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

Published on Dec 10, 2020in Journal of Personalized Medicine4.433
· DOI :10.3390/JPM10040269
Midori Filiz Nishimura1
Estimated H-index: 1
,
Takuro Igawa5
Estimated H-index: 5
+ 7 AuthorsYasuharu Sato32
Estimated H-index: 32
Sources
Abstract
Plasma cell type idiopathic multicentric Castleman disease (PC-iMCD) occasionally manifests as parenchymal lung disease. This study aimed to elucidate the detailed clinicopathological features of lung lesions in PC-iMCD and compare the findings with those in immunoglobulin (Ig) G4-related disease (IgG4-RD), the most difficult differential diagnosis of PC-iMCD. We analyzed the clinicopathological findings and immunohistochemical expression patterns of interleukin-6 (IL-6) and Igs in lung specimens from 16 patients with PC-iMCD and 7 patients with IgG4-RD. Histologically, pulmonary PC-iMCD could not be differentiated from IgG4-RD based on lesion distribution patterns, the number of lymphoid follicles and obliterative vasculitis, or fibrosis types. The eosinophil count was higher in the IgG4-RD group than in the PC-iMCD group (p = 0.004). The IgG4/IgG-positive cell ratio was significantly higher in the IgG4-RD group (p < 0.001). The IgA-positive cell count and IL-6 expression intensity were higher in the PC-iMCD group than in the IgG4-RD group (p < 0.001). Based on these findings, we proposed a new diagnostic approach to differentiate lung lesions of PC-iMCD and IgG4-RD. Our approach can be utilized to stratify patients with suspected lung-dominant PC-iMCD to identify candidates for strong immunosuppressive treatment, including IL-6 blockade, at an early stage.
References32
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#1Akira Satou (Aichi Medical University)H-Index: 8
#2Kenji NotoharaH-Index: 41
Last. Yasuharu Sato (RMIT: RMIT University)H-Index: 32
view all 7 authors...
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...
2 CitationsSource
#1Yanyan Han (Okayama University)H-Index: 1
#2Takuro Igawa (Okayama University)H-Index: 5
Last. Yasuharu Sato (RMIT: RMIT University)H-Index: 32
view all 7 authors...
Plasma cell-type Castleman disease (PCD) is a rare idiopathic atypical lymphoproliferative disorder. It is difficult to differentiate between PCD and IgG4-related disease (IgG4-RD) based on histology alone. As PCD often presents with abundant hemosiderin deposition, lymph node lesions obtained from 22 PCD patients and 12 IgG4-RD patients were analyzed using Prussian blue staining to clarify whether hemosiderin deposition is effective in distinguishing between these two diseases. The analysis dis...
2 CitationsSource
#1Zachary S. Wallace (Harvard University)H-Index: 27
#2Ray NadenH-Index: 7
Last. John H. Stone (Harvard University)H-Index: 94
view all 24 authors...
OBJECTIVE: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. METHODS: An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators use...
54 CitationsSource
#1Ming‐Tsung Chen (NDMC: National Defense Medical Center)H-Index: 1
#2Shih‐Chun Lee (NDMC: National Defense Medical Center)H-Index: 1
Last. Chen-Liang Tsai (NDMC: National Defense Medical Center)H-Index: 8
view all 4 authors...
: Castleman's disease (CD) is an uncommon lymphoproliferative disorder characterized as either unicentric or multicentric presentation based on the involving sites. The most frequent presentation of CD is a solitary mediastinal mass. We reported a patient with a history of heavy smoking with particular image features of CD, which presented as mediastinal lymphadenopathy and peribronchovascular interstitial thickening mimicking lung cancer or sarcoidosis initially.
2 CitationsSource
#1Frits van Rhee (University of Arkansas for Medical Sciences)H-Index: 64
#2Peter M. VoorheesH-Index: 34
Last. David C. Fajgenbaum (UPenn: University of Pennsylvania)H-Index: 16
view all 42 authors...
Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8–negative/idiopathic (iMCD). Treatment of iMCD is challenging, and outcomes can be poor because no uniform treatment guidelines exist, few systematic studies have been conducted, and no agreed upon r...
74 CitationsSource
#1Takeshi Saraya (Kyorin University)H-Index: 19
#2Kosuke Ohkuma (Kyorin University)H-Index: 5
Last. Hajime Takizawa (Kyorin University)H-Index: 20
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Abstract Background Immunoglobulin G4-related disease (IgG4-RD) is a rare multi-organ disorder. Physicians rarely encounter patients with IgG4-RD and its range of symptoms. Methods To elucidate the clinical characterization of IgG4-RD, along with the clinical significance of lung involvement, we retrospectively reviewed the medical records of patients who satisfied the comprehensive diagnostic criteria for IgG4-RD. Results We identified 52 patients with IgG4-RD. Of these, 32 patients underwent t...
8 CitationsSource
#1Yasuhiro Terasaki (Nippon Medical School)H-Index: 20
#2Shinichro IkushimaH-Index: 1
Last. Takashi OguraH-Index: 55
view all 21 authors...
Aims The lung lesion [immunoglobulin (Ig)G4-L] of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion [idiopathic multicentric Castleman's disease (iMCD-L)] of idiopathic multicentric Castleman's disease (iMCD). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data. Methods and results Nine patients had IgG4-L (high levels of ser...
14 CitationsSource
#1Li Yu (NCU: Nanchang University)H-Index: 4
#2Meifeng Tu (PKU: Peking University)H-Index: 7
Last. Ken H. Young (University of Texas Health Science Center at Houston)H-Index: 64
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Castleman disease (CD) comprises 3 poorly understood lymphoproliferative variants sharing several common histopathological features. Unicentric CD (UCD) is localized to a single region of lymph nodes. Multicentric CD (MCD) manifests with systemic inflammatory symptoms and organ dysfunction due to cytokine dysregulation and involves multiple lymph node regions. Human herpesvirus 8 (HHV-8) causes MCD (HHV-8–associated MCD) in immunocompromised individuals, such as HIV-infected patients. However, >...
61 CitationsSource
#1David C. Fajgenbaum (UPenn: University of Pennsylvania)H-Index: 16
#2Thomas S. Uldrick (NIH: National Institutes of Health)H-Index: 30
Last. Megan S. Lim (UPenn: University of Pennsylvania)H-Index: 55
view all 38 authors...
Human herpesvirus-8 (HHV-8)–negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accounts for one third to one half of all cases of MCD and can occur in individuals of any age. Accurate diagnosis is challenging, because no standard diagnostic criteria or diagnostic b...
147 CitationsSource
#1Akihiro Manabe (Okayama University)H-Index: 3
#2Takuro Igawa (Okayama University)H-Index: 5
Last. Yasuharu Sato (RMIT: RMIT University)H-Index: 32
view all 6 authors...
Plasma cell-type Castleman disease (PCD) is often encountered when differentiating IgG4-related disease (IgG4-RD). Given that serum IgA is often elevated in Castleman disease, we investigated whether IgA expression levels in histological specimens can be used to differentiate between the two diseases. Lymph node lesions obtained from 12 IgG4-RD and 11 PCD patients were analysed by immunohistochemistry with anti-IgG, -IgG4, and -IgA antibodies. In addition to all 12 cases of IgG4-RD, 8/11 cases (...
7 CitationsSource
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