Hemoptysis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Lung has two main vascular systems that include pulmonary circulation and bronchial circulation. There are multiple anastomoses between pulmonary and bronchial arterieswhich create physiologic right to left shunts. Blood in the hemoptysis is mostly originated from the Lung. However, it could be from the gastrointestinal system as well. Primary origin of the blood comes from bronchial arteries. Hemoptysis is an important symptom that has different etiologies and pathogenesis mechanisms. Hemoptysis may happen following infarction and ischemia of pulmonaryparenchyma and vascular engorgement with erosion. Hemoptysis may be classified according to severity into 3 groups of mild, moderate, and massive bleeding. Life-threatening causes of hemoptysis include pulmonary embolism. Common causes of hemoptysis include bronchiectasis, acute respiratory tract infections, chronic obstructive pulmonary disease, bronchitis, pneumonia, lung cancer, tuberculosis, cystic fibrosis, and idiopathic.

During 129-199 A.D., Galen identified the bronchial arteries as internal vessels of the lung. Leonardo da Vinci illustrated lung circulation. In the 1960s, angiography of the thoracic aorta and bronchial tree was done. In 1963, angiography of bronchial arteries was described by Viramonte. In 1974, Remy introduced bronchial artery embolization(BAE) as a successful, effective, non-invasive procedure to treat hemoptysis.

Hemoptysis may be classified into several subtypes based on duration of symptoms, severity and origin of the bleeding. Based on the duration of symptoms, hemoptysis may be classified as either acute or chronic. Hemoptysis may be classified according to severity into 3 groups of mild, moderate, and massive bleeding. Based on the origin of bleeding, hemoptysis may be classified into two groups of pulmonary vs extrapulmonary bleeding.

Lung has two main vascular systems that include pulmonary circulation and bronchial circulation. There are multiple anastomoses between pulmonary and bronchial arterieswhich create physiologic right to left shunts. Blood in the hemoptysis is mostly originated from the Lung. However, it could be from the gastrointestinal system as well. Primary origin of the blood comes from bronchial arteries. However, other sources of bleeding might be pulmonary vessels, aorta, intercostal, coronary, thoracic, and phrenic arteries. Hemoptysis is an important symptom that has different etiologies and pathogenesis mechanisms. Hemoptysis may happen following infarction and ischemia of pulmonaryparenchyma as seen in pulmonary embolism, vasculitis, and infections. Another mechanism of hemoptysis is vascular engorgement with erosion as seen in bronchitis, bronchiectasis, and toxic exposure to cigarette and other irritants. In some cases underlying cause can not be identified and they are considered as idiopathic. However, they might present with massive hemoptysis. There are multiple conditions that are associated with hemoptysis which include granulomatosis with polyangiitis, sarcoidosis, immunodeficiency, and indoor ice hockey play.

Life-threatening causes of hemoptysis include pulmonary embolism. Common causes of hemoptysis include bronchiectasis, acute respiratory tract infections, chronic obstructive pulmonary disease, bronchitis, pneumonia, lung cancer, tuberculosis, cystic fibrosis, and idiopathic. Less common causes of hemoptysis include ruptured aneurysms, lung abscess, aspergilloma, idiopathic pulmonary fibrosis, behçet’s disease, aortobronchial fistula, pulmonary endometriosis, goodpasture syndrome, and foreign body aspiration.

The incidence of hemoptysis is approximately 100 per 100,000 individuals in the outpatient setting. There is no enough data on prevalence of hemoptysis. The mortality rate of patients with massive hemoptysis is approximately 50-100%, if left untreated. During 1995-2005, in-hospital mortality rate of massive hemoptysis was 0-15%. Patients of all age groups may develop hemoptysis. There is no racial predilection to hemoptysis. Hemoptysis affects men and women equally. Hemoptysis is a symptom that might affect everyone. Underlying causes of hemoptysis might be different in developed countries than in developing countries. In the United States, incidence of tuberculosis in patients with massive hemoptysis is 7%, while in south africa it is 85%.

Common risk factors in the development of hemoptysis include cigarette smoking, chronic obstructive pulmonary disease, anticoagulant medications. Risk factors in the recurrence of hemoptysis include persistent residual mild bleeding following bronchial artery embolization, blood transfusion before the procedure, and aspergilloma. Less common risk factors in the development of hemoptysis include congestive heart failure and mitral regurgitation.

There is insufficient evidence to recommend routine screening for hemoptysis.

Hemoptysis is an important symptom that indicates an underlying pulmonary or extrapulmonary causes. Hemoptysis usually happens following bronchitis as an acute symptomand it resolves spontaneously or with antibiotic therapy within a week. Watchful observation in a patient with hemoptysis and normal chest x-ray is recommended. However, persistent and massive hemoptysis requires further investigations. Asphyxia and airway obstruction are common after massive hemoptysis. Prognosis of hemoptysis is generally excellent. However, massive hemoptysis has a poor prognosis and the mortality rate of patients with hemoptysis is approximately 50-100%, if left untreated.

The initial diagnostic study in a patient with hemoptysis is chest x-ray. If diagnosis is not found on chest x-ray, the next step is to perform high resolution CT scan or bronchoscopy. HRCT is better for diagnosis of bronchiectasis or lung carcinoma. Flexible bronchoscopy is better for diagnosis of mucosal abnormalities such as bronchitis, Dieulafoy disease or kaposi sarcoma.

