years (range: 42 to 76). Pulmonary lesions in 16 patients and pleural lesions in 5 patients were examined. Histologically, all lesions showed diffuse lymphoplasmacytic infiltration. Irregular fibrosis and obliterative vascular changes were more common in solid areas. Nine cases (43%) had eosinophilic infiltration with more than 5 cells per high-power field. Immunostaining revealed numerous IgG4-positive plasma cells in inflamed areas. Sclerosing inflammation was distributed with intrapulmonary connective tissue. Pulmonary lesions showed a variety of morphologic changes according to the predominant area of inflammation. Serum IgG4 concentrations were elevated in 9 of 11 patients tested (average 6.9 g/L; range 0.3 to 18.0 g/L; normal range <1.35 g/L). Extra-pulmonary and extra-pleural IgG4-related lesions were identified in 9 patients (43%), and developed simultaneously or asynchronously during follow up. All patients treated with steroids responded, but some radiologic abnormalities remained in 3 patients. Interestingly, 1 patient was found to have a primary adenocarcinoma against a background of IgG4-related lung disease during follow up. In conclusion, IgG4-related diseases show a greater variety of pulmonary and pleural lesions than previously thought. It is important, therefore, to know the morphologic variety and clinicopathologic characteristics of this disorder.
