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IgG4-Related Retroperitoneal Fibrosis

Retroperitoneal manifestation of IgG4-related disease characterized by chronic fibroinflammatory tissue surrounding the aorta and ureters.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

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What is IgG4-Related Retroperitoneal Fibrosis?

IgG4-related retroperitoneal fibrosis is the retroperitoneal form of IgG4-related disease, an immune-mediated fibroinflammatory condition characterized by lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. It commonly involves periaortic and periureteric tissue.

Patients typically present with insidious back, abdominal or flank pain, lower extremity edema and constitutional symptoms. Ureteric encasement leads to hydronephrosis and acute kidney injury. Imaging reveals soft tissue cuffing of the abdominal aorta with possible aortitis and aneurysmal change.

Diagnosis requires correlation of clinical findings, elevated serum IgG4, characteristic histopathology and exclusion of malignancy and idiopathic forms. Glucocorticoids are first-line therapy, with rituximab and steroid-sparing immunosuppressants for relapse or steroid intolerance.

Symptoms

Persistent low back or flank pain
Lower extremity edema
Hydronephrosis-related symptoms
Weight loss and fatigue
Hypertension
Constipation or urinary symptoms
Vascular claudication if aortitis

Risk Factors

Multifocal IgG4-related disease
Tobacco and asbestos exposure
Prior idiopathic retroperitoneal fibrosis
Male sex and middle age
Atopic background
Autoimmune diathesis
Family clustering of IgG4-related disease

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • New persistent flank or back pain
  • Decreased urine output or rising creatinine
  • Hydronephrosis on imaging
  • Aortic dilation or aortitis features
  • Multisystem features of IgG4-RD
  • Relapse on tapering corticosteroids

Treatment Methods

01
Glucocorticoids as first-line therapy
02
Steroid-sparing agents (mycophenolate, methotrexate)
03
Rituximab for refractory or relapsing disease
04
Ureteral stents or nephrostomy for obstruction
05
Vascular surgical consultation if aortitis
06
Long-term IgG4 and imaging surveillance
07
Multidisciplinary follow-up

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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