Comparison of clinical and pathological features of lung lesions of systemic IgG4‐related disease and idiopathic multicentric Castleman's disease

Terasaki, Yasuhiro; Ikushima, Soichiro; Matsui, Shoko; Hebisawa, Akira; Ichimura, Yasunori; Izumi, Shinyu; Ujita, Masuo; Arita, Machiko; Tomii, Keisuke; Komase, Yuko; Owan, Isoko; Kawamura, Tomohiro; Mori, Yasuo; Murakami, Miho; Ishimoto, Hiroshi; Kimura, Hiroshi; Bando, Masashi; Nishimoto, Norihiro; Kawabata, Yoshinori; Fukuda, Yuh; Ogura, Takashi

doi:10.1111/his.13186

Cited by 36 publications

(27 citation statements)

References 30 publications

(64 reference statements)

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“…MCD is a non-malignant lymphoproliferative disease with hyper IL-6 production. It may show mediastinal lymphadenopathy and GGO in bilateral lung fields with high concentrations of serum IgG4 and high levels of IgG4-positive lymphoplasmacytic infiltrates in lung tissues [36,37]. However, MCD may present with polyclonal hypergammopathy, with elevated serum CRP concentration and IL-6, and an absence of active fibrosis and lower amounts of eosinophil infiltrates in lung tissues unlike patients with IgG4-RRD.…”

Section: Multicentric Castleman's Diseasementioning

confidence: 99%

IgG4-related respiratory disease

Matsui

2019

Modern Rheumatology

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IgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respiratory disease (IgG4-RRD). IgG4-RRD has few subjective symptoms, and is usually detected during workup of patients with extra-thoracic lesions of IgG4-RD. IgG4-RRD is characterized by various conditions, including masses, nodules, thickening, and infiltration at numerous sites in the thorax through lymphatic routes. Although elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells are characteristic findings of IgG4-RD, other intrathoracic diseases, such as multicentric Castleman disease and malignancy, may present with similar findings. Developing diagnostic criteria for IgG4-RRD, including clinicoradiological and pathological characteristics, is necessary for its appropriate diagnosis.

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Section: Multicentric Castleman's Diseasementioning

confidence: 99%

IgG4-related respiratory disease

Matsui

2019

Modern Rheumatology

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“…The mimics of IgG4‐RD examined in this study include granulomatosis and polyangiitis, chronic sialadenitis including lymphoepithelial sialadenitis, non‐IgG4‐related retroperitoneal fibrosis and sclerosing mesenteritis, pulmonary plasma cell granuloma and organising pneumonia, rheumatoid arthritis, and non‐IgG4‐related orbital pseudotumour. Inflammatory myofibroblastic tumour and multicentric Castleman disease are other conditions that show overlapping features with IgG4‐RD . Granulomatosis and polyangiitis and inflammatory myofibroblastic tumour may show storiform‐type fibrosis, and obliterative phlebitis has been observed in the latter neoplasm .…”

Section: Discussionmentioning

confidence: 99%

“…Inflammatory myofibroblastic tumour and multicentric Castleman disease are other conditions that show overlapping features with IgG4-RD. 18 Granulomatosis and polyangiitis and inflammatory myofibroblastic tumour may show storiform-type fibrosis, and obliterative phlebitis has been observed in the latter neoplasm. 19,20 Almost all of the aforementioned diseases can show elevated numbers of IgG4-positive cells, although, with the exception of inflammatory myofibroblastic tumour and multicentric Castleman disease, an elevated IgG4/total IgG ratio is distinctly uncommon.…”