Patients with hemoptysis may have a positive history of upper respiratory tract infection, gastrointestinal disease, exposure to patients with tuberculosis, bleeding disorders, medications (anticoagulants), and cigarette smoking. Common symptoms of hemoptysis include bloody sputum, chronic cough, shortness of breath, pleuritic chest pain, and wheezing. Less common symptoms of hemoptysis depends on the etiology include weight loss, change in cough, fatigue.

Physical examination of patients with hemoptysis might be normal. However, patients might show different findings depend on underlying causes. Patients with hemoptysis usually appear anxious and depend on the severity of bleeding they might be critically ill. Patients with hemoptysis usually have abnormal vital signs indicating dehydration, other signs of mucosal bleeding, purulent bloody sputum, and abnormal lung exam indicating underlying pulmonary causes.

There are laboratory tests that are helpful for diagnosis the underlying cause of hemoptysis. Sputum must be evaluated for the cytology, gram stain, culture, and acid-fast stain. Arterial blood gases might show hypoxia. Complete blood count (CBC) might show elevated WBC, low platelet, and anemia. Signs of dehydration might be detected in laboratorytests such as BUN, Cr, urinalysis, or electrolytes. Coagulation studies might be abnormal.

There are no electrocardiogram findings associated with hemoptysis. However, electrocardiogram might be abnormal with some of the underlying causes of hemoptysis.

Chest x-ray is the first diagnostic modality that is used in a patient with hemoptysis. Chest x-ray might differentiate underlying causes of hemoptysis. Chest x-ray is usually used to compare with previous or later imagings in order to evaluate the progression and resolution of the underlying cause. However, chest x-ray might be completely normal in patients with hemoptysis.

There are no echocardiography/ultrasound findings associated with hemoptysis. However, echocardiography or ultrasound might be abnormal with some of the underlying causes of hemoptysis.

Chest CT scan and CT angiography may be helpful in the evaluation of a patient with hemoptysis. It is useful for assessing the cause of hemoptysis, localizing the origin of the blood, providing prognostic information by correlating between the extent of lobar involvement on high-resolution CT and the amount of bleeding, and assisting the interventional radiologist prior to treatment. CT scan is not helpful in unstable patients, patients with active bleeding who require bronchoscopy intervention, and patients with bilateral lung abnormalities.

Chest MRI may be helpful in the diagnosis of underlying causes of hemoptysis.

Other imaging findings may be helpful in the diagnosis of underlying causes of hemoptysis.

Rigid bronchoscopy may be helpful in the differentiating underlying etiologies of hemoptysis, localizing the bleeding site, identifying lung cancer, and even treatment of the underlying cause of hemoptysis in case of visible endoluminal lesions. Other diagnostic studies for localizing the site of bleeding in a patient with hemoptysis include angiographyand aortography.

Hemoptysis is a symptom that indicates an underlying pulmonary or extrapulmonary cause. Pharmacologic medical therapy depends on an underlying cause. However, the mainstay of treatment for massive hemoptysis is supportive and surgical therapy.

Massive hemoptysis is a life-threatening condition and requires prompt intensive care. Surgery is indicated in patients with hemoptysis who are resistant to embolization. Interventional techniques are used to stop bleeding which include bronchial arterial embolization, different bronchoscopic strategies such as cold saline lavage, topical vasoconstrictor agents, balloon tamponade, endobronchial stent placement, endobronchial spigot, oxidized regenerated cellulose, N-Butyl cyanoacrylate glue, fibrinogenthrombin, tranexamic acid, laser photocoagulation, argon plasma coagulation, and electrocautery. Surgical techniques that are used for management of hemoptysis include pulmonary resection, lobectomy, and bilobectomy. Surgical techniques are definitely curative, effective for localized lesions. However, surgery has a mortality rate of 10-30%. Currently, bronchial arterial embolization considered as a first line therapy for both new and recurrent hemoptysis.

Effective measures for the primary prevention of hemoptysis include smoking cessation, avoiding air pollutants, and use of physical barriers such as masks and gown.

There are no established measures for the secondary prevention of hemoptysis.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

During 129-199 A.D., Galen identified the bronchial arteries as internal vessels of the lung. Leonardo da Vinci illustrated lung circulation. In the 1960s, angiography of the thoracic aorta and bronchial tree was done. In 1963, angiography of bronchial arteries was described by Viramonte. In 1974, Remy introduced bronchial artery embolization (BAE) as a successful, effective, non-invasive procedure to treat hemoptysis.

• During 129-199 A.D., Galen identified the bronchial arteries as internal vessels of the lung.
• Leonardo da Vinci illustrated lung circulation.
• In the 1960s, angiography of the thoracic aorta and bronchial tree was done.^[1]
• In 1963, angiography of bronchial arteries was described by Viramonte.^[2]

• In 1974, Remy introduced bronchial artery embolization (BAE) as a successful, effective, non-invasive procedure to treat hemoptysis.^[3]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Hemoptysis may be classified into several subtypes based on duration of symptoms, severity and origin of the bleeding. Based on the duration of symptoms, hemoptysis may be classified as either acute or chronic. Hemoptysis may be classified according to severity into 3 groups of mild, moderate, and massive bleeding. Based on the origin of bleeding, hemoptysis may be classified into two groups of pulmonary versus extrapulmonary bleeding.

