Lymph node granulomas in immunoglobulin G4‐related disease

Bateman, Adrian C.; Ashton‐Key, Margaret; Jogai, Sanjay

doi:10.1111/his.12658

Cited by 18 publications

(9 citation statements)

References 11 publications

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“…These observations are in support of viewing the definite and borderline categories as a single entity and of broadening the histopathological spectrum of IgG4‐RD. The study also validates prior observations that perifollicular granulomas are occasionally noted, whereas a prominent neutrophilic infiltrate is uncommon …”

Section: Discussionsupporting

confidence: 89%

“…The study also validates prior observations that perifollicular granulomas are occasionally noted, whereas a prominent neutrophilic infiltrate is uncommon. 11 The prospective arm of this study highlights the importance of deriving an IgG4/total IgG ratio; 93% of patients in the prospective cohort showed a ratio of greater than 40% (>30% on ISH), making it the most sensitive tissue-based feature of IgG4-RD. Although the IgG4/total IgG ratio is emphasised in most diagnostic algorithms, 2,12 the cutpoints have not been tested in a large North American cohort.…”

Section: Discussionmentioning

confidence: 89%

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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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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 89%

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

et al. 2019

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“…Although an unusual finding, the presence of granulomas should not preclude a diagnosis of IgG4-RD in the appropriate clinicopathologic context. 12 This interesting case should remind readers that sarcoidosis is a diagnosis of exclusion even in the presence of biopsy features compatible with sarcoidosis.…”

Section: Discussionmentioning

confidence: 93%

48-Year-Old Man With Bilateral Mediastinal Lymphadenopathy, Orbital Pseudotumors, Kidney Masses, and Acute Pancreatitis

Albitar

Carmona

2019

Mayo Clinic Proceedings

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“…The typical histological features combine a dense lymphoplasmacytic infiltrate with abundant IgG4+ plasma cells, storiform fibrosis and obliterative phlebitis [7,13]. Nevertheless, some lesions are suggested to belong to the spectrum of IgG4-related disease even though they have atypical histological aspects [14,15].…”

Section: Discussionmentioning

confidence: 99%

“…Granulomas are usually regarded as a feature that is not seen in IgG4-related disease. In most settings their presence would make this diagnosis very unlikely, even though Bateman et al recently highlighted the fact that granulomas may be seen in lymph nodes in IgG4-related disease [15].…”

Section: Discussionmentioning

confidence: 99%

Hepatic nodular lymphoid lesion with increased IgG4-positive plasma cells associated with primary biliary cirrhosis: a report of two cases

et al. 2015

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The nodular lymphoid lesion of the liver known as reactive lymphoid hyperplasia or pseudolymphoma is rare and its pathogenesis is unknown. We report two cases of nodular lymphoid lesions of the liver with numerous IgG4-positive plasma cells in patients with primary biliary cirrhosis. Histologically, in both cases, the lesion showed a dense lymphoplasmacytic infiltrate with lymphoid follicles and granulomas. Fibrous tissue was scarce and without a storiform pattern. Obliterative phlebitis was not identified. The IgG4+ plasma cell counts were 82 and 76 per high power field, with an IgG4/IgG ratio of 75 and 64 %, respectively, which qualifies the lesions according to the diagnostic criteria for IgG4-related disease as « probable histological feature of IgG4-related disease ». There were no rearrangements of immunoglobulin heavy-chain genes and plasma cells had a polytypic pattern of kappa and lambda light-chain expression. The non-tumor liver showed primary biliary cirrhosis with destructive cholangitis without IgG4 plasma cells. In both cases, IgG4-related disease was not found in other organs neither at the time of diagnosis nor 3 years later. Serum IgG4 levels normalized after local ablation of the lesions. It seems unlikely that these lesions are a manifestation of IgG4-related disease. However, because the pathogenesis of both nodular lymphoid lesions and IgG4-related disease remains unclear, further studies are needed to elucidate a potential link between nodular lymphoid lesions of the liver and an increased number of IgG4 plasma cells. More definite conclusions will be possible when the pathogenesis of IgG4-related disease has been clarified.

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Lymph node granulomas in immunoglobulin G4‐related disease

Abstract: While an unusual finding, the presence of granulomas should not preclude a diagnosis of IgG4-RD in the appropriate clinicopathological context.

Cited by 18 publications

References 11 publications

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

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

48-Year-Old Man With Bilateral Mediastinal Lymphadenopathy, Orbital Pseudotumors, Kidney Masses, and Acute Pancreatitis

Hepatic nodular lymphoid lesion with increased IgG4-positive plasma cells associated with primary biliary cirrhosis: a report of two cases

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