E. Hemoptysis

Introduction

Hemoptysis, the act of coughing up blood, is an important symptom since it frequently reflects serious underlying lung disease. Because many of the lung conditions that are heralded by hemoptysis are treatable, the symptom requires systematic and thorough evaluation to discover its etiology. A possible exception is mild hemoptysis occurring in a patient with chronic bronchitis during an acute exacerbation. Hemoptysis in this situation is common, usually mild, and self-limited. Therefore, it may be observed without further work-up. However, if the hemoptysis is substantial, persistent, or recurrent then further evaluation is indicated, particularly since patients with chronic bronchitis related to smoking are at high risk for lung cancer.

Sources

The first step in the evaluation of hemoptysis is to decide if it is really hemoptysis—that is, is the blood coming from the bronchial tree or lungs or from some other site? In most cases, history will suggest that blood is actually being coughed up from the airways or lungs, but it may be difficult at times to distinguish blood being coughed up from the respiratory system from blood coming from two other sites: bleeding in the upper respiratory tract, in the nasopharynx or sinuses, or blood originating in the gastrointestinal tract that was regurgitated or vomited. A history of frequent nosebleeds, hoarseness, or some other change in the voice or history of mouth lesions might suggest bleeding from the upper respiratory tract. If bleeding is not clearly from the lungs then a thorough examination of the upper respiratory system is indicated. If the source remains equivocal, i.e., no abnormality in the upper respiratory tract is found on initial examination and no source is found after further pulmonary work-up as described below, then an examination by an otolaryngology specialist may be warranted. Hematemesis occasionally may be difficult to distinguish from hemoptysis; moreover, blood from a respiratory source may be swallowed and may present as coffee-ground emesis. Gastrointestinal symptoms suggest an upper GI work-up when the bleeding source is unclear.

The second question to be asked is whether the bleeding is massive (or life-threatening), which if present changes the approach to management as well as affecting the differential diagnosis. Massive or life-threatening hemoptysis has usually been defined by the rate of bleeding, defined as greater than 200 ml per day by various authors. The bleeding rate is critical since the problem with massive hemoptysis is not exsanguination but asphyxiation from blood that floods alveoli or clots that functionally obstruct airways. Thus, any amount of bleeding at a high rate, even over a short period of time, should be managed as being potentially life-threatening. The approach to massive hemoptysis is described in more detail below.

Differential Diagnosis

The differential diagnosis of hemoptysis is shown in Table 10. The most common causes are bronchitis, lung cancer, pneumonia, lung abscess, tuberculosis, bronchiectasis, and pulmonary thromboembolism. The prevalence of these disorders in causing hemoptysis appears to be changing and varies considerably in different series. In North America, tuberculosis (both active and inactive) and bronchiectasis appear to be decreasing as a cause of hemoptysis whereas they are still extremely frequent causes of hemoptysis in many other parts of the world. In many (but not all) series, a significant proportion of cases remain undiagnosed despite extensive work-up.

Conditions that cause massive hemoptysis are generally inflammatory disorders which erode into the bronchial circulation. Because the bronchial circulation is under systemic vascular pressure, the bleeding is likely to be more severe than if the source of bleeding were the pulmonary circulation. Thus, causes of massive hemoptysis consist mainly of suppurative or chronic infections or conditions complicated by infection (lung abscess, tuberculosis, bronchiectasis, or cystic fibrosis), but also include lung cancer.

Evaluation of Hemoptysis

The initial evaluation in all patients consists of a careful history, physical examination, and upright postero-anterior and lateral chest x-rays. The history should elicit and detail any acute or chronic pulmonary symptoms, including cough, sputum production, shortness of breath or wheezing, and any previous history of lung disease. Systemic symptoms such as fever, sweats, weight loss, and malaise may reflect ongoing inflammation or reflect a catabolic process related to cancer or chronic infection. The history should uncover symptoms associated with the specific causes in the differential diagnosis including symptoms of heart disease (especially mitral stenosis), vasculitis, and with particular attention given to pulmonary thromboembolism. In considering pulmonary thromboembolism, in addition to the acute onset of pulmonary symptoms and any leg symptoms reflecting possible deep venous thrombosis, the most important part of the history focuses on asking about possible risk factors for deep venous thrombosis.

Physical examination includes auscultation, listening for generalized wheezing (COPD/asthma), localized wheezing (local bronchial obstruction), or diffuse or localized crackles or rhonchi which may reflect infectious or inflammatory processes including lung abscess, pneumonia, and bronchiectasis. A careful cardiovascular examination should be done, particularly looking for congestive heart failure, evidence of mitral stenosis and signs of deep venous thrombosis. It is important to recognize that signs of deep venous thrombosis are lacking in at least half of the cases in which deep venous thrombosis is eventually proven. A negative result of the examination, therefore, clearly does not rule out deep venous thrombosis or the possibility of pulmonary thromboembolism.