• Hemoptysis may be classified into several subtypes based on:^[1][2][3]
  • Duration of symptoms
  • Severity
  • Origin of the bleeding
• Based on the severity of bleeding, hemoptysis may be classified as mild, moderate, and massive:^[4][5][6][7]

Category	Amount
Mild bleeding	<30 ml blood in 24 hours
Moderate bleeding	30-200 ml blood in 24 hours
Massive bleeding	200-600 ml blood in 24 hours

• Based on the origin of bleeding, hemoptysis may be classified into two groups:^[8]
  • Pulmonary bleeding (in 90% of patients)
  • Extrapulmonary bleeding

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Lung has two main vascular systems that include pulmonary circulation and bronchial circulation. There are multiple anastomoses between pulmonary and bronchial arteries which create physiologic right to left shunts. Blood in the hemoptysis is mostly originated from the Lung. However, it could be from the gastrointestinal system as well. Primary origin of the blood comes from bronchial arteries. However, other sources of bleeding might be pulmonary vessels, aorta, intercostal, coronary, thoracic, and phrenic arteries. Hemoptysis is an important symptom that has different etiologies and pathogenesis mechanisms. Hemoptysis may happen following infarction and ischemia of pulmonary parenchyma as seen in pulmonary embolism, vasculitis, and infections. Another mechanism of hemoptysis is vascular engorgement with erosion as seen in bronchitis, bronchiectasis, and toxic exposure to cigarette and other irritants. In some cases underlying cause can not be identified and they are considered as idiopathic. However, they might present with massive hemoptysis. There are multiple conditions that are associated with hemoptysis which include granulomatosis with polyangiitis, sarcoidosis, immunodeficiency, and indoor ice hockey play.

• Lung has two main vascular systems that include pulmonary circulation and bronchial circulation.
• There are multiple anastomoses between pulmonary and bronchial arteries which create physiologic right to left shunts.
  • Pulmonary circulation
    • It provides gas exchange where deoxygenated blood enters the lungs through pulmonary arteries and oxygenated blood returns to circulation through pulmonary veins.
    • Pulmonary arteries
    • Pulmonary veins

• Bronchial vessels

• Bronchial vessels provide blood and nutrients to the bronchi and connective tissue of the lungs. They supply blood to the respiratory bronchioles and the visceral pleura of the lung.
• Bronchial arteries carry oxygenated blood to the lung tissues. They are arised from the thoracic aorta. There are usually two branches to the left lung and one to the right lung.
• Bronchial veins return the deoxygenated blood from the lung tissues to the systemic circulation. The right side drains into the azygos vein and the left side drains into the left superior intercostal vein or the accessory hemiazygos vein.

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• Hemoptysis is an important symptom that has different etiologies and pathogenesis mechanisms.^[3]
• Blood in the hemoptysis is mostly originated from the Lung. However, it could be from the gastrointestinal system as well.^[4]
• Primary origin of the blood comes from bronchial arteries.^[5][6]
• Other sources of bleeding might be:^[7][8][6]
  • Aorta
  • Intercostal arteries
  • Coronary arteries
  • Thoracic arteries
  • The upper and inferior phrenic arteries
  • Pulmonary vessels
• Hemoptysis may happen following infarction and ischemia of pulmonary parenchyma. It is seen in following conditions:^[9][10]
  • Pulmonary emboli
  • Vasculitis including:
    • Granulomatosis with polyangiitis
  • Infections including:
    • Staphylococcus aureus
    • Pseudomonas aeruginosa
    • Aspergillus fumigatus
    • The phycomycetes
• Another mechanism of hemoptysis is vascular engorgement with erosion. It is seen in following conditions:^[11][12]
  • Acute infection such as:
    • Viral bronchitis
    • Bacterial bronchitis
  • Chronic infection such as:
    • Bronchiectasis
  • Toxic exposure
    • Cigarette smoke
    • Asbestos
• In some cases underlying cause can not be identified and they are considered as idiopathic. However, they might present with massive hemoptysis.^[13][14]

• There are multiple conditions that are associated with hemoptysis which include:
  • Granulomatosis with polyangiitis
  • Sarcoidosis
  • Immunodeficiency
  • Indoor ice hockey play^[15][16]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Teresa Stahl, M.D. [2]; Sadaf Sharfaei M.D.[3]

Life-threatening causes of hemoptysis include pulmonary embolism. Common causes of hemoptysis include bronchiectasis, acute respiratory tract infections, chronic obstructive pulmonary disease, bronchitis, pneumonia, lung cancer, tuberculosis, cystic fibrosis, and idiopathic. Less common causes of hemoptysis include ruptured aneurysms, lung abscess, aspergilloma, idiopathic pulmonary fibrosis, behçet’s disease, aortobronchial fistula, pulmonary endometriosis, goodpasture syndrome, and foreign body aspiration.

