Pulmonary Manifestations of Plasma Cell Type Idiopathic Multicentric Castleman Disease: A Clinicopathological Study in Comparison with IgG4-Related Disease

Nishimura, M.; Igawa, Takuro; Gion, Yuka; Tomita, Sakura; Inoue, Dai; Izumozaki, Akira; Ubara, Yoshifumi; Nishimura, Yoshito; Yoshino, Tadashi; Sato, Yasuharu

doi:10.3390/jpm10040269

Cited by 15 publications

(13 citation statements)

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“…In such cases, it is essential to rule out the possibility of hyper IL-6 syndrome, including PC-iMCD, before confirming the diagnosis of IgG4-RD. As our previous reports have shown, 11,21 immunohistochemical staining for IgA and IL-6 may be useful in making the diagnosis of hyper IL-6 syndrome in this situation.…”

Section: Discussionmentioning

confidence: 79%

“…In IgG4‐RD, there were two cases that met the exclusion criteria. In one of the cases, dense proliferation of mature PCs was observed, but there was also a prominent eosinophilic and lymphocytic infiltration (Figure S1), which was more suggestive of IgG4‐RD 11 . In addition, both cases showed a characteristic distribution of lesions in IgG4‐RD (lacrimal and salivary glands in one case and pancreatic lesions in the other), and steroid administration resulted in rapid improvement of symptoms and laboratory data, indicating that these cases should have been diagnosed as IgG4‐RD.…”

Section: Discussionmentioning

confidence: 97%

“…Note that if the exclusion criteria are met, it does not mean that the possibility of IgG4‐RD is completely ruled out. It is important to make an accurate diagnosis based on the combination of clinical symptoms (including affected organ distribution) and other pathological findings, such as eosinophilic infiltration and immunostaining for IgA and IL‐6 11,21 . In addition, the evaluation of sheet‐like proliferation of mature PCs can vary among pathologists, and the evaluation method could be improved in the future.…”

Section: Discussionmentioning

confidence: 99%

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Investigation of IgG4‐positive cells in idiopathic multicentric Castleman disease and validation of the 2020 exclusion criteria for IgG4‐related disease

Nishikori

Nishimura

et al. 2021

Pathology International

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Patients with plasma cell type idiopathic multicentric Castleman disease (PC‐iMCD) often show elevated serum IgG4 levels and IgG4‐positive cell infiltration in tissues due to overproduction of interleukin‐6, and may meet the diagnostic criteria for IgG4‐related disease (IgG4‐RD). Although PC‐iMCD has been listed as a major exclusion disease for IgG4‐RD, distinguishing between these diseases is challenging due to a lack of highly specific diagnostic biomarkers. In 2020, we proposed exclusion criteria of IgG4‐RD mimickers. In this paper, we validated the accuracy of the criteria in excluding one of the mimickers, PC‐iMCD, from IgG4‐RD. Validation was performed on 57 PC‐iMCD patients (39 presenting lymph node lesions and 19 with lung lesions) and 29 IgG4‐RD patients (22 presenting lymph node lesions and seven with lung lesions). According to our results, 20.5% of the PC‐iMCD patients with lymph node lesions and 42.1% of those with lung lesions met the diagnostic criteria for IgG4‐RD. All these patients with PC‐iMCD were excluded from a diagnosis of IgG4‐RD by the proposed criteria. Additionally, 6.9% of IgG4‐RD patients met the exclusion criteria. Thus, if the exclusion criteria are met, diagnosis should be made based on a combination of findings including organ distribution of disease, response to steroid therapy, and other pathological findings.

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Section: Discussionmentioning

confidence: 79%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

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Investigation of IgG4‐positive cells in idiopathic multicentric Castleman disease and validation of the 2020 exclusion criteria for IgG4‐related disease

Nishikori

Nishimura

et al. 2021

Pathology International

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“…IgG4-RD share similarities with Castleman disease, but there are also differences. MCD and IgG4-RD can be distinguished based on the following aspects: (1) Clinical manifestations: lymph node enlargement in MCD is more prominent and is often accompanied by fever, anemia, severe hypoproteinemia, and other systemic symptoms, while lymph node lesions of IgG4-RD are usually less than 2 cm in diameter and often involve the lacrimal glands, salivary glands, pancreas, and retroperitoneum; (2) Inflammation indicators: CRP, IL-6, and vascular endothelial growth factor are usually significantly increased in MCD patients; (3) In terms of immunoglobulin and complement, increased IgG in MCD patients may be accompanied by increased IgA and IgM, with normal complement levels, while the course of IgG4-RD may involve the activation process of complement, leading to decreased complement levels; (4) Pathological features: IgG4+ plasma cells may appear in MCD patients, but IgG4+/IgG+ plasma cells usually account for less than 40%; and (5) Therapeutic response of glucocorticoids: IgG4-RD patients respond well to initial treatment with glucocorticoids, while MCD patients generally respond poorly[ 4 , 23 - 25 ].…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

