Pulmonary Hemorrhage

Karen Z. Voter, MD, FAAP, and Clement L. Ren, MD, MS

Introduction/Etiology/Epidemiology

•Hemoptysis in children is uncommon and is frequently related to a known underlying condition.

•Reports of “coughing up blood” can be related to bleeding from the nasopharynx, esophagus, lower airways, or lung parenchyma.

•Underlying conditions associated with pulmonary bleeding include

—Tracheostomy

—Cystic fibrosis (CF)

—Pulmonary hypertension, usually associated with congenital heart disease

—Autoimmune diseases, including granulomatosis with polyangiitis (formerly known as Wegener granulomatosis), systemic lupus erythematosus, microscopic polyangiitis, and Goodpasture syndrome

—Infection, usually tuberculous or fungal, though it can be bacterial

—Endobronchial mass

—Foreign body

Pathophysiology

•Mucosal irritation from dry secretions or trauma

•Mucosal trauma, especially with a tracheostomy or foreign body

•Pulmonary hypertension from congenital heart disease or arteriovenous malformation

•Pulmonary embolism

•Autoimmune vasculitis

•Erosion through pulmonary tissue into bronchial vessels

—Can be massive if a bronchial artery is eroded

—Occurs in CF

•Idiopathic pulmonary hemosiderosis (IPH)

—Thought to be immune mediated, but without detectable autoantibodies

—Heiner syndrome is a subform of IPH associated with allergy to milk protein

•Coughing or suctioning up blood is the most common presentation.

•Anemia (iron deficiency) may be present.

•There may be opacities on chest radiographs that are typically more transient than those of pneumonia.

•The patient may have multiple episodes of bleeding.

•Especially with massive bleeding, patients can often localize the site.

•Signs of infection include fever and leukocytosis.

•Signs of pulmonary embolism may be present.

—Risk factors include therapy with oral contraceptives.

•The presence of renal disease or rash suggests an autoimmune disorder.

Differential Diagnosis

•Nonpulmonary source of bleeding

—Nose

—Upper airway

—Gastrointestinal

•Pneumonia

•Complication of an underlying diagnosis

•Congenital heart disease

•IPH