• Hemoptysis may be a life-threatening condition depending on the severity of bleeding and must be treated appropriately.
• Life-threatening causes of hemoptysis include:
  • Pulmonary embolism

• Common causes of hemoptysis include:^{[1][2][3][4][5]}
  • Bronchiectasis
  • Acute respiratory tract infections
  • Chronic obstructive pulmonary disease
  • Bronchitis
  • Pneumonia
  • Lung cancer
  • Tuberculosis
  • Cystic fibrosis
  • Idiopathic

• Less common causes of hemoptysis include:^[6][7]
  • Ruptured aneurysms which include:
    • Ruptured aortic aneurysm
    • Ruptured bronchial artery aneurysm
    • Ruptured pulmonary artery aneurysm
  • Lung abscess
  • Aspergilloma
  • Idiopathic pulmonary fibrosis
  • Behçet’s disease
  • Aortobronchial fistula

• Pulmonary endometriosis

• Goodpasture syndrome
• Foreign body aspiration

Cardiovascular	Absence of pulmonary artery, adult respiratory distress syndrome (ARDS), aortic aneurysm, arterial bronchial fistula, arteritis, arteriovenous malformation, bronchial artery aneurysm, Bronchial varices, cardiac failure, common ventricle, congenital heart disease, congenital mitral malformation, congenital mitral stenosis, congestive heart failure, Ehler-danlos syndrome (vascular type), Eisenmenger syndrome, fat embolism, heart failure, heart valve damage, hereditary haemorrhagic telangiectasia, isolated unilateral absence of pulmonary artery, left atrial hypertension, left atrial myxoma, leukocytoclastic angiitis, Meadows syndrome, microscopic polyangiitis, mitral stenosis, polyarteritis nodosa, portal vein obstruction, pulmonary arteriovenous fistula, pulmonary arteriovenous malformation, pulmonary artery agenesis, pulmonary artery rupture, pulmonary artery stenosis, pulmonary embolism, pulmonary hemangioma, pulmonary hypertension, pulmonary infarction, pulmonary thrombosis, pulmonary vein stenosis, Rasmussen’s aneurysm, ruptured aortic aneurysm, ruptured arteriovenous fistula, septic pulmonary emboli, tetralogy of Fallot, tracheal-innominate artery fistula, tricuspid endocarditis, vasculitis, Wegener granulomatosis
Chemical / poisoning	Asbestosis, chronic berylliosis, cocaine, crack cocaine, cytotoxic drug use, inhalation of nitrogen dioxide, inhalation of pesticides, naphtha poisoning, sulfuric acid poisoning, toluene diisocyanate poisoning, vanadium poisoning
Dermatologic	Follicular hamartoma–alopecia–cystic fibrosis
Drug Side Effect	Amiodarone, anticoagulants, aspirin, bedaquiline fumarate, bevacizumab, Cidofovir, cocaine, crack cocaine, cytotoxic drug use, dicoumarol, herbal agent adverse reaction, penicillamine, Pentamidine Isethionate, phenprocoumon, pramipexole, propylthiouracil, rifapentin,sertraline, Tiagabine, warfarin, zonisamide,
Ear Nose Throat	Carcinoma of the vocal tract, laceration vocal cords, laryngeal carcinoma, laryngitis, oropharyngeal cancer, pharyngeal cancer, pharyngeal polyps, pharyngeal tumor, pharyngeal ulceration, severe nosebleed, stomatitis, tonsillectomy, tonsillitis
Endocrine	No underlying causes
Environmental	Asbestosis, chronic berylliosis
Gastroenterologic	Cirrhosis of liver, Dieulafoy’s disease, esophageal carcinoma, esophageal tumors, oropharingeal cancer, portal vein obstruction, severe vomiting, tracheo-esophageal fistula and esophageal varices
Genetic	Alpha 1-antitrypsin deficiency, carbamoylphosphate synthetase deficiency, cystic fibrosis, Ehler-danlos syndrome (vascular type), follicular hamartoma–alopecia–cystic fibrosis , hemophilia, hereditary haemorrhagic telangiectasia
Hematologic	Blood dyscrasias, coagulopathy, disseminated intravascular coagulation, essential thrombocytosis, fetal and neonatal alloimmune thrombocytopenia, hemangioma, hemophilia, hemorrhagic diathesis, Henoch-Schönlein purpura, leukemia, malignant lymphoma, thrombotic thrombocytopenic purpura, Von Willebrand disease
Iatrogenic	Bronchoscopy, endotracheal tube, laryngoscopy, lung biopsy, lymphangiography, mediastinoscopy, post fontan procedure, pulmonary artery catheter, spirometry, suctioning of airways, Swan Ganz catheter-induced infarction, tonsillectomy, tracheotomy, transbronchial biopsy, transtracheal aspiration
Infectious Disease	Actinomycosis, allergic bronchopulmonary aspergillosis, alveolar hydatid disease, anerobic pneumonia, ascariasis, aspergilloma, coccidioidomycosis, dengue, dirofilaria immitis, echinococcal cyst, echinococcus granulosus, francisella tularensis, histoplasmosis, HIV, hookworm, influenza, klebsiella, Lemierre’s syndrome, linguatula serrata, lung abscess, lupus pneumonitis, melioidosis, mucormycosis, mycetoma, mycobacterium tuberculosis, mycobacterium xenopi, necrotic pneumonia, nocardiosis, nosocomial pneumonia, paracoccidioidomycosis, paragonimiasis, pneumonia, pneumonic plague, pulmonary helminth infection, rhodococcus equi, serratia marcescens, stachybotrys atra, stachybotrys chartarum, staphylococcal respiratory infection, stomatitis, torulopsis, tropical pulmonary eosinophilia, tuberculosis, yersinia pestis, zygomycosis
Musculoskeletal / Ortho	Ehler-danlos syndrome (vascular type)
Neurologic	No underlying causes
Nutritional / Metabolic	Carbamoylphosphate synthetase deficiency
Obstetric/Gynecologic	Catamenial hemoptysis, endometriosis, intrathoracic endometriosis, meadows syndrome
Oncologic	Adenocarcinoma of lung, adenoid cystic carcinoma, adenoma, adult small cell lung cancer, benign lung tumor, bronchial adenoma, bronchiolo-alveolar adenocarcinoma, bronchogenic carcinoma, carcinoma of the vocal tract, esophageal carcinoma, esophageal tumors, large cell carcinoma, laryngeal carcinoma, leukemia, lung metastases, lung neoplasm, malignant lymphoma, mediastinal tumors, mesothelioma, mixed type non small cell carcinoma, oropharyngeal cancer, papillomatosis carcinoid, pharyngeal cancer, pharyngeal tumor, pleuropulmonary blastoma, pulmonary hemangioma, rib tumor, tracheal cancer
Opthalmologic	No underlying causes
Overdose / Toxicity	Inhalation of pesticides
Psychiatric	Münchausen syndrome
Pulmonary	Acute or chronic bronchitis, adenocarcinoma of lung, adenoid cystic carcinoma, adenoma, adult respiratory distress syndrome (ARDS), adult small cell lung cancer, alpha 1-antitrypsin deficiency, alveolar hydatid disease, alveolitis, anerobic pneumonia, anti-glomerular basement membrane antibody-mediated disease, arterial bronchial fistula, asbestosis, benign lung tumor, bronchial adenoma, bronchial artery aneurysm, bronchial varices, bronchiectasis, bronchiolo-alveolar adenocarcinoma, bronchitis, bronchogenic carcinoma, bronchogenic cyst, bullous emphysema, catamenial hemoptysis, chronic berylliosis, chronic bronchitis, cystic fibrosis, esophageal carcinoma, esophageal tumors, fat embolism, foreign body in respiratory tract, Goodpasture syndrome, hemangioma, hereditary haemorrhagic telangiectasia, histiocytosis x, histoplasmosis, Hughes-Stovin syndrome, idiopathic pulmonary hemosiderosis, inflammatory pseudotumor of the lung, intrathoracic endometriosis, isolated unilateral absence of pulmonary artery, Kartagener syndrome, large cell carcinoma, lung abscess, lung fibrosis, lung metastases, lung neoplasm, lung polyps, lung trauma, lung ulceration, lung vasculitis, lupus pneumonitis, lymphangiomyomatosis, lymphocytic interstitial pneumonia, lymphomatoid granulomatosis, malignant lymphoma, mesothelioma, mixed type non small cell carcinoma, papillomatosis carcinoid, penetrating chest wound, pleuropulmonary blastoma, pulmonary arteriovenous fistula, pulmonary arteriovenous malformation, pulmonary artery agenesis, pulmonary artery rupture, pulmonary artery stenosis, pulmonary congestion, pulmonary edema, pulmonary embolism, pulmonary fibrosis, pulmonary hypertension, pulmonary infarction, pulmonary sequestration, pulmonary thrombosis, pulmonary vein stenosis, Rasmussen’s aneurysm, ruptured bronchi, septic pulmonary emboli, Swan Ganz catheter-induced infarction, tracheal cancer, tracheal trauma, tracheal ulceration, tracheal-innominate artery fistula, tracheitis, tracheo-esophageal fistula and esophageal varices, tracheobronchitis, tracheobronchopathia osteoplastica , tropical pulmonary eosinophilia, uremic lung
Renal / Electrolyte	Goodpasture syndrome , uremic lung
Rheum / Immune / Allergy	Amyloidosis, anti-glomerular basement membrane antibody-mediated disease, Behcet’s syndrome, extrinsic allergic alveolitis, fetal and neonatal alloimmune thrombocytopenia, Goodpasture syndrome, Henoch-Schönlein purpura, histiocytosis x, HIV, Hughes-Stovin syndrome, immunodeficiency, lymphocytic interstitial pneumonia, microscopic polyangiitis, sarcoidosis, systemic lupus erythematosus, tropical pulmonary eosinophilia
Sexual	No underlying causes
Trauma	Chest injury, laceration vocal cords, lung contusion, lung trauma, penetrating chest wound, tracheal trauma
Urologic	No underlying causes
Dental	No underlying causes
Miscellaneous	Bronchogenic cyst, clove cigarettes, cocaine, crack cocaine, idiopathic, Kartagener syndrome, shrapnel, violent coughing

