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IgG4-Related Cardiovascular Involvement

Fibroinflammatory IgG4-related disease affecting the aorta, coronary arteries and pericardium.

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 Cardiovascular Involvement?

IgG4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory disorder. Cardiovascular involvement includes inflammatory aortic aneurysms, periaortitis, retroperitoneal fibrosis, coronary periarteritis and constrictive pericarditis.

Histopathology shows lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis, with elevated serum IgG4 in most patients.

Recognition matters because corticosteroids and B-cell depletion are highly effective, in contrast with progressive damage if it is mistaken for ordinary atherosclerosis or large-vessel vasculitis.

Symptoms

Back or abdominal pain (periaortitis)
Chest pain or angina (coronary periarteritis)
Symptoms of constrictive pericarditis (edema, dyspnea)
Hydronephrosis from retroperitoneal fibrosis
Multi-organ involvement (pancreas, salivary glands, lacrimal glands)
Constitutional symptoms: fatigue, weight loss
Lower-limb claudication

Risk Factors

Middle-aged to older men predominantly
Personal or family history of IgG4-RD
Atopic background
Other autoimmune disorders
Smoking (cardiovascular risk addition)

When to See a Doctor?

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

  • Newly diagnosed thoracic or abdominal aortic aneurysm with periaortic soft tissue
  • Constrictive pericarditis without typical cause
  • Recurrent ureteral obstruction
  • Multiorgan symptoms suggesting IgG4-RD
  • Steroid-responsive vasculitic picture

Treatment Methods

01
High-dose oral glucocorticoids (induction)
02
Steroid-sparing agents: rituximab, mycophenolate, azathioprine
03
Surgical or endovascular repair of high-risk aortic aneurysms
04
Pericardiectomy for refractory constrictive pericarditis
05
Cardiovascular risk-factor management
06
Long-term follow-up with serum IgG4 and imaging

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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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.