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Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease
Giovanni Maria Rossi1, Rossana Rocco1, Eugenia Accorsi Buttini1
1Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy.
Abstract:
Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.
Insights
Retroperitoneal fibrosis (RPF) is a rare condition causing fibrous tissue growth. Idiopathic RPF (IRF) is now recognized as part of IgG4-related disease (IgG4-RD), impacting clinical practice.
Area of Science:
- Fibro-inflammatory disorders
- Immunopathology
- Retroperitoneal diseases
Background:
- Retroperitoneal fibrosis (RPF) is a rare condition characterized by retroperitoneal fibrous tissue proliferation.
- RPF can be idiopathic or secondary to infections, malignancies, or drug use.
- Idiopathic RPF (IRF) accounts for 75% of cases and typically responds to immunosuppressive therapy.
Purpose of the Study:
- To review the clinical and therapeutic challenges of IRF.
- To discuss the implications of classifying IRF within the IgG4-related disease (IgG4-RD) spectrum.
- To provide insights for clinical practice regarding IRF and IgG4-RD.
Main Methods:
- Literature review of idiopathic retroperitoneal fibrosis (IRF).
- Analysis of the relationship between IRF and IgG4-related disease (IgG4-RD).
- Discussion of clinical presentations and therapeutic strategies.
Main Results:
- IgG4-related disease (IgG4-RD) has illuminated many previously distinct fibro-inflammatory disorders.
- Idiopathic retroperitoneal fibrosis (IRF) is now considered part of the IgG4-RD spectrum.
- Both IRF and IgG4-RD are associated with immune-mediated mechanisms.
Conclusions:
- IRF presents significant clinical and therapeutic challenges for internists.
- The inclusion of IRF within the IgG4-RD spectrum offers new perspectives for diagnosis and management.
- Understanding the immune-mediated nature of IRF and IgG4-RD is crucial for effective patient care.
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