Absence of pulmonary artery Actinomycosis Acute or chronic bronchitis Adenocarcinoma of lung Adenoid cystic carcinoma Adenoma Adult respiratory distress syndrome (ARDS) Adult small cell lung cancer Allergic bronchopulmonary aspergillosis Alpha 1-antitrypsin deficiency Alveolar hydatid disease Alveolitis Amiodarone[8] Amyloidosis Anerobic pneumonia Anti-glomerular basement membrane antibody-mediated disease Anticoagulants Aortic aneurysm Arterial bronchial fistula Arteriovenous malformation Arteritis Asbestosis Ascariasis Aspergilloma Aspirin[9][10] Behcet’s syndrome [11] [12] Benign lung tumor Bevacizumab [13] Blood dyscrasias Bronchial adenoma Bronchial artery aneurysm Bronchialvarices Bronchiectasis Bronchiolo-alveolar adenocarcinoma Bronchitis Bronchogenic carcinoma Bronchogenic cyst Bronchoscopy Bullous emphysema Carbamoylphosphate synthetase deficiency Carcinoma of the vocal tract Cardiac failure Catamenial hemoptysis [14] Chest injury Chronic berylliosis Chronic bronchitis Cidofovir Cirrhosis of liver Clove cigarettes Coagulopathy Cocaine [15] Coccidioidomycosis Common ventricle Congenital heart disease Congenital mitral malformation Congenital mitral stenosis Congestive heart failure Crack cocaine[16] Cystic fibrosis Cytotoxic drug use [17] Dengue Dicoumarol Dieulafoy’s disease Dirofilaria immitis Disseminated intravascular coagulation[18] Echinococcal cyst Echinococcus granulosus Ehler-danlos syndrome (vascular type) Eisenmenger syndrome Endometriosis Endotracheal tube Esophageal carcinoma Esophageal tumors Essential thrombocytosis Extrinsic allergic alveolitis Fat embolism Fetal and neonatal alloimmune thrombocytopenia Follicular hamartoma–alopecia–cystic fibrosis Foreign body in respiratory tract

