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IgG4-Related Disease

Newly defined systemic fibroinflammatory disease causing tumor-like lesions in multiple organs.

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)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is IgG4-Related Disease?

IgG4-related disease (IgG4-RD), defined in the 2000s, is a systemic fibroinflammatory disease causing mass lesions in multiple organs, depositional fibrosis, and IgG4-positive plasma cell infiltration. The clinical spectrum, beginning with type 1 autoimmune pancreatitis, is now known to be very broad.

Affected organs include the pancreas (autoimmune pancreatitis type 1), salivary glands (Mikulicz disease), retroperitoneum (Ormond disease), aorta (inflammatory aortitis), thyroid (Riedel thyroiditis), bile ducts (IgG4 sclerosing cholangitis), orbit, lung, and kidney (tubulointerstitial nephritis).

Although serum IgG4 is generally elevated, it is insufficient for diagnosis; definitive diagnosis requires biopsy with storiform fibrosis and obliterative phlebitis along with IgG4+/IgG+ plasma cell ratio >40%. Excellent response to steroids is one of the clinical features.

Symptoms

Variable symptoms based on involved organ
Painless salivary and lacrimal gland swelling
Painless pancreatic mass and jaundice
Retroperitoneal fibrosis and back pain
Painless neck mass (Riedel thyroiditis)
Proptosis and orbital involvement findings
Hydronephrosis (ureteral compression)
Mild systemic symptoms (fatigue, weight loss)

Risk Factors

Advanced adult age (mean over 60 years)
Male sex (2-4 times more common)
Atopy and allergy history (~40%)
History of autoimmune pancreatitis
Concomitant chronic fibrosis diseases
Increased frequency in Asian populations
Family history of systemic fibrosis
Eosinophilia and high IgE

When to See a Doctor?

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

  • Painless organ enlargements and mass lesions
  • Unexplained obstructive jaundice
  • Recurrent salivary gland swellings
  • Detection of retroperitoneal fibrosis
  • Unexplained lesions in multiple organs
  • Reported high IgG4 level

Treatment Methods

01
Glucocorticoid therapy (oral prednisolone 30-40 mg/day) as first-line
02
Recurrence monitoring during steroid taper
03
Rituximab in resistant or relapsing cases
04
Azathioprine, mycophenolate, methotrexate as maintenance agents
05
Surgery usually unnecessary (mass is responsive)
06
Multidisciplinary organ-specific follow-up
07
Confirmation by biopsy and IgG4 staining at diagnosis
08
Serum IgG4 monitoring for treatment response

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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IgG4-Related Disease — Detailed Review

Dahiliye (İç Hastalıkları)

IgG4-related disease is a multisystem chronic fibroinflammatory syndrome characterized by tumefactive lesions, dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis; it commonly affects the pancreas, biliary tree, salivary glands, retroperitoneum, and orbits, and responds dramatically to glucocorticoids and rituximab.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.