A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge

Gormley, Sinead; Bravo, Paola T.; Kos, Xavier; Solanki, Kamal; Khashram, Manar

doi:10.1016/j.jvscit.2022.02.003

Cited by 6 publications

(6 citation statements)

References 11 publications

(12 reference statements)

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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%

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%

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

Farook,

Jilani,

Islam

et al. 2023

Clinical Case Reports

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“…In general, the response to steroids is good and lesion reduction is observed. [14][15][16][17] If steroids are effective, the catheter may be removed and the patient monitored periodically for hydronephrosis. Other immunosuppressant drugs such as rituximab are reported to be effective against retroperitoneal fibrosis due to IgG4-RD.…”

Section: Discussionmentioning

confidence: 99%

Importance of Awareness and Careful Follow-Up of Suspected IgG4-Related Periaortitis

et al. 2023

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IgG4-related disease may cause large vessel vasculitis, which often affects males in their 60s. Here, we report a case of suspected IgG4-related periaortitis in a 76-year-old man with lower left-side chest pain and hypertension based on computed tomography findings of thickened lesions surrounding the abdominal aorta and mesenteric arteries after ruling out acute cardiovascular diseases. His serum IgG4 levels were high, but the Creactive protein and D-dimer levels were within normal limits. Because IgG4-related periaortitis was suspected, the patient was carefully monitored for blood pressure control, inflammatory markers, and renal function. Steroid therapy was not initiated, however, due to the difficulties performing a biopsy targeting periaortitis to obtain a definitive diagnosis and possible severe complications. During follow-up observation, IgG4-related kidney disease was suspected based on a slight increase in the serum creatinine levels and a renal biopsy was considered. Just before performing the renal biopsy, we observed left renal hydronephrosis caused by spreading retroperitoneal fibrosis. Immediate ureteral stent implantation and initiation of steroid therapy successfully improved the renal function and decreased the serum IgG4 level, respectively. Although relatively rare, IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis should be considered in the differential diagnosis of aortic diseases, even after ruling out serious major acute cardiovascular diseases. Cardiologists should also be aware of the possible progression and systemic spread of this disease.

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“…Może ona dotyczyć wielu narządów, najczęściej trzustki, gruczołów łzowych, przestrzeni zaotrzewnowej i aorty i charakteryzuje się obecnością nacieku limfoplazmatycznego IgG4, zwłóknienia i zarostowego zapalenia żył. [4,7].…”

Section: Idiopatyczne Włóknienie Zaotrzewnoweunclassified

Ormond's disease - the current state of knowledge about diagnosis and treatment

Dankiewicz

Bróż

Żelazny

et al. 2022

J Educ Health Sport

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Introduction: Retroperitoneal fibrosis (Ormond's disease; RPF) is a rare condition characterized by chronic inflammation and resulting progressive fibrosis of the retroperitoneal space. Due to its low prevalence and uncharacteristic symptoms, it is often overlooked in the diagnostic process, resulting in a late-stage diagnosis when complications are already present and treatment options are limited. The aim of the study: The purpose of our study was to present, based on the available literature, the latest reports on the diagnosis and treatment of Ormond disease. State of knowledge: The diagnosis of RPF is based on an anamnesis and imaging. Sometimes a surgical biopsy of the fibrous tissue is necessary. The pathogenesis remains partly unclear. There is no characteristic laboratory marker for early diagnosis of the disease. It is important to distinguish the primary form from the secondary form, in which the trigger factors are known. In therapy, steroid therapy is still the standard. Attempts are being made to treat with other immunosuppressants and biologic drugs with good results, which speaks to the autoimmune basis of the disease. In some cases, surgical treatment and removal of fibrous tissue compressing abdominal structures is essential. Summary: Although the state of knowledge about retroperitoneal fibrosis has been greatly expanded in recent years, many questions remain to be clarified, especially about its pathogenesis and possible early clinical clues to facilitate diagnosis.

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A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge

Cited by 6 publications

References 11 publications

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

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

Importance of Awareness and Careful Follow-Up of Suspected IgG4-Related Periaortitis

Ormond's disease - the current state of knowledge about diagnosis and treatment

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