Francisella tularensis Goodpasture syndrome Heart failure Heart valve damage Hemangioma Hemophilia Hemorrhagic diathesis Henoch-Schönlein purpura Herbal agent adverse reaction Hereditary haemorrhagic telangiectasia [19] Histiocytosis x Histoplasmosis HIV Hookworm Hughes-Stovin syndrome Idiopathic Idiopathic pulmonary hemosiderosis[20] Immunodeficiency Inflammatory pseudotumor of the lung Influenza Inhalation of nitrogen dioxide Inhalation of pesticides Intrathoracic endometriosis Isolated unilateral absence of pulmonary artery Kartagener syndrome[21] Klebsiella Laceration vocal cords Large cell carcinoma Laryngeal carcinoma Laryngitis Laryngoscopy Left atrial hypertension Left atrial myxoma Lemierre’s syndrome [22] Leukemia Leukocytoclastic angiitis Linguatula serrata Lung abscess Lung biopsy Lung contusion Lung fibrosis Lung metastases Lung neoplasm Lung polyps Lung trauma Lung ulceration Lung vasculitis Lupus pneumonitis Lymphangiography Lymphangiomyomatosis [23] Lymphocytic interstitial pneumonia Lymphomatoid granulomatosis Malignant lymphoma Meadows syndrome Mediastinal tumors Mediastinoscopy Melioidosis Mesothelioma Microscopic polyangiitis Mitral stenosis Mixed type non small cell carcinoma Mucormycosis Münchausen syndrome[24] Mycetoma Mycobacterium tuberculosis Mycobacterium xenopi Naphtha poisoning Necrotic pneumonia[25] Nocardiosis Nosocomial pneumonia Oropharyngeal cancer Papillomatosis carcinoid Paracoccidioidomycosis Paragonimiasis [26] Penetrating chest wound Penicillamine[27][28] Pharyngeal cancer Pharyngeal polyps

Pharyngeal tumor Pharyngeal ulceration Phenprocoumon[29] Pleuropulmonary blastoma Pneumonia Pneumonic plague Polyarteritis nodosa Portal vein obstruction Post fontan procedure[30] Propylthiouracil[31] Pulmonary arteriovenous fistula Pulmonary arteriovenous malformation Pulmonary artery agenesis Pulmonary artery catheter Pulmonary artery rupture Pulmonary artery stenosis Pulmonary congestion Pulmonary edema Pulmonary embolism Pulmonary fibrosis Pulmonary helminth infection Pulmonary hemangioma Pulmonary hypertension Pulmonary infarction Pulmonary sequestration Pulmonary thrombosis Pulmonary vein stenosis Rasmussen’s aneurysm Rhodococcus equi Rib tumor Ruptured aortic aneurysm Ruptured arteriovenous fistula Ruptured bronchi Sarcoidosis Septic pulmonary emboli Serratia marcescens Severe nosebleed Severe vomiting Shrapnel Spirometry Stachybotrys atra Stachybotrys chartarum Staphylococcal respiratory infection Stomatitis Suctioning of airways Sulfuric acid poisoning Swan ganz catheter-induced infarction Systemic lupus erythematosus Tetralogy of fallot Thrombocytopenia Thrombotic thrombocytopenic purpura Tiagabine Toluene diisocyanate poisoning Tonsillectomy Tonsillitis Torulopsis Tracheal cancer Tracheal trauma Tracheal ulceration Tracheal-innominate artery fistula Tracheitis Tracheo-esophageal fistula and esophageal varices Tracheobronchitis Tracheobronchopathia osteoplastica Tracheotomy Transbronchial biopsy[32] Transtracheal aspiration Tricuspid endocarditis Tropical pulmonary eosinophilia Tuberculosis [33] Uremic lung Vanadium poisoning Vasculitis Violent coughing Von Willebrand disease Warfarin [34] Wegener granulomatosis Yersinia pestis Zygomycosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Karina Zavaleta, MD [2], Anmol Pitliya, M.B.B.S. M.D.[3]

