scholarly journals A Rare Case: IgG4-Related Chronic Inflammatory Disease with Kidney Involvement

2021 ◽  
Author(s):  
Fatos Mete ◽  
Tuba Mengeneci ◽  
Emre Albayrak ◽  
Yavuz AYAR ◽  
Melike Nalbant ◽  
...  
Keyword(s):  
Lymph Nodes ◽  
Inflammatory Disease ◽  
Rare Case ◽  
Retroperitoneal Fibrosis ◽  
Chronic Inflammatory Disease ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Kidney Involvement ◽  
Multisystemic Disease

IgG4 related disease is an immune-mediated chronic inflammatory disease. It is a multisystemic disease that can lead to retroperitoneal fibrosis with involvement of the pancreas, glands, thyroid, lymph nodes, etc. and characterized histopathologically by lymphoplasmocytic cell infiltration. We evaluated a patient diagnosed with IGG4 related disease impaired kidney function.

scholarly journals Disparities between IgG4-related disease with and without kidney involvement: a case-control study based on 450 patients

2020 ◽  
Author(s):  
Qiaozhu Zeng ◽  
Jingyuan Gao ◽  
Xinyu Zhang ◽  
Aichun Liu ◽  
Zhenfan Wang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Kidney Disease ◽  
Retroperitoneal Fibrosis ◽  
Imaging Finding ◽  
Age At Onset ◽  
Female Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Kidney Involvement ◽  
Low Serum

Abstract Background We aimed to compare the demographic, clinical and laboratory characteristics between IgG4-related kidney disease (IgG4-RKD+) and extrarenal IgG4-related disease (IgG4-RKD-) in a large Chinese cohort, as well as describing the radiological and pathological features of IgG4-RKD+.Methods We retrospectively analyzed the medical records of 450 IgG4-RD patients at Peking University People’s Hospital from January 2004 to January 2020.Results Among the 450 IgG4-RD patients, 66 were diagnosed with IgG4-RKD+. Compared with IgG4-RKD- patients, IgG4-RKD+ patients had older age at onset and at diagnosis (59 vs 54, P=0.007; 61 vs 56, P=0.002, respectively). Male to female ratio of IgG4-RKD+ patients is significantly higher (2.5:1 vs 1.2:1, P=0.015). In the IgG4-RKD+ group, the most commonly involved organs were salivary gland (54.6%), lymph nodes (47.0%) and pancreas (40.9%). More than three organs involved (OR=6.212), retroperitoneal fibrosis involvement (RPF) (OR=12.460) and low serum C3 level (OR=0.202) were risk factors for the kidney disease in IgG4-RD patients. It was found that renal function was impaired in more than 50% of IgG4-RKD+ patients. The most common imaging finding is multiple, often bilateral, hypodense lesions, followed by ureteric obstruction and hydronephrosis related to RPF, and thickening of the renal pelvic wall. Conclusions Involvement of three or more organs, retroperitoneal fibrosis and low serum C3 level were risk factors for the kidney disease in IgG4-RD patients. These findings indicate potential differences in pathogenesis of these two phenotypes.

Clinical features of IgG4-related retroperitoneal fibrosis among 407 patients with IgG4-related disease: a retrospective study

Rheumatology ◽  
2020 ◽  
Author(s):  
Yanying Liu ◽  
Lijuan Zhu ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Fei Yang ◽  
...  
Keyword(s):  
Medical Records ◽  
Retroperitoneal Fibrosis ◽  
Disease Onset ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Kidney Involvement ◽  
Peking University ◽  
Male Patients ◽  
The Common

Abstract Objectives IgG4-related disease (IgG4-RD) is recently recognized as a fibro-inflammatory condition featured by tumefactive lesions in multiple organs, and the retroperitoneum is one of the common involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with retroperitoneum lesion (IgG4-RD RPF+) and retroperitoneum free IgG4-RD (IgG4-RD RPF−) in a large cohort. Methods We carried out a retrospective review of the medical records of 407 cases of IgG4-RD diagnosed at Peking University People’s Hospital between March 2009 and May 2019. Results Among 407 patients, 58 had retroperitoneum affected. As compared with IgG4-RD RPF− patients, IgG4-RD RPF+ patients showed older age at disease onset and diagnosis. IgG4-RD RPF+ group involved more male patients. In terms of organ involvement, IgG4-RD RPF+ group was more frequently presented with kidney involvement, while salivary gland, lacrimal gland and pancreas were more prominent in the IgG4-RD RPF− group. In addition, the CRP, ESR level and creatinine level were significantly higher in IgG4-RD RPF+ patients, and hypocomplementemia were more common in this group. Conclusion We have revealed demographic, clinical and laboratory differences between IgG4-RD RPF+ and RPF− patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

scholarly journals A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110169
Author(s):  
Kim Rouven Liedtke ◽  
Christoph Käding ◽  
Paula Döring ◽  
Sander Bekeschus ◽  
Anne Susann Glitsch
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Inflammatory Diseases ◽  
Histological Change ◽  
Iliac Vessel ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Cystic Tumors ◽  
Retroperitoneal Cyst ◽  
Retroperitoneal Lymphangioma ◽  
Severe Fibrosis

Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4+ fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic.

scholarly journals Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes

2012 ◽  
Vol 25 (7) ◽  
pp. 956-967 ◽  
Author(s):  
Yasuharu Sato ◽  
Dai Inoue ◽  
Naoko Asano ◽  
Katsuyoshi Takata ◽  
Hideki Asaoku ◽  
...  
Keyword(s):  
Lymph Nodes ◽  
Germinal Centers ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals Erdheim-Chester Disease: The Importance of Information Integration

2017 ◽  
Vol 10 (2) ◽  
pp. 613-619 ◽  
Author(s):  
Anna Nikonova ◽  
Khashayar Esfahani ◽  
Guillaume Chausse ◽  
Stephan Probst ◽  
Tina Petrogiannis-Haliotis ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Braf V600e Mutation ◽  
Braf V600e ◽  
High Dose ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Erdheim Chester Disease ◽  
History Of ◽  
Erdheim Chester ◽  
Chester Disease

Background: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. Case Presentation: We describe herein a patient with a history of stroke-like symptoms and retroperitoneal fibrosis that on initial pathology raised the possibility of IgG4-related disease. However, the patient was refractory to high-dose steroids and progressed further, developing an epicardial soft tissue mass and recurrent neurological symptoms. Integration of the above findings with new information at another hospital about a radiological history of symmetrical lower extremities long bone lesions raised the differential diagnosis of ECD. Molecular analysis of formalin-fixed paraffin-embedded tissue of both of the patient’s retroperitoneal biopsies (the second one of which had shown a small focus of foamy histiocytes, CD68+/CD1a–) was positive for BRAF mutation, confirming the diagnosis of ECD. The patient demonstrated a dramatic and sustained metabolic response to vemurafenib on follow-up positron emission tomography scans. Conclusion: This case highlights the need for developing a high index of suspicion for presentations of retroperitoneal fibrosis that could represent IgG4-related disease but fail to respond to steroids. When unusual multisystem involvement occurs, one should consider a diagnosis of a rare histiocytosis. Vemurafenib appears to be an effective treatment for even advanced cases of both ECD and Langerhans histiocytosis bearing the BRAF V600E mutation.

scholarly journals Retroperitoneal Fibrosis Diagnosed as IgG4-related Disease after 35 Years

2019 ◽  
Vol 58 (4) ◽  
pp. 609-613 ◽  
Author(s):  
Satoshi Konno ◽  
Yoshihiro Matsuno ◽  
Shingo Ichimiya ◽  
Masaharu Nishimura ◽  
Yoshikazu Kawakami
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals Ossifying fibrous epulis as an IgG4-related disease of the oral cavity: a case report and literature review

2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Yoshiko Ike ◽  
Takahiro Shimizu ◽  
Masaru Ogawa ◽  
Takahiro Yamaguchi ◽  
Keisuke Suzuki ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Lymph Nodes ◽  
Plasma Cells ◽  
Maxillofacial Surgery ◽  
The Body ◽  
Pathological Diagnosis ◽  
Cervical Lymph Nodes ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Organ Manifestations

Abstract Background Fibrous sclerosing tumours and hypertrophic lesions in IgG4-related disease (IgG4-RD) are formed in various organs throughout the body, but disease in the oral region is not included among individual organ manifestations. We report a case of ossifying fibrous epulis that developed from the gingiva, as an instance of IgG4-RD. Case presentation A 60-year-old Japanese man visited the Department of Oral and Maxillofacial Surgery, Gunma University Hospital, with a chief complaint of swelling of the left mandibular gingiva. A 65 mm × 45 mm pedunculated tumour was observed. The bilateral submandibular lymph nodes were enlarged. The intraoperative pathological diagnosis of the enlarged cervical lymph nodes was inflammation. Based on this diagnosis, surgical excision was limited to the intraoral tumour, which was subsequently pathologically diagnosed as ossifying fibrous epulis. Histopathologically, the ossifying fibrous epulis exhibited increased levels of fibroblasts and collagen fibres, as well as infiltration by numerous plasma cells. The IgG4/IgG cell ratio was > 40%. Serologic analysis revealed hyper-IgG4-emia (> 135 mg/dL). The patient met the comprehensive clinical diagnosis criteria and the American College of Rheumatology and European League Against Rheumatism classification criteria for IgG4-RD. Based on these criteria, we diagnosed the ossifying fibrous epulis in our patient as an IgG4-related disease. A pathological diagnosis of IgG4-related lymphadenopathy was established for the cervical lymph nodes. Concomitant clinical findings were consistent with type II IgG4-related lymphadenopathy. Conclusions A routine serological test may be needed in cases with marked fibrous changes (such as epulis) in the oral cavity and plasma cells, accompanied by tumour formation, to determine the possibility of individual-organ manifestations of IgG4-related disease.

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