CC: Shortness of Breath, Hemoptysis
HPI: This is a 23 year-old male with no past medical history who presents with progressive fatigue, pleuritic chest pain, shortness of breath, and hemoptysis for 2 months. Initially his cough was productive with occasional blood-streaked sputum but progressed to frank hemoptysis in the last 3-4 weeks. Additionally, he reports subjective intermittent fevers and chills for 3-4 weeks. Also notable is a 30 lbs unintentional weight loss over 2 months. Exam shows patient saturating at 78% on room air, tachypneic to 30, febrile to 38.6, and tachycardic to 128. Patient has diffuse crackles and wheezes from bases to mid lung fields but no rashes or joint findings. His labs were notable for a normocytic anemia and leukocytosis with left shift. CXR showed bilateral diffuse disease (see below) with CT demonstrating ground-glass opacities in a central distribution. On BAL, diffuse blood and secretions throughout all segments of lobes on both side of the lungs were visualized. A diagnosis of Diffuse Alveolar Hemorrhage was made with the underlying etiology being Systemic Lupus Erythematosus based off other findings of nephritic syndrome with renal biopsy demonstrating Lupus Nephritis Class 3, +ANA, +dsDNA, low complements.
Morning Report Pearls:
Clinical suspicion for Diffuse Alveolar Hemorrhage should always be present when diffuse infiltrates on Chest X-ray are seen in the setting of anemia, respiratory failure and hemoptysis. Of note, one third of patients will not have hemoptysis!
Diagnosis is made on BAL when it demonstrates serial samples that are progressively hemorrhagic. Yield is higher if performed within the first 48 hours of symptoms.
There are many causes of DAH with overlapping pathophysiology. DAH can result from small vessel inflammation resulting in alveoli necrosis followed by leakage of RBCs into the lung, bleeding into the alveolar space as a result of diffuse alveolar damage (ARDS pathologies), or bland hemorrhage resulting from etiologies like coagulopathies.
Pulmonary Capillaritis Conditions:
- Systemic Vasculitis (GPA, MPA, Cryoglobunemia, HSP, IgA vasculitis)
- Connective Tissue Disorders (SLE, MCTD, RA, APLS)
- Drugs like Retinoic acid or PTU
- Lung transplant rejection
Bland Pulmonary Hemorrhage Conditions:
- Left sided heart failure
- Mitral stenosis or mitral regurgitation
- Anticoagulants or coagulopathies like DIC
- Pulmonary Veno-occlusive Disease
- Drugs like Marcobid
Diffuse Alveolar Damage Conditions:
- Anything that can cause ARDS like infections
- Bone marrow transplantation
- Crack cocaine inhalation
When having a patient with DAH, always consider the Pulmonary-Renal Syndromes which is defined as DAH with Glomerulonephritis. This is one of many reasons to always obtain a urinalysis on these patients. The pulmonary-renal syndromes really narrow the above list to Vasculitis and Connective Tissue Disorders if present.