A complete blood count and coagulation studies should be ordered. A posteroanterior and lateral chest x-ray should be routinely obtained. The chest x-ray may be very helpful in suggesting a source of the hemoptysis, such as pulmonary inflammatory disease or cancer. If the chest x-ray is abnormal, it will often suggest subsequent steps in the work-up. Sputum cytology on expectorated sputum should be obtained in any patient at significant risk for lung cancer based on epidemiologic considerations, whether or not the chest x-ray is suspicious for cancer. This includes all patients with chronic obstructive pulmonary disease.

If the chest x-ray is negative or unrevealing it does not rule out important disease as a cause of hemoptysis. Therefore, one must make a clinical decision about how much further to go in the evaluation. This decision should be individualized according to each clinical situation and the availability of diagnostic facilities and subspecialty consultation. Generally, computed tomography (CT) of the chest is the preferred next study since it is noninvasive, can detect small cancers in the bronchial tree and lung parenchyma, and can diagnose bronchiectasis. Chest CT might also provide information useful to the bronchcoscopist if bronchoscopy becomes a consideration. Three forms of chest CT are available that might be helpful in diagnosing the cause of hemoptysis: chest CT with contrast; high resolution chest CT; and spiral CT of the chest. Each has its advantages for diagnosing some of the conditions in the differential diagnosis. Consultation may be indicated to determine which form of chest CT should be ordered for evaluation of a given patient.

Specialized Work-Up

Fiberoptic bronchoscopy is generally the next study to be considered. The decision to perform bronchoscopy should be made in consultation with a pulmonologist. Fiberoptic bronchoscopy may identify an endobronchial lesion, most often lung cancer, as the cause for hemoptysis and can help localize the lobe or segment from which the blood is coming. The combination of fiberoptic bronchoscopy and chest CT has been shown to give a higher yield of specific diagnoses than either test alone.

Fiberoptic bronchoscopy is indicated in certain categories of patients: those in whom the diagnosis is not evident from history, physical examination, chest x-ray, or chest CT; those with significant bleeding (greater than 30 ml per day) or in whom hemoptysis persists for longer than one week; and those who have systemic symptoms suggesting cancer or who are at particularly high risk for lung cancer, especially cigarette smokers over the age of forty. If none of these conditions is present, then the chance of finding lung cancer on bronchoscopy is very low and a decision to observe the patient should be considered.

If suspicion of pulmonary embolus is moderate, particularly if risk factors exist for deep venous thrombosis and pulmonary thromboembolism, then a ventilation/perfusion lung scan should be obtained.

Therapy

The therapy of hemoptysis consists of that treatment appropriate for the underlying disease process, for example, antibiotic therapy for infectious etiologies. Otherwise, the treatment is nonspecific. The exception to this is when massive hemoptysis is present.

Management of Massive Hemoptysis

When the rate of bleeding qualifies as massive hemoptysis (a rate of greater than 200 ml per day) the situation should be considered to be a medical emergency requiring referral for immediate diagnostic and therapeutic steps. The treatment of massive hemoptysis includes consideration of either surgical removal of the bleeding site or bronchial angiography with embolization of the bleeding site when feasible. Although there is some debate regarding the role of bronchial embolization, with some authors suggesting it be performed in all cases, the standard management for life-threatening bleeding due to localized disease in a patient with good pulmonary reserve is usually surgical resection. If emergent surgery is being considered, the diagnostic goal consists of localizing the bleeding site, first, as to which lung is bleeding and then, if possible, as to which lobe or segment contains the bleeding source. Bronchial arterial embolization is usually indicated in patients with nonlocalized disease and/or limited breathing reserve to preserve pulmonary parenchyma and function. Localization can also be helpful when bronchial embolization is being considered because it permits selective bronchial angiography to be undertaken, which markedly shortens the angiographic procedure. Bronchoscopy, either fiberoptic or rigid bronchoscopy, should be performed as soon as possible in an attempt to localize the site of bleeding within the lung. Localization of the bleeding source is much more successful if some degree of active bleeding is still occurring.

If the lung from which the bleeding is occurring is suspected (e.g., based on the chest x-ray or the patient’s subjective impression), the patient should be positioned with the affected lung placed in a dependent position to prevent drainage of blood into the contralateral lung. The patient may be lightly sedated or tranquilized to diminish cough, but the state of consciousness should not be impaired such that the patient is unable to cough and maintain a clear airway. Once it is decided that surgery is indicated, then control of the airways should be obtained. Ideally, a double lumen tube should be inserted. However, if the required experience is not available, a standard endotracheal tube should be placed. If the bleeding is suspected to be from the left lung then the tube can be placed in the right mainstem bronchus and the right lung ventilated while the patient is prepared for emergent surgery.

