Mediastinal fibrosis

Mediastinal fibrosis (idiopathic mediastinal fibrosis) is of unknown etiology, based on sclerosing mediastinal connective tissue. It is a rare disease, usually affecting middle-aged and older adults, but can occur at any time. Disabilities of both sexes are about the same.

Origin
The very existence of idiopathic mediastinal fibrosis has been controversial in the past. It was thought to be a secondary condition, e.g., Tuberculosis mediastinal lymphadenitis, Syphilis changes, after irradiation, or similar conditions. Another assumption was that it was a rare complication of Histiocytosis. It is now thought to be an organ-specific manifestation IgG4-associated disease, at least in some cases. The disease is often associated with other chronic idiopathic sclerosing diseases.

Clinical manifestations
A typical clinical manifestation is the sudden onset of symptoms in full health. Manifestations are initially mild and may be intermittent. Swelling of the upper limbs and face usually occurs first, especially in the morning. Later manifestations of upper vena cava syndrome. A conspicuous manifestation is an ashen face, neck and arms of the patient, conjunctival dysfunction, dizziness, Tinnitus, Headache, Epistaxis, Hemoptysis, feelings of pressure in the head and intolerance to crowded areas. Manifestations are exacerbated by coughing, bending, exertion or exercise. After 6-9 months, there is usually a significant improvement, but there is no complete remission of symptoms. The development of the collateral vascular bed is probably at least partly responsible for this improvement. The disease is not painful in itself.

Other possible lesions of the mediastinal organs have also been described, which of course differ in the spectrum of manifestations: compression aorta, pulmonary artery obstruction with development cor pulmonale, pulmonary vein stenosis, pericarditis, obstruction Trachea with manifestations resembling asthma, stenosis esophagus and obstruction coronary arteries.

Diagnosis
Biochemical and serological examination is usually without obvious abnormalities. Bronchoscopy and esophagoscopic examination do not usually detect pathology. No pathology may be seen on the chest X-ray, but enlargement Mediastinum may be evident. The actual diagnosis is usually based on the presence of upper vena cava syndrome and the absence of a clinically and radiologically demonstrable cause.

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 * IgG4 asocciated disease