Section: Discussionmentioning

confidence: 99%

The histological diagnosis of IgG4‐related disease on small biopsies: challenges and pitfalls

et al. 2019

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Aims The pathological diagnosis of IgG4‐related disease (IgG4‐RD) relies on histology, IgG4‐positive cells, and an increased IgG4/IgG ratio. Small biopsies from patients with a presumptive diagnosis of IgG4‐RD often fail to meet consensus histological criteria. The aims of this study were to evaluate consecutive small biopsies from patients with a presumptive diagnosis of IgG4‐RD, and to assess the significance of the pathological findings. Methods and results We evaluated 55 small biopsies from patients with a presumptive diagnosis of IgG4‐RD. The retrospective cohort comprised 71 patients with IgG4‐RD and 57 mimics. We performed immunohistochemistry (IHC) and in‐situ hybridisation (ISH) for IgG4 and IgG. Twenty‐six patients from the prospective cohort met the histological criteria for IgG4‐RD (definite); 29 patients lacked one or more pathological features (borderline). Twenty biopsies (36%) lacked both storiform fibrosis and obliterative phlebitis, and nine (16%) lacked an increase in the number of IgG4‐positive plasma cells. Ninety‐three per cent of patients showed an IgG4/total IgG ratio of >40% (>30% by ISH). There were no differences in the incidence of multiorgan disease (P = 0.9), serum IgG4 levels (P = 0.6) and response to therapy between the definite and borderline groups. A strong correlation (Pearson 0.77) between the IHC and ISH platforms was noted with regard to the IgG4/total IgG ratio. Conclusion Patients with a presumptive diagnosis of IgG4‐RD but lacking the characteristic pathological features of this disease appear to be clinically similar to those who meet the current pathological criteria. An elevated IgG4/total IgG ratio is the most sensitive pathological feature, and ISH provides a robust quantification platform. We recommend evaluating tumefactive lymphoplasmacytic infiltrates with an increased IgG4/IgG ratio, regardless of histological features, for IgG4‐RD.

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“…The 3 main pathological features are dense lymphoplasmacytic infiltration, obliterative phlebitis, and a storiform fibrosis pattern defined as radially arranged collagen fibers that seem to weave through the tissue [64,101], with occasional eosinophilic infiltration [86]. Lesions may have a lymphatic distribution [75,102]. Epithelioid cell granulomas and prominent neutrophilic infiltrate are considered inconsistent with IgG4-RD diagnosis [101].…”

Section: Igg4-related Respiratory Diseasementioning

confidence: 99%

“…The appropriate cut-off for IgG4 plasma cell counts may vary from organ to organ and according to the importance of fibrosis [101]. IgG4-RRD should not be diagnosed only based on IgG4 plasma cell accumulation as it can also occur in a large wide of inflammatory conditions (Castleman disease, collagen disease-related lung disease, vasculitis, Rosai-Dorfman disease), lymphomas, or lung cancer [79,101,102]. Association with other patterns has been reported including usual interstitial pneumonia, nonspecific interstitial pneumonia, organizing pneumonia, and granulomatosis with polyangiitis [75,103,104,105].…”

Section: Igg4-related Respiratory Diseasementioning

confidence: 99%

The Lung in Dysregulated States of Humoral Immunity

Uzunhan¹,

Jeny²,

Kambouchner³

et al. 2017

Respiration

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In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis. IgG4-related respiratory disease, often associated with extrapulmonary localizations, presents with solitary nodules or masses, diffuse interstitial lung diseases, bronchiolitis, lymphadenopathy, and pleural or pericardial involvement. Diagnosis relies on international criteria including serum IgG4 dosage and significantly increased IgG4/IgG plasma cells ratio in pathologically suggestive biopsy. Respiratory amyloidosis presents with tracheobronchial, nodular, and cystic or diffuse interstitial lung infiltration. Usually of AL (amyloid light chain) subtype, it may be localized or systemic, primary or secondary to a lymphoproliferative process. Very rare other diseases due to nonamyloid IgG deposits are described. Among the various lung manifestations of dysregulated states of humoral immunity, this article covers only those associated with the common variable immunodeficiency, IgG4-related disease, amyloidosis, and pulmonary light-chain deposition disease. Autoimmune connective-vascular tissue diseases or lymphoproliferative disorders are addressed in other chapters of this issue.

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Comparison of clinical and pathological features of lung lesions of systemic IgG4‐related disease and idiopathic multicentric Castleman's disease

Cited by 36 publications

References 30 publications

IgG4-related respiratory disease

IgG4-related respiratory disease

The histological diagnosis of IgG4‐related disease on small biopsies: challenges and pitfalls

The Lung in Dysregulated States of Humoral Immunity

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