Overview

Fibrosing mediastinitis is a rare but non-cancerous condition where tough, fibrous tissue builds up in the space between the lungs (the mediastinum). This can put pressure on important structures like the windpipe, food pipe (oesophagus), blood vessels, and airways, leading to breathing difficulties, problems with swallowing, or circulation issues.¹ This disease can affect individuals between the ages of 13 and 65, with young women being more commonly diagnosed.² There are two main types of this condition:  granulomatous and nongranulomatous. Primary causes of the granulomatous type include tuberculosis and histoplasmosis, whereas the non-granulomatous form is an unexplained response to radiation, medication, and autoimmune disorders.²  

The main goal of treating fibrosing mediastinitis is to clear the airway and vascular blockages.³ A chest X-ray may show swelling in the chest area or enlarged lymph nodes, although these signs aren’t always specific to this condition.² In some cases, this might result in the trachea (windpipe) and main airways (bronchi) becoming more narrow, causing breathing difficulties. 

Causes

Fibrosing mediastinitis occurs from fibrous infiltration of the hilar regions, which are the spaces between the upper and lower lobes of each lung. It also occurs due to the mediastinum (middle part of the chest between the lungs) narrowing or obstructing the oesophagus, bronchi, or the superior vena cava (the major blood vessel that returns blood from the upper part of the body to the heart).⁴  Moreover, a huge mass forming could block important vessels, reducing oxygen exchange.⁵  

Symptoms

A blockage of the structures mentioned earlier is what causes the symptoms of fibrosing mediastinitis. The most prevalent symptom is repeated haemoptysis, while the most serious complications are airway and vascular occlusion.⁵  

Individuals suffering from fibrosing mediastinitis may exhibit breathing difficulties, exhaustion or decreased energy, bloody or non-bloody coughing, persistent chest discomfort, frequent pneumonia or pulmonary infections, swallowing difficulties, or facial and arm oedema.⁴ 

When a central airway is blocked, the most typical symptoms are coughs and breathing difficulties. If the pulmonary veins are affected, people may experience shortness of breath, coughing up blood, and in some cases,  ly a build-up of fluid in the area between the lung and the chest wall.⁴  

Support and management: steroid treatment

Tamoxifen, immunosuppressive medications, and steroids have reportedly been utilised to treat fibrosing mediastinitis and other similar conditions.⁶ 

It is claimed that a 75-year-old woman with fibrosing mediastinitis and sclerosing cervicitis, which resulted in a restriction of the left common carotid artery, was effectively treated with steroids.⁶  20 mg/d of prednisolone was administered to this patient following surgery due to the narrowing of the left common carotid artery caused by the mediastinal lesion.⁶ Three months later, a CT scan revealed a noticeable decrease in the lesion size and nearly full healing of the left common carotid artery.⁶ 

Another type of steroid treatment is tamoxifen. The first study that focused on the efficacy of tamoxifen was conducted by Clark and colleagues. They treated two patients with retroperitoneal fibrosis with this medication and saw a significant improvement in their obstructive ureter and small-bowel blockage.⁷  

Two patients with fibrosing mediastinitis who received steroid treatment were described by Bays and coworkers. One patient responded well to steroid-only therapy, while the other patient's symptoms didn't improve until they also took tamoxifen.⁸  

Endovascular interventions

The purpose of endovascular treatments is to relieve pulmonary artery blockage. A study was conducted where 59 stents were inserted into 47 different pulmonary vessels and the investigation revealed a decrease in pulmonary symptoms, and only one patient experienced pulmonary artery restenosis.⁹  However, the fragile pulmonary vessel increases the likelihood of adverse effects.     

Non-invasive ventilation 

In individuals who are breathing on their own, continuous positive airway pressure (CPAP) is a form of positive airway pressure in which airflow is pushed into the airways to keep a steady pressure to keep the airways open.¹⁰  A variety of factors can lead to airway collapse, and in many of these cases, CPAP is used to help keep it open. Therefore, CPAP can be an efficient method of managing breathing difficulties in fibrosing mediastinitis.   

Bilevel positive airway pressure (BPAP) is a breathing support device that helps people who can breathe on their own but aren’t getting enough oxygen or are struggling to remove carbon dioxide.¹¹  The nose, mouth, and windpipe (trachea) are part of the airway. BPAP pumps air into these areas at a greater pressure.¹¹ This increased pressure can facilitate the flow of air into and out of the lungs by keeping the airway open.¹¹  

Mechanical ventilation 

When a patient is unable to breathe on their own, mechanical ventilation is a form of therapy that can assist them in breathing. Although this treatment cannot cure a disease on its own, it can stabilise fibrosing mediastinitis patients while the body heals from other medical procedures and medicines.¹² 

Modern mechanical ventilation uses positive pressure is the method used to push air into the lungs.¹² There are two types:  invasive and noninvasive.. In invasive ventilation, a breathing tube is placed into the airway (either through the mouth or a small hole in the neck) which is then connected to a ventilator.¹² Noninvasive ventilation, on the other hand, uses a ventilator and a face mask, and air is pumped into the lungs by the ventilator, without needing a tube.¹² 

Physiotherapy

Respiratory physiotherapy is used to clear the patient’s airways for them to resume exercise and physical activity. This treatment can be used for several purposes, including airway clearing methods, early mobility, and pulmonary (lung) rehabilitation.¹³  Subsequently, this can lead to enhanced physical fitness, fewer breathing problems, and increased sputum (phlegm) clearing. 

The airways are lined with small hair-like structures called cilia, which keep the airways clean and clear. The typical function of the cilia in the airways is to keep them clean and open.¹³ The mucus that continuously flows across the surface of the airways and into the upper respiratory tract is caused by the cilia-covered tissue that surrounds the airways.¹³ A ciliary action moves mucus in the direction of the trachea (windpipe), where extra secretions can be swallowed. 

Summary

Overall, fibrosing mediastinitis is a rare condition that does not have a cure for patients. Despite this, there are several ways of managing this illness, from steroid therapies to respiratory management. This article has highlighted some case studies with different types of therapies used, suggesting that there is promise for several interventions in managing this condition. However, some treatment methods may not be successful for every patient. Therefore, more research and investigation are required into the different treatment methods and their efficacy.