Idiopathic retroperitoneal fibrosis: a cross-sectional study of 142 Chinese patients

Liao, Shengyou; Wang, Y; Li, K; Zhu, Jin; Zhang, J; Huang, Feng

doi:10.1080/03009742.2017.1363280

Cited by 5 publications

(2 citation statements)

References 28 publications

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“…In one series, 58% of patients had damage in at least one organ at diagnosis [ 99 ]. Examples of damage include sicca syndrome from resection of a salivary gland, proptosis from a fibrotic orbital pseudotumour, anosmia from sinonasal involvement [ 100–102 ], large vessel aneurysms or dissections [ 103 , 104 ], and chronic ureteral obstruction or tubulointerstitial nephritis leading to chronic kidney disease, including end-stage kidney disease [ 99 , 105–107 ].…”

Section: Monitoring Disease Activity and Assessing Damage In Igg4-rdmentioning

confidence: 99%

Current and future advances in practice: IgG4-related disease

Wallace,

Katz,

Hernandez-Barco

et al. 2024

Rheumatology Advances in Practice

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IgG4-related disease (IgG4-RD) is an increasingly recognized cause of fibroinflammatory lesions in patients of diverse racial and ethnic backgrounds and is associated with an increased risk of death. The aetiology of IgG4-RD is incompletely understood, but evidence to date suggests that B and T cells are important players in pathogenesis, both of which are key targets of ongoing drug development programmes. The diagnosis of IgG4-RD requires clinicopathological correlation because there is no highly specific or sensitive test. Glucocorticoids are highly effective, but their use is limited by toxicity, highlighting the need for studies investigating the efficacy of glucocorticoid-sparing agents. B cell-targeted therapies, particularly rituximab, have demonstrated benefit, but no randomized clinical trials have evaluated their efficacy. If untreated or under-treated, IgG4-RD can cause irreversible organ damage, hence close monitoring and consideration for long-term immunosuppression is warranted in certain cases.

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Section: Monitoring Disease Activity and Assessing Damage In Igg4-rdmentioning

confidence: 99%

Current and future advances in practice: IgG4-related disease

Wallace,

Katz,

Hernandez-Barco

et al. 2024

Rheumatology Advances in Practice

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“…Less frequently, RPF may manifest itself in vascular complications, such as venous thrombosis or ischemic claudication [16]. Patients with IgG4-related RPF complained of symptoms related to associated autoimmune illnesses rather than the more classic abdominal and/or back pain associated with IRPF [34,35].…”

Section: Clinical Presentationmentioning

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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A review of the current treatment methods for retroperitoneal fibrosis with obstructive uropathy

Carey,

Gurumurthy,

Napier‐Hemy

et al. 2024

BJUI Compass

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Introduction and aimsRetroperitoneal fibrosis (RPF) is a fibroinflammatory disease in which patients may suffer obstructive uropathy (OU). The optimum treatment strategy for RPF with secondary OU is currently unclear, and the aim of this literature review is to assess the methods used to treat this patient cohort.MethodsMedline, Embase, Cinahl, the Cochrane Library and PubMed were systematically searched to find studies assessing treatment outcomes in this patient cohort. After reviewing the studies' titles, abstracts and full texts, 12 were found that matched our search aims. Data from these publications were analysed and reported.ResultsThe demographic and symptomatic features of patients across the 12 studies were representative of the general RPF population. No randomised control trials (RCTs) were found, and just one study formally compared outcomes between patients who underwent different treatment strategies. Many of the studies concluded that using medical and surgical methods in combination led to positive outcomes; whereas, others found positive outcomes following a variety of regimens. Many studies also highlighted, however, that significant minorities required further treatment after initial therapy. Conclusions regarding optimum treatment methods were limited as most publications did not formally compare outcomes following different strategies and had an observational study design.ConclusionAlthough positive outcomes were commonly seen following medical, surgical and a combination of treatments, the literature currently lacks research formally comparing outcomes after assigning specific treatment protocols to groups of RPF patients. More research is therefore required to determine how to best manage RPF leading to secondary OU.

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Idiopathic retroperitoneal fibrosis: a cross-sectional study of 142 Chinese patients

Abstract: There was a higher rate in males and younger age at disease onset in these Chinese iRPF patients than in other populations. Acute-phase reactants and serum IgG were elevated in some patients. Tobacco use may be a risk factor for iRPF in Chinese populations.

Cited by 5 publications

References 28 publications

Current and future advances in practice: IgG4-related disease

Current and future advances in practice: IgG4-related disease

Retroperitoneal Fibrosis Is Still an Underdiagnosed Entity with Poor Prognosis

A review of the current treatment methods for retroperitoneal fibrosis with obstructive uropathy

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