IgG4-related Lung and Pleural Disease: A Clinicopathologic Study of 21 Cases

Institute of Liver Studies, King's College Hospital, London, UK.
The American journal of surgical pathology (Impact Factor: 5.15). 10/2009; 33(12):1886-93. DOI: 10.1097/PAS.0b013e3181bd535b
Source: PubMed


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.

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    • "Since the first case of IgG4-related lung disease was reported in 20045, several cases have been reported in small sized case series. Zen et al.3 classified IgG4-related lung disease into four categories based on radiologic findings: the solid nodular type, the bronchovascular type, the alveolar interstitial type, and the round GGO type. Of these, the alveolar interstitial type, which is characterized by diffuse GGOs and reticular opacities, traction bronchiectasis, and honeycombing, resembles NSIP both radiologically and histologically. "
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    ABSTRACT: Intrathoracic involvement of immunoglobulin G4 (IgG4)-related disease has recently been reported. However, a subset of the disease presenting as interstitial lung disease is rare. Here, we report a case of a 35-year-old man with IgG4-related lung disease with manifestations similar to those of interstitial lung disease. Chest computed tomography showed diffuse ground glass opacities and rapidly progressive pleural and subpleural fibrosis in both upper lobes. Histological findings showed diffuse interstitial lymphoplasmacytic infiltration with an increased number of IgG4-positive plasma cells. Serum levels of IgG and IgG4 were also increased. The patient was diagnosed with IgG4-related lung disease, treated with anti-inflammatory agents, and showed improvement. Lung involvement of IgG4-related disease can present as interstitial lung disease and, therefore, should be differentiated when evaluating interstitial lung disease.
    Tuberculosis and Respiratory Diseases 08/2014; 77(2):85-9. DOI:10.4046/trd.2014.77.2.85
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    • "The frequency and patterns of involvement are not well established. Zen et al.9 collected 21 cases of IgG4-related lung disease, and reported that 5 had pleural lesions. Matsui et al.10 analyzed 18 patients retrospectively by their clinical, radiological and pathological features. "
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    ABSTRACT: Immunoglobulin (Ig) G4-related disease is a recently recognized systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with elevated circulating levels of IgG4. The disease can either be localized to one or two organs, or present as diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or metachronously. In the pulmonary manefestations, lesions associated with IgG4-related disease have been described in the lung parenchyma, airways and pleura, as well as the mediastinum. We report a case of IgG4-related disease presenting as massive pleural effusion and thrombophlebitis.
    Tuberculosis and Respiratory Diseases 04/2014; 76(4):179-83. DOI:10.4046/trd.2014.76.4.179
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    • "Common microscopic findings of these lesions can be characterized as obliterative vasculitis with dense fibrosis and marked infiltration of IgG4-positive plasma cells5. According to the review by Zen et al.1, IgG4-LP can be radiologically categorized into pulmonary lesions (patterns of nodular, bronchovascular, interstitial, and round shaped ground glass opacity) and pleural lesion, and also histologically into solid nodular, bronchovascular and alveolar interstitial type. On clinical and radiologic evaluations, IgG4-LP also can be masqued as interstitial or bronchopneumonia as well as malignancies of lung or pleura1. "
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    ABSTRACT: An immunoglobulin G4 (IgG4)-related disease is a recently emerging entity, and a few cases of IgG4-related disease in lung and pleura have been reported. Herein, we report the case of a 74-year-old man with IgG4-related disease of lung and pleura, clinically suspicious of malignant mesothelioma. Chest computed tomography showed diffuse nodular pleural thickening, and microscopic finding disclosed diffuse thickening of visceral pleura with infiltrations of many lymphoplasma cells with increased number of IgG4-positive plasma cells and a few multinucleated giant cells. It is important for pathologists and clinicians to recognize this rare entity and its histologic finding, because it can be confused with malignant tumors on the radiologic examination although it can be treated with steroid therapy.
    Tuberculosis and Respiratory Diseases 01/2014; 76(1):42-5. DOI:10.4046/trd.2014.76.1.42
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