Organ system		Diseases	Clinical manifestations								Diagnosis				Other features
			Symptoms							Physical exam
			Onset	Duration	Productive cough	Hemoptysis	Weight lost	Fever	Dyspnea	Ascultation	Lab findings	Imaging	PFT	Gold standard
Respiratory	Lower airway	Bronchiectasis[1]	Chronic	Months to years	+ Mucopurulent sputum	+	−	−	+	Crackles Wheezing Shortness of breath	Complete blood count (CBC) IgG, IgM and IgA Sputum culture for fungi, bacteria and mycobacteria	Linear atelectasis and dilated airways in chest X−Ray	FEV1/FVC <70% Normal FVC Low levels of FEV1	CT of chest	Digital clubbing Recurrent pleurisy
		Foreing body aspiration[2][3][4]	Acute	Variable	+	+	−	+	+	Wheezing Decreased breath sounds	No specific tests	Hyperinflated lungs, atelectasis, and mediastinitis Shift in chest radiograph when the object is radio−opaque CT may be helpful	Not specific	Bronchoscopy	In children <1 year and adults >75 years Organic materials in children Inorganic materials in adults
	Parenchyma	Lung cancer[5][6]	Chronic	Years	+	+	+	+/−	+	Hoarseness	The following investigations may be helpful: Complete blood count (CBC) ALT, AST Calcium Alkaline phosphatase LDH Creatinine	Contrast−enhanced CT of chest and upper abdomen	Not specific	Tissue biopsy (sample should be sufficient for molecular testing)	Risk factor: Cigarette smoking Types Small cell lung cancer (SCLC) Non−small cell lung cancer (NSCLC)
		Interstitial lung disease[7][8]	Chronic	Variable	−	+	+	−	+	Wheezing Crackles or velcro rales Inspiratory high−pitched rhonchi	The following investigations may be helpful: Hepatic function test Renal function test CBC Serological testing	Nodular, reticular or both pattern in chest X−ray CT in patients with diffuse pulmonary lung disease	Reduction in FVC, RV, FRC, TLC and FEV1 on spirometry FEV1/FVC normal or increase Lung volumes Diffusion capacity (DLCO reduced)	Lung biopsy when lab, imaging, and PFT has indeterminate result	Clubbing is common in asbestosis and idiopathic pulmonary fibrosis
		Tuberculosis (TB)[9][10]	Chronic	More than 2 or 3 weeks	+	+	+	+	+	Pleural effusion Crackles Whispered pectoriloquy Decreased fremitus Rhonchi	Sputum acid−fast bacilli (AFB) smear may be positive Mycobacterial culture may be positive Molecular testing may be helpful	Reactivation of TB is observed as infiltration in the upper lobe in chest X−Ray In patients with HIV, Tb is observed as lobar infiltration, adenopathy, lung mass named tuberculoma, small fibronodular lesions, and/or pleural effusion on chest X−Ray CT can detect early nodal process	Decreased FEV1 Reduced FVC	Isolation of Mycobacterium tuberculosis from some secretion	Etiology: Mycobacterium tuberculosis Complications: Pneumothorax, bronchiectasis, pulmonary destruction and chronic pulmonary aspergillosis
Organ system		Diseases	Onset	Duration	Productive cough	Hemoptysis	Weight lost	Fever	Dyspnea	Ascultation	Lab findings	Imaging	PFT	Gold standard	Other features
Cardiac		Mitral Stenosis[11][12]	Chronic	Variable	+ Pink frothy	+	−	−	+	Crackles Hoarseness	Not specifc	Electrocardiogram may be helpful Enlargement of left atrium and appendage in chest radiograph	FVC reduced	Resting transthoracic echocardiography	Stress testing Cardiac catheterization
		Pulmonary hypertension[13][14]	Chronic	More than 2 years	−	+	+	−	+	Hoarseness	The following investigations may be helpful: HIV serology Antinuclear antibody (ANA) Rheumatoid factor (RF) Anti−neutrophil cytoplasmic antibody (ANCA)	Enlargement of the central pulmonary artery and right heart in chest X−Ray Pulmonary artery systolic pressure can be estimated in echocardiography	Low levels of FEV1 Decreased FVC DLCO reduced	Mean pulmonary artery pressure more than 25 mmHg at rest	Chest pain Ascites Syncope Peripherial edema
Autoimmune		Goodpasture syndrome[15][16]	Chronic	Variable	−	+	−	−	+	Shortness of breath	The following investigations may be helpful: Complete blood count (CBC) ANCA positive Anti−GBM in ELISA or western blot	Pulmonary infiltratation in chest X−Ray CT scan for parenchymal involvement	Increased DLCO Decreased TLC Decreased FVC	Renal biopsy	Hematuria Proteinuria
		Wegener’s disease (GPA) [17][18]	Chronic	Months	+	+	+	+	+	Hoarseness Stridor Wheezing	The following investigations may be helpful: ANCA, P−ANCA, C−ANCA BUN Creatinine Complete blood count Urinalysis Lung biopsy	Nodules, pulmonary infiltrates, reticular margins, pleural opacities and cavities in chest X−Ray Nodules, cavities and stellate−shaped peripherial pulmonary in chest CT Bronchoscopy may be helpful	Low levels of DLCO Reduce lung volumes	Tissue biopsy	Nasal crusting, sinus pain, chronic rhinosinusitis, nasal obstruction and discharge in upper airway Saddle nose deformity Purpura in lower extremities
		Microscopic polyangitis (MPA)[19]	Chronic	Variable	+	+	+	+	+	Hoarseness Stridor Wheezing	The following investigations may be helpful: ANCA positive BUN Creatinine Complete blood count Urinalysis	Cavitation, nodules, and alveolar opacities in chest X−ray Head and chest CT may be helpful Electromyography/nerve conduction study may also be helpful	Reduced lung volumes	Tissue biopsy	Nerve damage Rhinosinusitis Purpura involving lower extremities
		Churg−Strauss[20][21]	Chronic	Variable	+	+	+	+	+	Wheezing Rales Rhonchi Expiratory sounds(related to asthma)	Peripherial eosinophilia In active phase CRP and erytrocyte sedimentation rate high Elevated IgE ANCA positive	Infiltrates in chest X−Ray Ground glass opacities, tree−in−bud sign and small nodules in chest CT	Lung volumes decreased FVC reduced FEV1/FVC ratio <70%	Tissue biopsy	Asthma Eosinophilia Rhinosinusitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

