Related Experiment Video
Updated: Aug 9, 2026
Chronic Salmonella Infection Induced Intestinal Fibrosis
Published on: September 22, 2019
IgG4-related disease and isolated retroperitoneal fibrosis: A narrative review
Almurtada Razok1, Maria Emilia Romero Noboa1, Faria Sami1
1Internal Medicine, John H. Stroger Jr. Hospital of Cook County.
Abstract:
Retroperitoneal fibrosis (RPF) can occur due to many etiologies and is categorized into idiopathic and secondary. Etiologies of secondary RPF include medications, autoimmune disease, malignancy, and IgG4-related disease (IgG4-RD). Although IgG4-RD usually involves multiple systems synchronically including the pancreas, aorta, and kidneys, it can present with isolated RPF without involvement of other organ systems. Caution must be exercised in these instances as the diagnosis should be confirmed based on specific clinical, radiographic, and histopathologic criteria. Such confirmation can affect the work-up and therapeutic approach as treatment with corticosteroids can lead to remission, both clinically and radiographically.
Insights
Retroperitoneal fibrosis (RPF) has various causes, including IgG4-related disease (IgG4-RD). Diagnosing isolated RPF due to IgG4-RD requires strict criteria for effective corticosteroid treatment.
Area of Science:
- Nephrology
- Rheumatology
- Pathology
Background:
- Retroperitoneal fibrosis (RPF) is a condition with diverse etiologies, broadly classified as idiopathic or secondary.
- Secondary RPF causes include medications, autoimmune disorders, malignancies, and IgG4-related disease (IgG4-RD).
- IgG4-RD typically affects multiple organs but can manifest as isolated RPF.
Conclusions:
- Isolated RPF requires careful evaluation to identify potential underlying IgG4-related disease.
- Adherence to diagnostic criteria is essential for differentiating causes of RPF.
- Corticosteroid therapy is effective for confirmed IgG4-related RPF, leading to clinical and radiographic remission.
