Reprint of: Pathologic manifestations of Immunoglobulin(Ig)G4-related lung disease.

Published on Sep 1, 2018in Seminars in Diagnostic Pathology2.511
· DOI :10.1053/J.SEMDP.2018.09.004
Eunhee S. Yi47
Estimated H-index: 47
(Mayo Clinic),
Hiroshi Sekiguchi17
Estimated H-index: 17
(Mayo Clinic)
+ 2 AuthorsThomas V. Colby105
Estimated H-index: 105
(Mayo Clinic)
Immunoglobulin(Ig)G4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect virtually any organ and usually presents as tumefactive lesions involving multiple sites. Characteristic histopathology of IgG4-RD consists of dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis, accompanied by tissue infiltration of IgG4-positive plasma cells with or without elevation of serum IgG4 level. Despite a general similarity in the morphologic manifestations of IgG4-RD, site-specific unique morphologic features have been described in some organs including the lung. Compared with other sites, pulmonary involvement by IgG4-RD has been recognized more recently, and lung biopsy interpretation for this condition is often challenging, as both a relative paucity of pathognomonic features and a plethora of overlapping findings with other fibroinflammatory processes of the lung. This review is focused on the morphologic spectrum of IgG4-related lung disease documented in the current literature and on the pertinent issues in the differential diagnoses with other conditions encountered in the lung.
#1Vikram Deshpande (Harvard University)H-Index: 100
#2Yoh Zen (University of Cambridge)H-Index: 69
Last. John H. Stone (Harvard University)H-Index: 76
view all 41 authors...
IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; and—often but not always—elevated serum IgG4 concentrations. An international symposium on IgG4-related disease was held in Boston, MA, on 4–7 October 2011. The organizing committee comprising 35 IgG4-related disease experts from Japan, Korea, Hong Kong, the United Kingdom, Germany, Ital...
1,429 CitationsSource
Lymphadenopathy is frequently observed in patients with immunoglobulin G4-related disease (IgG4-RD) and sometimes appears as the first manifestation of the disease. The diagnosis of IgG4-related lymphadenopathy is complicated owing to a great histological diversity, with at least 5 histological subtypes. Indeed, lymph node biopsy may be performed under the suspicion that the lymphadenopathy is a malignant lymphoma or other lymphoproliferative disorder. The diagnosis of IgG4-RD is characterized b...
56 CitationsSource
#1Jay H. Ryu (Mayo Clinic)H-Index: 80
#2Ryohei Horie (Mayo Clinic)H-Index: 6
Last. Eunhee S. YiH-Index: 47
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IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease ass...
67 CitationsSource
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Fibrosing mediastinitis (FM) and IgG4-related disease (IgG4-RD) are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criter...
46 CitationsSource
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#2Karina A. Keogh (Mayo Clinic)H-Index: 22
Last. Eunhee S. YiH-Index: 47
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Granulomatosis with polyangiitis (Wegener's) (GPA) may mimic IgG4-related disease (IgG4-RD) on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral c...
59 CitationsSource
#1Jay H. Ryu (Mayo Clinic)H-Index: 80
#2Hiroshi SekiguchiH-Index: 17
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Immunoglobulin (Ig)G4-related sclerosing disease (ISD) (also called IgG4-related systemic disease, IgG4-related disease or hyper-IgG4 disease) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4. Although initial descriptions of this disorder focused on its pancreatic presentation (autoimmune pancreatitis), it has become apparent that ISD is a systemic disease with many facets. The lesion of ISD is characterised by lymphoplasmacytic infl...
109 CitationsSource
#1Tai-Di Chen (Memorial Hospital of South Bend)H-Index: 9
#2Li-Yu LeeH-Index: 38
Rosai-Dorfman disease (RDD) is an infrequently encountered disorder characterized by histiocytes exhibiting emperipolesis and varying degrees of chronic inflammatory cell infiltrates. It is generally considered an important differential diagnosis of IgG4related sclerosing disease because of their similar histopathologic features. We report herein a case of RDD in the parotid gland with an increase in the absolute number of IgG4plasma cells as well as an increase in the ratio of IgG4 to IgG plasm...
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#2Jason L. Hornick (Brigham and Women's Hospital)H-Index: 73
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Inflammatory myofibroblastic tumor is a rare mesenchymal neoplasm that harbors an anaplastic lymphoma kinase (ALK) gene rearrangement in the majority of cases. It is composed of fibroblastic–myofibroblastic cells with a characteristic inflammatory infiltrate that consists predominantly of plasma cells. In contrast, IgG4-related sclerosing disease is a recently described multisystem disorder with a histological appearance similar to inflammatory myofibroblastic tumor. The plasma cell infiltrate i...
59 CitationsSource
Purpose of reviewThe spectrum of IgG4-related systemic disease (IgG4-RSD) continues to widen. At most of the sites involved by this condition, the clinical presentation can mimic neoplasm. Pathologic assessment of small biopsies can be critical to proper management. This review summarizes the histol
77 CitationsSource
IgG4-related disease has been identified in various organs, but whether or not there are organ-specific characteristics related to the etiologic factors is still unknown. Here, we carried out a cross-sectional study of 114 patients with IgG4-related disease. On the basis of the location of the lesio
563 CitationsSource
Cited By4
#1Akira Satou (Aichi Medical University)H-Index: 8
#2Kenji NotoharaH-Index: 37
Last. Yasuharu Sato (RMIT: RMIT University)H-Index: 31
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IgG4-related disease (RD) is a relatively new entity, which was first proposed in 2001. Since then, clinical and pathological characteristics of the disease have been investigated. As IgG4-RD has been studied extensively, the diagnostic criteria for IgG4-RD of each organ and the comprehensive diagnostic criteria for IgG4-RD have also been developed. However, one of the biggest challenges in the field is distinguishing between IgG4-RD and mimickers, which show overlapping features with IgG4-RD. I...
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#1Marta Casal Moura (Mayo Clinic)H-Index: 3
#2Ria Gripaldo (Emory University)
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Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that has been recognized to involve virtually any organ in the body and typically manifests mass-like lesions (tumefactive). Although initial reports of this disease (autoimmune pancreatitis [AIP]) were described in the Japanese population, it has since been reported worldwide. It is most commonly seen in adults of middle age or older, more often men than women. The pathogenesis of IgG4-RD is largely unknown, bu...
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