KEY POINTS
Ensure proper oxygenation and secure the patient’s airway if necessary.
Correct coagulation abnormalities.
Localize bleeding with bronchoscopy (unstable patient) or CT scan (stable patient) and position the bleeding site in a dependent position.
In unstable, hypoxemic patients consider urgent bronchoscopy for suctioning, endobronchial hemostatic therapy and balloon tamponade.
Interventional radiology-guided bronchial artery embolization is effective and should be performed after initial stabilization.
Surgery is required in rare circumstances.
INTRODUCTION
Hemoptysis, or the expectoration of blood, can result from a wide variety of illnesses (Table 57-1). The prevalence of these different etiologies depends on the characteristics of the specific patient population studied. For example, tuberculosis is the most common cause of hemoptysis in underdeveloped countries while bronchiectasis is the most common cause in the industrialized world.1-2
Causes of Massive Hemoptysis and Pulmonary Hemorrhage
Localized Bleeding |
Infections |
Mycobacteria (TB most common) |
Necrotizing bacterial pneumonia (particularly Klebsiella and Staphylococcus aureus) |
Lung abscess |
Mycetoma (Aspergillus most common) |
Bronchiectasis (eg, cystic fibrosis or immune deficiencies) |
Parasites (Hydatid cyst, paragonimiasis) |
Leptospirosis |
Tumors |
Bronchogenic carcinoma (ie, squamous cell) |
Pulmonary metastatic disease |
Bronchial adenoma |
Sarcoma |
Pulmonary vascular problems |
Pulmonary arteriovenous malformations (eg, Rendu-Osler-Weber syndrome) |
Pulmonary embolus with infarction |
Pulmonary aneurysm (eg, Behçet syndrome) |
Pulmonary artery catheterization with pulmonary arterial rupture |
Mitral stenosis |
Coagulopathy (usually requires coexisting mucosal disruption) |
Thrombocytopenia or platelet dysfunction (eg, von Willebrand disease, uremia) |
Hemophilia A or B |
Prolonged coagulation tests (due to coagulation factor production or consumption defect) |
Trauma |
Miscellaneous |
Lymphangioleiomyomatosis |
Catamenial (endometriosis) |
Cryptogenic |
Broncholithiasis |
Sarcoidosis (usually from cavitary lesions with mycetoma) |
Diffuse Bleeding |
Capillaritis (seen on biopsy) |
Drug- and chemical-induced (propylthiouracil, phenytoin, retinoic acid) |
Connective tissue diseases (ie, systemic lupus erythematosus, rheumatoid arthritis, mixed connective tissue disease, systemic sclerosis, antiphospholipid antibody syndrome, polymyositis) |
Systemic vasculitides (Behçet, cryoglobulinemia, IgA nephropathy, microscopic polyangiitis, granulomatous vasculitis, pauci-immune, Henoch-Schonlein purpura) |
Bland hemorrhage (seen on biopsy) |
Connective tissue diseases (ie, systemic lupus erythematosus, Goodpasture syndrome) |
Drugs (anticoagulant and antiplatelet therapy-glycoprotein IIa/IIIb inhibitors) |
Other (pulmonary veno-occlusive disease, mitral stenosis, idiopathic pulmonary hemosiderosis) |
Diffuse alveolar damage with bleeding (seen only on biopsy) |
Infection (any infection that can cause ARDS) |
Drugs (amiodarone, crack cocaine, nitrofurantoin, penicillamine, sirolimus, most cytotoxic drugs) |
Connective tissue diseases (systemic lupus erythematosus, rheumatoid arthritis, polymyositis) |
Other (pulmonary capillary hemangiomatosis, pulmonary infarct, ARDS of any cause) |
Massive hemoptysis has been variably defined as production of more than 300 to 600 mL of blood in 12 to 24 hours, depending on the study. However, estimating the amount of hemoptysis is unreliable and has very little clinical utility. Accordingly, a “magnitude-of-effect” is the preferred clinical approach3 as it uses the clinical consequences of hemoptysis such as airway obstruction, hypoxemia, hemodynamic instability and underlying cardiopulmonary abnormalities to guide treatment and triage decisions. As an example of this principle, patients with diffuse alveolar hemorrhage can present with life-threatening hypoxemia and diffuse parenchymal infiltrates, meeting all the criteria for the acute respiratory distress syndrome (ARDS), yet have little or no hemoptysis.
The conditions listed in Table 57-1 most often associated with massive hemoptysis are: bronchiectasis, mycetoma, tuberculosis, bronchogenic carcinoma, necrotizing pneumonia, and vascular-bronchial fistulas. The relative incidence of these conditions depends on the origin of the case series examined. For example, when examining recent case series from China, Singapore, France, and Austria the relative prevalence of each condition differed: bronchiectasis (23%, 66%, 40%, 9%), tuberculosis (55%, 10%, 14%, 23%), bronchogenic carcinoma (6%, 7%, 17%, 35%), respectively.1,3-5
Although hemoptysis is classified as massive in only 5% of patients this condition is associated with a mortality of 9% to 38%,4,6 with death attributed to asphyxiation more commonly than to exsanguination. Massive hemoptysis originates from the bronchial circulation in 90% of patients.7 Nonbronchial systemic vessels (5%) and pulmonary vessels (5%) are rarely the primary source of bleeding (although they make some contribution to the bleeding in up to a third of cases).7 Bleeding from vessels other than bronchial arteries most commonly occurs as a result of inflammatory or infectious lung diseases causing anastomoses and collateral vessels to develop at the site of injury.6