Hemoptysis

Henri G. Colt

Hemoptysis (i.e., coughing up blood) is a frightening event for both healthcare provider and patient and may occur in a variety of clinical conditions. The amount and quality can range from blood-streaked sputum to several cups of blood or even massive bleeding leading to exsanguination. Death is rare, but may occur as a consequence of asphyxiation and respiratory arrest associated with flooding of the tracheobronchial tree. Massive hemoptysis is a life-threatening medical emergency that may not be controllable even by endotracheal intubation and mechanical ventilation.

The incidence of hemoptysis reflects the type of population studied (e.g., surgical vs medical or cancer center vs tuberculosis clinic). In the United States, the most common causes are chronic bronchitis, bronchiectasis, and bronchogenic carcinoma, followed by tuberculosis, fungal infections (especially aspergillosis or aspergilloma), bacterial pneumonia and abscess, and pulmonary infarction. Less common causes of hemoptysis include mitral stenosis, Goodpasture syndrome, endobronchial foreign bodies, bronchial adenoma, pulmonary arteriovenous (AV) fistulas, Behçet disease, lung parasites (ascariasis, paragonimiasis, and schistosomiasis), Wegener granulomatosis, drugs (cocaine, anticoagulants, penicillamine), cystic fibrosis, lymphangioleiomyomatosis, pulmonary artery injury from a balloon-tipped catheter, coagulopathies, and even bioterrorism (pneumonic plague, tularemia, and tricothecene mycotoxin). Hemoptysis also may be caused by airway inflammation, granulation tissue overgrowth, and erosion of tracheobronchial mucosa from indwelling metal, hybrid, and silicone airway stents. When a diagnosis is not found, hemoptysis is said to be cryptogenic. This should be a diagnosis of exclusion, although it has been reported in up to 40% of cases, usually in tobacco smokers.

The approach to the diagnosis and initial management focuses on the following questions:

1. What is the origin of the bleeding (lungs, the airways, nasopharynx, or digestive tract)?

2. Can the bleeding be stopped?

3. Will the bleeding recur at some time in the future?

4. Does the patient have a systemic disease that predisposes to bleeding?

5. Is emergency intervention needed? If so, what kind of surveillance should be instituted, and what might be done to prevent recurrent bleeding?

The anatomic source of bleeding depends on the specific pathologic process. However, bronchial arteries and collaterals from axillary, intercostal, diaphragmatic, and other systemic arteries of the thorax are the source of bleeding in most cases. Inflammation associated with infection and carcinoma can cause reactive hypervascularity of bronchial arteries and stimulation of collaterals. Localized inflammation can result in bleeding by erosion of these hypervascular networks of vessels. Chronic inflammation may encourage enlargement of the bronchial arteries as a result of enhanced abnormal communication with pulmonary arterioles. Furthermore, angiogenic growth factors can be released that promote neovascularization and recruitment of collateral circulation from systemic blood vessels. Pulmonary arteries, capillaries, and veins are the source of hemoptysis in fewer than 10% of cases.

Hemoptysis associated with chronic bronchitis accounts for more than 50% of hemoptysis cases in the United States and arises from superficial vessels in the bronchial mucosa. Hemoptysis associated with chronic fibrocavitary disorders such as tuberculosis, is caused by rupture or erosion of enlarged bronchial arteries and bronchopulmonary anastomoses. Pulmonary artery aneurysms and vessel rupture secondary to wall invasion can also occur. In mitral stenosis, the primary sites of bleeding are bronchial veins with blood supplied from both bronchial arteries and reversed blood flow from pulmonary veins.

Recent world events warrant special emphasis on potential bioterrorist causes of hemoptysis. Pneumonic plague is caused by Yersinia pestis, a Gram-negative bacillus that can be weaponized and spread by aerosolized droplets, causing rapidly progressive pneumonia, chest pain, and hemoptysis. Treatment includes oral doxycycline or ciprofloxacin. Tularemia, caused by the aerobic Gram-negative coccobacillus Francisella tularensis, has also been weaponized. Its aerosolization causes influenzae-like symptoms with rapidly progressive pneumonia and hemoptysis. The treatment of choice is intravenous gentamicin. Finally, tricothecene mycotoxin, also known as “yellow rain” in its aerosolized form, causes sore throat, skin necrosis, and hemoptysis. An oily residue on the facial skin of victims of biologic attacks might lead one to suspect the diagnosis. Treatment is, for the most part, supportive.

All instances of hemoptysis require careful evaluation to determine the cause and site of bleeding. The history is invaluable and establishes the duration and extent of bleeding, prior episodes, and the presence of known cardiopulmonary or other diseases. Hemoptysis must be differentiated from hematemesis and nasopharyngeal bleeding. The physical examination provides specific clues to the diagnosis (e.g., oronasopharyngeal bleeding site, microtelangiectasia, pulmonary or cardiac findings). The chest radiograph may suggest the cause and location of the hemoptysis in up to 50% of cases. A “negative” radiograph is not reassuring: one study suggested that the radiographs of 25% of patients with hemoptysis due to lung cancer provided no clues to this diagnosis. The characteristic radiographic finding of blood in the air spaces is a confluent or patchy alveolar filling pattern that becomes reticular over days and clears in 3 to 10 days. However, the pattern may represent blood aspirated from another bleeding site elsewhere in the lungs, making precise localization of a bleeding site challenging. Computed tomography (CT) scans, including multi-detector CT with angiography (MDCTA) (high resolution angiographic studies performed with a single breath hold to reduce scanning time and respiratory motion artifacts) can identify underlying disease, assist in the diagnosis and mapping of bronchiectasis, usually precluding surgical resection if bronchiectasis is bilateral or diffuse, as well as direct surgical interventions in case focal pulmonary or vascular abnormalities are identified. More importantly, CT may detect vascular lesions such as aneurysms and arteriovenous malformations, and has a high accuracy predicting involvement of nonbronchial systemic arteries in patients with massive bleeding. Information from MDCTA is essential for planning arterial embolization, providing more accurate information about bronchial and nonbronchial systemic arteries than conventional angiography.

Other relevant laboratory studies include a complete blood count; smear, culture, and cytologic examination of the sputum; and, when appropriate, arterial blood gas analysis, as well as ventilation/perfusion ()/() lung scans if pulmonary emboli are suspected. Perfusion scans are not, however useful in localizing a bleeding site. Several studies have found an elevated single-breath diffusion capacity (DLCO) in patients with intrapulmonary bleeding, although it does not appear that DLCO plays a useful role in the diagnosis or care of most patients (except, possibly, in patients with suspected Goodpasture syndrome).

Flexible bronchoscopy is indicated for a patient with hemoptysis of uncertain cause. Bronchogenic carcinoma has been detected bronchoscopically in at least 2% to 13% of patients with hemoptysis and a normal chest radiograph. Foreign bodies, bronchial adenoma, and other causes of bleeding are also readily identified. Furthermore, the site of the bleeding can usually be determined if a careful, systematic bronchoscopic inspection is performed, or if patients have previously undergone biopsy of endobronchial abnormalities.

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