Pulmonary Haemorrhage

Pulmonary haemorrhage is not uncommon in paediatric respiratory practice and can present with a wide spectrum of severity. Early recognition is essential, as identifying the precise source of bleeding — whether airway, lung parenchyma, or extrapulmonary — is crucial for guiding accurate diagnosis and management. Prompt, targeted intervention improves outcomes and helps prevent recurrence or complications.

Introduction

• Pulmonary haemorrhage is a condition which results in the extravasation of blood into the lung airways, parenchyma or alveoli.
• It can either be from the bronchial circulation or the pulmonary circulation and can either be focal or diffuse bleeding.
• The cause of focal pulmonary haemorrhage is usually from the airways and is frequently from the bronchial circulation and spares the alveoli.
• Diffuse alveolar haemorrhage (DAH) occurs when there is bleeding as a result of injury to the pulmonary capillaries, venules or arterioles.
• DAH leads to blood filling the alveolar spaces and can rapidly compromise gas exchange.
• This chapter focuses on diffuse alveolar haemorrhage in children.

Table 1: Causes of Haemoptysis

Table 2: Mimics of Haemoptysis

Clinical Presentation

Diagnosis

• Classic triad of haemoptysis, chest infiltrates and anaemia.
• In children, haemoptysis may be absent and present as cough.
• Usually normocytic, normochromic anaemia; if chronic, may be microcytic hypochromic.

History

• Recurrent respiratory symptoms; may or may not report haemoptysis.
• Treated for anaemia but not responding to therapy.
• Underlying clinical conditions:
  • Connective tissue disease.
  • Systemic lupus erythematosus (SLE).
  • Renal disease.
  • Infections including pulmonary tuberculosis.
  • Congenital malformations.
  • Cardiovascular disease.
  • Bleeding disorder.

Clinical signs

• As above, with signs of underlying condition.
• Acute respiratory distress (if acute bleed).
• Tachypnoea.

Investigations

Blood

• Full blood count (FBC), reticulocyte count.
• Coagulation screen (INR, fibrinogen).
• Immunoglobulins (IgA, IgG, IgE).
• *Autoimmune screen:
  • Rheumatoid factor.
  • Anti-dsDNA (double stranded deoxyribonucleic acid).
  • ANA (antinuclear antibody).
  • p-ANCA (perinuclear anti-neutrophil cytoplasmic antibodies).
  • c-ANCA (cytoplasmic anti-neutrophil cytoplasmic antibodies).
  • Anti-ribonucleic acid antibodies.
  • Factor VIII.
  • Von Willebrand’s factor.
  • Anti-GBM (glomerular basement membrane) antibodies.
  • Anti-MPO (myeloperoxidase) antibodies.
  • Anti-PR3 (proteinase 3) antibodies.
• Cow’s milk allergen IgE.
• Coeliac IgA transmutase.
• Urine dipstick.

Bronchoscopy

• Blood or blood-stained fluid; identification of cause of bleed (congenital malformations, lymph nodes, pulsatile masses, pulmonary tuberculosis lesions, etc.).
• Bronchoalveolar lavage: haemosiderin-laden macrophages, free iron.

Chest radiograph

• Findings depend on nature of bleed.
• Diffuse lung changes.
• Acute/massive bleed:
  • Consolidation.
  • “White out”.
  • Chest infiltrates.
• Chronic disease:
  • Chest infiltrates.
  • Interstitial changes.
  • Diffuse changes.

Chest CT scan

• Ground-glass appearance with mosaic attenuation.
• Fibrosis.
• Cysts.

Echocardiogram

• Presence of congenital heart disease, arteriovenous malformations, mitral valve disease.
• Presence of pulmonary hypertension.

Lung biopsy*

• Not mandatory but can guide management.
• Tests performed (see appendix)*.
• May show haemosiderin-laden macrophages, free iron, red blood cells, fibrosis, capillaritis with or without immunoglobulins, complement factors, micro-organisms.

Figure 1: Management of Children with Diffuse Alveolar Haemorrhage

Follow-up

• Regular review as needed and until stable.
• FBC, reticulocyte count and chest radiograph at every visit.
• Lung function testing:
  • Spirometry – in poor chronic control, restrictive pattern may be seen, although normal function is also frequent.
  • DLCO (diffusing capacity of the lungs for carbon monoxide), as available.
• Active bleed:
• Drop in haemoglobin and increase in reticulocyte count with or without presence of respiratory symptoms.
• Changes in lung function tests consistent with acute bleed.
• Increase in DLCO
• Chest radiograph

Complications

• If repeated episodes:
  • Pulmonary fibrosis.
  • Respiratory failure.
• If acute massive bleed:
  • Decompensation.
  • Respiratory failure and cardiovascular collapse.
  • Death.

Prognosis

• Varies between patients.
• Depends on underlying condition.
• Aim to use lowest dose of steroids necessary for control of disease.
• Bone-protective treatment:
  • Calcium supplement and vitamin D (400 IU orally daily).
• Isoniazid prophylaxis* for those on certain immunosuppressants and biologicals.

References

1. Godfrey S. Pulmonary hemorrhage/hemoptysis in children. Pediatr Pulmonol. 2004 Jun;37(6):476–84. doi: 10.1002/ppul.20020. PMID: 15114547.
2. Avital A, Springer C, Godfrey S. Pulmonary haemorrhagic syndromes in children. Paediatr Respir Rev. 2000 Sep;1(3):266–73. doi: 10.1053/prrv.2000.0058. PMID: 12531089.
3. Balfour-Lynn IM. Haemoptysis: is it really from the lungs? The well child who spits out blood. Arch Dis Child. 2023 Nov;108(11):879–883. doi: 10.1136/archdischild-2022-324276. Epub 2023 Mar 29. PMID: 36990647.