Lung Lesions
IgG4-related lung lesions are characterized by inflammatory cell infiltration and fibrosis occurring in the connective tissue (lymph tract) within the lung, mainly along the bronchovascular bundle, interlobular septa, and alveolar interstitium. As a resul
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Lung Lesions Dai Inoue, Yoh Zen, Shoko Matsui, Yuko Waseda, Osamu Matsui, and Toshifumi Gabata
14.1
Introductory Remarks
A variety of pulmonary lesions have been reported in IgG4-RD in the past several years, but many uncertainties remain regarding their pathophysiology and diagnosis. Such lung lesions had originally been reconized as interstinal pneumonia and inflammatory pseudotumor in patients with autoimmune pancreatitis or “Mikulicz’s disease” [1–7]. Knowledge of the wide spectrum of the pulmonary phenotype in IgG4-RD continues to expand [8]. The radiologic features of IgG4-related lung disease rival the clinical findings in their diversity. At least two factors contributed to the delay in recognition of IgG4-related lung disease in the past. First, mucosal lesions in the lung appear to be uncommon. And second, approaches to the diagnosis based upon transbronchial lung biopsy (TBLB) have a low yield. Histopathological diagnosis from lung tissue is essential when no other organ
involvement is evident. In some cases, a definite diagnosis can be obtained only via a video-assisted thoracoscopy (VATS) procedure. IgG4-RD occurs principally in middle-aged and elderly men across the full spectrum of organ involvement. Most cases are associated with elevated serum IgG4 concentrations and good responses to glucocorticoid therapy. Lesions show lymphoplasmacytic cell infiltration and fibrosis, and the infiltrating plasma cells demonstrate disproportionate staining for IgG4. Clinicians from every specialty must become familiar with the clinical and imaging findings specific not only to their individual organ of interest but also of the features that are common to the broader disease as a whole. In this chapter, we outline the lung lesions of IgG4-RD. Our focus is primarily on the imaging findings of IgG4-related lung disease, but where appropriate we also indicate other conditions in the differential diagnosis that must be excluded before settling upon the diagnosis of IgG4-RD.
14.2 D. Inoue (*) Department of Radiology, Kanazawa University, Takara-machi, Kanazawa, Ishikawa 920-8641, Japan Department of Radiology, Toyama Prefectural Central Hospital, Toyama City, Japan e-mail: [email protected] Y. Zen Institute of Liver Studies, King’s College Hospital, London, UK S. Matsui Health Administration Center, University of Toyama 2630 Sugitani, Toyama city, Toyama 930-0194, Japan e-mail: [email protected] Y. Waseda Department of Respiratory Medicine, Kanazawa University, Kanazawa, Japan O. Matsui • T. Gabata Department of Radiology, Kanazawa University, Takara-machi, Kanazawa, Ishikawa 920-8641, Japan
What Lesions Occur Where?
Following careful radiopathologic correlation of surgical biopsies, we observed that four major types of IgG4-related lung disease are readily apparent. These types include (1) solid, nodular lesions; (2) rounded ground-glass opacities (GGO); (3) alveolar-interstitial infiltrates; and (4) a bronchovascular pattern [9]. We also have observed that the inflammatory cell infiltration and fibrosis
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