Hao¹,

Yang²,

Bian³

et al. 2021

WJCC

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BACKGROUND Immunoglobulin (Ig) G4-associated diseases are a group of systemic diseases involving multiple organs and are also known as IgG4-associated sclerosing diseases. IgG4-associated lymphadenopathy occurring in the lymph nodes is characterized by a lack of specificity due to its clinicopathological characteristics and must be differentiated from a variety of lesions, such as Castleman disease, lymphatic follicular reactive hyperplasia, and lymphoma. CASE SUMMARY A 65-year-old male patient, with Guillain-Barre syndrome for 5 years, presented to our hospital complaining of bilateral orbital mass for 2 years. After hospitalization, the results of the patient’s laboratory tests showed that immunoglobulin subgroup IgG4 was 33.90 g/L and IgG was 30.30 g/L, but serum interleukin-6 was normal. The pathological morphology of orbital mass and cervical lymph node were consistent, which showed that a large number of plasma cells and eosinophils were observed in the lymphatic follicles, and the interstitial fibrous tissue was proliferative. Immunohistochemistry showed that CD20 (B cells) (+), CD3 (T cells) (+), CD38 (+), IgG (+), IgG4 positive cells > 100/high powered field, and IgG4/IgG > 40%. Combined with clinical and immunohistochemical results, lymphadenopathy was consistent with Castleman disease-like IgG4-associated sclerosing disease. Prednisone acetate treatment was given at 40 mg/d. After 2 wk, the superficial lymph nodes and orbital masses shrank, and the IgG4 level decreased. As prednisone acetate was regularly used at a reduced dosage, no recurrence of the disease has been observed. CONCLUSION This case suggested that it is necessary to proceed cautiously in clinical practice with such patients, and immunoglobulin, complement, interleukin-6, C-reactive protein, and other examinations should be performed to confirm the diagnosis.

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“…Clinically, findings suggestive of iMCD are the presence of fever, high CRP, IL-6, and IgA levels and the absence of orbital, salivary gland, and pancreatic involvement. At the histology level, findings compatible with iMCD are the presence of IgA positive cells, expression of IL-6 and hemosiderin deposition, and the absence of eosinophil infiltrate and obliterative phlebitis or arteritis in lymph nodes and other tissues such as the lung 67–70 …”

Section: Mikulicz/systemic Phenotypementioning

confidence: 99%

IgG4-Related Disease

et al. 2021

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Background:The tendency of IgG4-related disease (IgG4-RD) to form pseudotumors, as well as its multisystemic nature, makes it the perfect mimicker of many conditions. Moreover, some clinical, serological, radiological, or histological features of the disease might be shared with some mimickers.Recently, 4 clinical phenotypes have been identified, and patients grouped in each phenotype have distinctive demographic, clinical, and serological features and outcomes, and, as expected, for each phenotype, a set of differential diagnoses should be considered. Summary of the Literature:The main differential diagnoses for the pancreato-hepato-biliary phenotype are pancreatic adenocarcinoma and cholangiocarcinoma. Other differential diagnoses include type 2 autoimmune pancreatitis and primary sclerosing cholangitis. In patients with retroperitoneal/aortic phenotype, inflammatory conditions such as idiopathic retroperitoneal fibrosis and large vessel vasculitides should be ruled out, and most of the time, a biopsy will be needed to exclude malignancies. In head and neck limited phenotype, autoimmune conditions (eg, granulomatosis with polyangiitis, Graves orbitopathy, sarcoidosis), malignancies, and histiocytosis should be ruled out, whereas the main differential diagnoses of the Mikulicz/ systemic phenotype are Sjögren syndrome, granulomatosis with polyangiitis, and multicentric Castleman disease.Conclusions: Approaching a patient with probable IgG4-RD through a clinical phenotype framework will ease the diagnostic algorithm and facilitate the prompt recognition of the disease. There are certain clinical, serological, radiological, and histological features in each clinical phenotype that, if present, increase the likelihood that a patient may have IgG4-RD instead of the mimicker condition. Those clues that point toward IgG4-RD diagnosis should be actively sought in the workup of patients.

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Pulmonary Manifestations of Plasma Cell Type Idiopathic Multicentric Castleman Disease: A Clinicopathological Study in Comparison with IgG4-Related Disease

Cited by 15 publications

References 31 publications

Investigation of IgG4‐positive cells in idiopathic multicentric Castleman disease and validation of the 2020 exclusion criteria for IgG4‐related disease

Investigation of IgG4‐positive cells in idiopathic multicentric Castleman disease and validation of the 2020 exclusion criteria for IgG4‐related disease

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

IgG4-Related Disease

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