The incidence of hemoptysis is approximately 100 per 100,000 individuals in the outpatient setting. There is no enough data on prevalence of hemoptysis. The mortality rate of patients with massive hemoptysis is approximately 50-100%, if left untreated. During 1995-2005, in-hospital mortality rate of massive hemoptysis was 0-15%. Patients of all age groups may develop hemoptysis. There is no racial predilection to hemoptysis. Hemoptysis affects men and women equally. Hemoptysis is a symptom that might affect everyone. Underlying causes of hemoptysis might be different in developed countries than in developing countries. In the United States, incidence of tuberculosis in patients with massive hemoptysis is 7%, while in south africa it is 85%.

• The incidence of hemoptysis is approximately 100 per 100,000 individuals in the outpatient setting.^[1]

• There is no enough data on prevalence of hemoptysis.

• The mortality rate of patients with massive hemoptysis is approximately 50-100%, if left untreated.^[2][3]
• During 2000-2005, in-hospital mortality rate of massive hemoptysis was 0%.^[4]
• During 1995-1999, in-hospital mortality rate of massive hemoptysis was 15%.^[4]

• Patients of all age groups may develop hemoptysis.

• There is no racial predilection to hemoptysis.

• Hemoptysis affects men and women equally.

• Hemoptysis is a symptom that might affect everyone.

• Underlying causes of hemoptysis might be different in developed countries.
• In the United States, incidence of tuberculosis in patients with massive hemoptysis is 7%.^[5]

• Underlying causes of hemoptysis might be different in developing countries.
• In south africa, incidence of tuberculosis in patients with massive hemoptysis is 85%.^[5]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Common risk factors in the development of hemoptysis include cigarette smoking, chronic obstructive pulmonary disease, anticoagulant medications. Risk factors in the recurrence of hemoptysis include persistent residual mild bleeding following bronchial artery embolization, blood transfusion before the procedure, and aspergilloma. Less common risk factors in the development of hemoptysis include congestive heart failure and mitral regurgitation.

• Common risk factors in the development of hemoptysis include:^[1][2][3]
  • Cigarette smoking
  • Chronic obstructive pulmonary disease
  • Anticoagulant medications
• Risk factors in the recurrence of hemoptysis include:^[4]
  • Persistent residual mild bleeding following bronchial artery embolization
  • Blood transfusion before the procedure
  • Aspergilloma
• Less common risk factors in the development of hemoptysis include:
  • Congestive heart failure
  • Mitral regurgitation

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

There is insufficient evidence to recommend routine screening for hemoptysis.

There is insufficient evidence to recommend routine screening for hemoptysis.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Hemoptysis is an important symptom that indicates an underlying pulmonary or extrapulmonary causes. Hemoptysis usually happens following bronchitis as an acute symptom and it resolves spontaneously or with antibiotic therapy within a week. Watchful observation in a patient with hemoptysis and normal chest x-ray is recommended. However, persistent and massive hemoptysis requires further investigations. Asphyxia and airway obstruction are common after massive hemoptysis. Prognosis of hemoptysis is generally excellent. However, massive hemoptysis has a poor prognosis and the mortality rate of patients with hemoptysis is approximately 50-100%, if left untreated.

• Hemoptysis is an important symptom that indicates an underlying pulmonary or extrapulmonary causes.^[1][2]
• Hemoptysis usually happens following bronchitis as an acute symptom and it resolves spontaneously or with antibiotic therapy within a week.^[3]
• Watchful observation in a patient with hemoptysis and normal chest x-ray is recommended.
• Patients with warning signs must be investigated. Warning signs are as follow:^[4][5]
  • Persistent hemoptysis more than one week
  • Chest pain
  • Weight loss
  • Night sweats
  • Fever higher than 101 degrees
  • Shortness of breath
  • Change in cough
  • Fatigue

• Common complications of massive hemoptysis include:^[6][7]
  • Dehydration
  • Asphyxia 
  • Airway obstruction
  • Shock
  • Exsanguination
• Complications after bronchial artery embolization include:^[8][9][10]
  • Spinal cord injury
  • Esophageal ulcer
  • Stroke
  • Bronchial infarction
  • Transient chest pain

• Prognosis of hemoptysis is generally excellent. However, it requires further investigations.^[3]
• Massive hemoptysis has a poor prognosis and the mortality rate of patients with hemoptysis is approximately 50-100%, if left untreated.^[11][12]