IgG4 related disease as a cause of isolated retroperitoneal fibrosis with no other organ involvement; Case report

Razok, Almurtada; Malik, Rubab Fatima; Cackamvalli, Priyanka; Zahid, Muhammad

doi:10.1016/j.amsu.2020.12.028

Cited by 3 publications

(4 citation statements)

References 18 publications

(1 reference statement)

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“…In our reported case, imaging tests revealed a retroperitoneal tumorous mass encasing the abdominal aorta, inferior mesenteric artery, inferior vena cava along with bilateral hydronephrosis. Similar findings were reported by Gormley et al and Razok et al in patients with IgG4‐RPF 14,15 . However, imaging techniques cannot distinguish IgG4‐RPF from non‐IgG4‐RPF.…”

Section: Discussionsupporting

confidence: 89%

“…Similar findings were reported by Gormley et al and Razok et al in patients with IgG4-RPF. 14,15 However, imaging techniques cannot distinguish IgG4-RPF from non-IgG4-RPF. Histopathological examination being the key diagnostic test can exclude benign and malignant lesions and can particularly perceive the presence of inflammatory infiltrates in IgG4-RPF.…”

Section: Case Reportmentioning

confidence: 99%

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IgG4‐related retroperitoneal fibrosis: A case report of a challenging disease

Farook,

Jilani,

Islam

et al. 2023

Clinical Case Reports

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Immunoglobulin G4 (IgG4)‐related disease (IgG4‐RD) is a multi‐organ immune‐mediated fibroinflammatory disorder that may imitate malignancy, infectious or any other inflammatory disorder. IgG4‐related retroperitoneal fibrosis (IgG4‐RPF) is a rare form of IgG4‐RD, diagnosis of which is often relied on radiological technology. Herein, we describe a case of 60 year old male, presenting with low back pain and weight loss for a period of 2 months and 15 days. Imaging studies showed a retroperitoneal tumorous mass along with bilateral hydroureteronephrosis, which was later confirmed to be IgG4‐related retroperitoneal fibrosis on the basis of extensive histopathological analysis. Immunosuppressive therapy resulted in a decrease in fibrosis and restoration of renal function.

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Section: Discussionsupporting

confidence: 89%

Section: Case Reportmentioning

confidence: 99%

IgG4‐related retroperitoneal fibrosis: A case report of a challenging disease

Farook,

Jilani,

Islam

et al. 2023

Clinical Case Reports

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“…Figure (5): retroperitoneal soft tissue fibrosis (arrow) with markedly decreased FDP uptake compared to a previous study (Figure 2).…”

Section: Case Presentationmentioning

confidence: 58%

“…the majority of the cases are asymptomatic and usually involve multiple organs. However, isolated IgG4 retroperitoneal fibrosis is extremely rare [5] . It is worth noting that these estimates and statistics are based on small populations and may not represent the whole spectrum of the disease, that's why reporting these cases is essential to create data for future reviews and meta-analysis.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related disease presenting with renal injury and obstructive nephropathy discovered incidentally: a case report with literature review

2022

IJCR

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IgG-4 related disease (IgG4-RD) is a chronic inflammatory condition characterized by tissues infiltration with lymphocytes and IgG4 secreting plasma cells, significant fibrosis and response well to steroids. It is typically a systemic disease that involves various organs. We are presenting a 57-year-old female who was found to have elevated serum creatinine, Proteinuria. An ultrasound scan revealed bilateral hydronephrosis and an abdominal computed tomography (CT) scan illustrated a large retroperitoneal mass. Further evaluation of the mass with whole-body positron emission tomography (PET CT) scan was suggestive of retroperitoneal fibrosis or lymphoma. A CT guided biopsy was done, and results were consistent with fibrosis in the context of IgG-4 related disease. IgG-4 serum levels were elevated. The patient was treated with oral steroids. Azathioprine and Rituximab. Renal function improved significantly and was stable upon 3 months follow up with a decrease in IgG-4 levels and with a significant reduction in the mass lesion.

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組織生検で診断に至ったIgG4関連後腹膜線維症の1例

Nakada

Kumagai

Muraki

et al. 2022

Fukushima Medical Journal

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An 82 -yearold man presented to our department in December 2020 with a complaint of right lumbar back pain. An abdominal computed tomography showed an irregular mass at the right renal hilum. The abdominal CT, retrograde pyelography, and urine cytology did not reveal a renal or urinary tract malignancy, and retroperitoneal fibrosis was also suspected. An echoguided biopsy was performed to confirm the diagnosis because of high levels of serum IgG and IgG4. The histopathological specimens showed diffuse lymphocyte aggregation and fibrosis. The patient was diagnosed as having IgG4related retroperitoneal fibrosis because the IgG4/IgGpositive cell ratio increased to 40 or more. After treatment with prednisolone 20 mg/day, the right hilar mass shrank and the serum IgG4 level decreased.

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IgG4 related disease as a cause of isolated retroperitoneal fibrosis with no other organ involvement; Case report

Cited by 3 publications

References 18 publications

IgG4‐related retroperitoneal fibrosis: A case report of a challenging disease

IgG4‐related retroperitoneal fibrosis: A case report of a challenging disease

IgG4-related disease presenting with renal injury and obstructive nephropathy discovered incidentally: a case report with literature review

組織生検で診断に至ったIgG4関連後腹膜線維症の1例

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