When to Refer

Indications for referral to subspecialists for evaluation of hemoptysis include the following:

Consideration of CT scan (to help determine type)
Consideration of bronchoscopy (see indications above)
Presence of massive or life-threatening hemoptysis
Persistent or recurrent undiagnosed hemoptysis.

Medicolegal Concerns

The biggest concern for liability on the part of the physician in evaluation of hemoptysis is failure to diagnose lung cancer. A less frequently occurring situation is the failure to diagnose pulmonary thromboembolism. Because these clinical situations have differing concerns they will be treated separately.

Eventual diagnosis of lung cancer when the patient presented earlier with hemoptysis is a cause for malpractice litigation. Two considerations allow appropriate evaluation while protecting the physician from risk. The first is to recognize the patient at risk for lung cancer from demographic data and smoking history, and include a sputum cytology (and, if warranted clinically, fiberoptic bronchoscopy) in the initial work-up. The second is to clearly record the advice given to the patient with suspected bronchitis and mild hemoptysis that stops spontaneously to return for further evaluation should hemoptysis recur.

Hemoptysis is an infrequent but important symptom in pulmonary thromboembolism. The liability here is failure to consider pulmonary thromboembolism and to order appropriate tests when hemoptysis is associated with other acute symptoms (including shortness of breath and/or pleuritic chest pain) in a patient at risk for deep venous thrombosis.

Summary

There are several important steps in evaluation of the patient with hemoptysis. First, it should be determined whether the bleeding represents true hemoptysis or whether the source of bleeding is in the upper airway or in the gastrointestinal tract. Second, the significance of the bleeding should be evaluated, specifically ascertaining whether life-threatening bleeding is present. Third, a differential diagnosis based on the initial history should be developed; this will help focus subsequent questioning, physical examination, and laboratory studies on likely sources of bleeding for the specific clinical situation. A chest x-ray should be obtained. If history, physical examination, and a chest x-ray do not reveal the source of bleeding, then a chest CT should be considered. Patients who are candidates for bronchoscopy include those with bleeding of more than 30 ml per day, hemoptysis which has been persistent for one week, and patients at high risk for lung cancer, particularly cigarette smokers older than forty years of age. Massive or life-threatening hemoptysis (bleeding at a rate of greater than 200 ml per day) constitutes an emergency with the major diagnostic objective being localizing the source of the bleeding so that emergent surgery to remove the bleeding site can be carried out. Bronchial arteriography and embolization should be considered in patients with poor pulmonary reserve due to pre-existing lung disease.

References

Goldman JM. Hemoptysis: Emergency assessment and management. Emerg Med Clin N Amer 1989; 7:325-339. This review addresses the evaluation and management of hemoptysis from the point of view of the emergency physician. Nonetheless, it is relatively thorough and useful to the primary physician. Massive hemoptysis is particularly, but not exclusively, emphasized.

Marshall TJ, Flower CDR, Jackson JE. The role of radiology in the investigation and management of patients with haemoptysis. Clinical Radiol 1996; 51:391-400. A thorough review of the literature on this subject, especially focusing on the role of computed tomography of the chest.

Santiago S, Tobias J, Williams AJ. A reappraisal of the causes of hemoptysis. Arch Intern Med 1991; 151:2449-2451.

Johnston H, Reisz G. Changing spectrum of hemoptysis: Underlying causes in 148 patients undergoing diagnostic flexible fiberoptic bronchoscopy. Arch Intern Med 1989; 149:1666-1668.

Hirschberg B, Biran I, Glazer M, Kramer MR. Hemoptysis: Etiology, evaluation, and outcome in a tertiary referral hospital. Chest 1997; 112:440-444. These three series are the most recent to describe the prevalence of the various causes of hemoptysis. All three reflect prevalence in a subspecialty practice; no recent series exists (to our knowledge) of hemoptysis causes presenting to a primary care physician. The two studies from the U.S. (Santiago and Johnston & Reisz) suggest that the incidence of hemoptysis secondary to tuberculosis and bronchiectasis has decreased in contrast to older series. Bronchitis and bronchogenic carcinoma were the most frequent causes in both series. The study by Hirschberg et. al. from Israel found that bronchiectasis, lung cancer, bronchitis, and pneumonia were the most common causes of hemoptysis.

Cahill BC, Ingbar DH. Massive hemoptysis: Assessment and management. Clin Chest Med 1994; 15:147-167. An excellent review of this subject. A step-by-step approach to evaluation and management is described with recommendations by the authors on controversial management issues.

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