Disorders of the Respiratory System and Critical Care Illness
A 52-year-old woman from Indiana presents with worsening dyspnea on exertion and a cough for a year. She denies dyspnea at rest. The cough is predominantly dry, but occasionally productive of a gritty mucus. Her past medical history is positive for hypertension and hypothyroidism. She takes benazepril and levothyroxine. She has primarily worked as a landscaper throughout her adult life. On physical examination, she appears in no distress. Her oxygen saturation is 95% on room air. Chest is clear to auscultation. Cardiovascular examination is unremarkable. She has no peripheral edema. The chest radiograph shows an old granuloma in the right lung and mediastinal calcifications. A CT scan is performed, which confirms the healed granuloma. Extensive mediastinal calcification is seen. The calcifications encase the superior vena cava and the right mainstem bronchus. An interferon-γ assay is negative. Which of the following statements regarding the patient’s condition is true?
ExplanationChronic fibrosing mediastinitis most commonly occurs after granulomatous inflammation in the lymph nodes in the mediastinum which leads to an exuberant calcification response. Over time, the inflammation can cause signifcant disruption to the vital structures that course through the mediastinum and lead to the clinical symptoms of fibrosing mediastinitis. The most common causes of fibrosing mediastinitis are histoplasmosis and tuberculosis. Other causes include sarcoidosis, silicosis, or other fungal diseases. Symptoms are related to compression of mediastinal structures including the superior vena cava, pulmonary arteries or veins, or large airway compression. The phrenic or recurrent laryngeal nerves may also become paralyzed. The most common symptom is dyspnea on exertion. Patients may also develop a chronic cough with lithophytic or hemoptysis due to erosion of the calcific lymph nodes into the airways. Patients may describe lithophytic as gritty or sandy sputum. Hemoptysis can be a large volume and may necessitate surgical intervention for control. However, other than antituberculous therapy for tuberculous mediastinitis, no medical or surgical therapy has any effectiveness on the treatment of fibrosing mediastinitis. This patient most likely has histoplasmosis as the cause of her disease because histoplasmosis is endemic in Indiana. Because the fibrosing mediastinitis is a sequela of an old infection, the urine Histoplasma antigen test would not yield a positive result.