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 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 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.

A Case of IgG4-Related Retroperitoneal Fibrosis from the Renal Pelvis Mimicking Bilateral Hydronephrosis

2014 ◽  
Vol 97 (1) ◽  
pp. 118-122 ◽  
Author(s):  
Seiya Inoue ◽  
Chihiro Takahashi ◽  
Katsuya Hikita
Keyword(s):  
Computed Tomography ◽  
Malignant Disease ◽  
Ureteropelvic Junction Obstruction ◽  
Retroperitoneal Fibrosis ◽  
Retrograde Pyelography ◽  
Bilateral Hydronephrosis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Show Evidence

We describe a 49-year-old woman who presented with continuous bilateral lumbago. As the patient's ultrasonography manifestations were very similar to those of bilateral hydronephrosis, we performed retrograde pyelography and ureteroscopy. However, apart from slight left ureteropelvic junction obstruction, there was no hydronephrosis. Since malignant disease could not be completely denied, computed tomography-guided biopsy was performed. However, the tissue did not show evidence of malignancy. As the patient continued to have lumbago, we measured serum IgG4 levels because of suspicion of retroperitoneal fibrosis secondary to IgG4-related disease, which proved to be high. Further, immunostaining of the renal pelvic biopsy samples showed IgG4-positive cells. Therefore, diagnosing IgG4-related retroperitoneal fibrosis, we administered corticosteroids. The patient responded favorably to the drug, with gradual regression of the lesion.

A case of IgG4-related disease with features of Mikulicz’s disease, and retroperitoneal fibrosis and lymphadenopathy mimicking Castleman’s disease

2011 ◽  
Vol 21 (4) ◽  
pp. 410-414 ◽  
Author(s):  
Kenchi Takenaka ◽  
Kazuki Takada ◽  
Daisuke Kobayashi ◽  
Masato Moriguchi ◽  
Masayoshi Harigai ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Castleman’S Disease ◽  
Castleman's Disease ◽  
Mikulicz’S Disease ◽  
Related Disease ◽  
Igg4 Related Disease

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.

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