IgG4-related Retroperitoneal Fibrosis: An Emerging Masquerader With a Sinister Presentation

Chandna, Abhishek; Sharma, Aditya; Pareek, Tarun; Devana, Sudheer Kumar; Bora, Girdhar S.; Mavuduru, Ravimohan; Das, Ashim; Mandal, Arup Kumar

doi:10.1016/j.urology.2019.06.007

Cited by 6 publications

(5 citation statements)

References 12 publications

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“…Por otro lado, al evaluar la terapia con corticoides en pacientes con FRP idiopática debe tenerse en cuenta la remodelación arterial, porque la dilatación aórtica progresiva puede ocurrir durante el tratamiento con esteroides. Aunque tales cambios suelen ser subclínicos, se han reportado casos que requirieron tratamiento quirúrgico por rotura, como sucedió en nuestro paciente (7,9,10).…”

Section: Discussionunclassified

Abdominal aortic rupture in idiopathic retroperitoneal fibrosis

Norese,

Malagrini,

Bevacqua

et al. 2023

Angiologia

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Conflicto de interés: los autores declaran no tener conflictos de interés. RESUMENIntroducción: la fibrosis retroperitoneal (FRP) es una enfermedad poco frecuente que se caracteriza por la formación de tejido inflamatorio y fibroso en el espacio retroperitoneal, alrededor de la aorta abdominal y DE las arterias ilÍacas, incluyendo con frecuencia los uréteres. Caso clínico: describimos el caso de un paciente de 90 años con diagnóstico de FRP tratado previamente con metilprednisona que acudió a urgencias por la rotura de un aneurisma inflamatorio de la aorta abdominal.Palabras clave: Fibrosis retroperitoneal idiopática. Periaortitis crónica.Aneurisma inflamatorio de aorta abdominal.

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Section: Discussionunclassified

Abdominal aortic rupture in idiopathic retroperitoneal fibrosis

Norese,

Malagrini,

Bevacqua

et al. 2023

Angiologia

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“…Although, the treatment modalities of IgG4-related RPF do not differ much from those of idiopathic RPF, differentiation between the two diseases is essential [ 24 ]. The availability of serum IgG4 levels for monitoring treatment response and follow-up can curtail the repeated radiological imaging and associated contrast exposure [ 25 ]. Second, the diagnosis of IgG4-related RPF should alert the clinician to look out for extraretroperitoneal diseases on follow-up of this multiorgan disease [ 26 ].…”

Section: Discussionmentioning

confidence: 99%

Retroperitoneal fibrosis, a rare entity with urorenal and vascular subtypes – preliminary data

et al. 2022

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Introduction Retroperitoneal fibrosis (RPF) is a rare disease associated with the formation of hard inflammatory and fibrous tissue in the retroperitoneum. Taking into consideration the fact that RPF is a rare disease with different subtypes, we compared the basal clinical and biochemical characteristics of the vascular and urorenal subtypes. Patients and methods From January 2005 until December 2021, 27 patients were identified as vascular subtype (18 males) and 11 as urorenal subtype (9 males). Results Patients with a primary urorenal origin had significantly worse kidney function as reflected by serum creatinine and eGFR (both p < 0.001); they also had higher serum cholesterol ( p < 0.01). Hypertension, diabetes, hyperlipidemia and nicotinism were significantly more prevalent in vascular subtype (all p < 0.001). Conclusion Vascular subtype is more prevalent in our study with more cardiovascular risk factor present. Due to the diversity of symptoms, diagnosis of RPF becomes a challenge for specialists as well as therapy.

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“…In the forms of RPF associated with aortic aneurysm, surgical follow-up is recommended, and indication to intervention, surgical or endovascular, does not differ from that considered for common atherosclerotic aortic aneurysms [72]. Although the treatment modalities of IgG4related RPF do not differ much from idiopathic RPF, differentiation between the 2 diseases is essential.…”

Section: Treatmentmentioning

confidence: 99%

Retroperitoneal Fibrosis Is Still an Underdiagnosed Entity with Poor Prognosis

Łoń¹,

Wieliczko²,

Lewandowski³

et al. 2021

Kidney Blood Press Res

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Background: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles abdominal organs including aorta and ureters. Data on the incidence of this disease are limited. Summary: The disease may be idiopathic or secondary to infections, malignancies, drugs or radiotherapy. Idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in aorta and surrounding tissues. Taking into account the dominant symptoms and clinical charac-teristics of patients with periaortitis, two subtypes of disease could be distinguished. Vascular subtype include patients with non-dilated aorta or with inflammatory abdominal aortic aneu-rysm, both with and without involvement of adjacent structures and with numerous risk factors for atherosclerosis. In renoureteral subtype obstructive uropathy manifesting with hydronephro-sis and acute kidney injury is predominant finding. Due to the variety of symptoms, diagnosis of RPF remains challenging, difficult and often delayed. A series of diagnostic tests should be performed, in order to confirm the diagnosis idiopathic RPF. Laboratory work-up include eval-uation of inflammatory indices and immunological studies. A biopsy and histopathological evaluation may be necessary to confirm diagnosis and differentiate the disease. Computed to-mography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) are the modalities of choice for the diagnosis and follow-up of this disease. Management of ureteral obstruction, hydronephrosis, and aortic aneurysms often requires surgical evaluation and treatment. The pharmacological treatment of RPF has been evaluated in a few randomized trials and is mainly based on observational studies. Steroid therapy remains the gold standard of treatment. Key messages: Nowadays multidisciplinary team approach with clinical and diagnos-tic experience in both primary and secondary RPF as well as two major subtypes should be offered. Centers specialized in rare diseases with collaboration with other units and referral sys-tem yield the best possible outcomes.

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IgG4-related Retroperitoneal Fibrosis: An Emerging Masquerader With a Sinister Presentation

Cited by 6 publications

References 12 publications

Abdominal aortic rupture in idiopathic retroperitoneal fibrosis

Abdominal aortic rupture in idiopathic retroperitoneal fibrosis

Retroperitoneal fibrosis, a rare entity with urorenal and vascular subtypes – preliminary data

Retroperitoneal Fibrosis Is Still an Underdiagnosed Entity with Poor Prognosis

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