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Primary Mediastinal Fibrosis (Fibrosing Mediastinitis)

Rare progressive proliferation of dense fibrous tissue in the mediastinum that compresses central vessels, airways and esophagus.

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 Primary Mediastinal Fibrosis (Fibrosing Mediastinitis)?

Primary (idiopathic) mediastinal fibrosis or fibrosing mediastinitis is a rare disorder characterized by an exuberant proliferation of mature fibrous tissue within the mediastinum.

The fibrotic process compresses or invades adjacent vital structures: superior vena cava, pulmonary arteries and veins, central airways, and esophagus. In endemic regions most cases follow histoplasmosis; remaining cases are idiopathic or associated with IgG4-related disease, tuberculosis, or other granulomatous infections.

Diagnosis is suggested by CT/MRI showing infiltrative mediastinal mass with calcifications and confirmed when needed by tissue biopsy.

Symptoms

Superior vena cava syndrome (face/neck swelling, distended neck veins)
Dyspnea on exertion or at rest
Cough, sometimes wheezing
Hemoptysis from pulmonary vein obstruction
Dysphagia from esophageal compression
Recurrent pulmonary infections or post-obstructive pneumonia
Chest pain or pressure

Risk Factors

Prior histoplasmosis exposure (endemic regions)
IgG4-related sclerosing disease
Active or healed tuberculosis
Sarcoidosis
Autoimmune fibroinflammatory disorders
Prior mediastinal radiation
Idiopathic predisposition

When to See a Doctor?

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

  • Persistent SVC syndrome symptoms
  • Progressive dyspnea or wheezing
  • Hemoptysis
  • Mediastinal mass on imaging
  • Recurrent pneumonia in same lobe
  • Worsening dysphagia
  • Chest pain with positional change

Treatment Methods

01
Endovascular SVC stenting for symptomatic compression
02
Pulmonary artery angioplasty/stenting for vascular obstruction
03
Airway stenting in selected cases
04
Anticoagulation if vascular thrombosis present
05
Antifungal therapy for active histoplasmosis
06
Corticosteroids and rituximab for IgG4-related disease
07
Tamoxifen and rituximab in idiopathic disease (selected cases)

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.

Learn About Dahiliye (İç Hastalıkları) Department

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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.