Diffuse Alveolar Hemorrhage: Importance of Early Recognition and Treatment

Granulomatosis with Polyangiitis (GPA) is a small-vessel vasculitis classically associated with focal necrotizing vasculitis, necrotizing glomerulonephritis, and necrotizing granulomas in the lungs and upper airway. GPA can be classified as limited or severe. Those with severe disease were older at disease onset, had a higher likelihood of diffuse alveolar hemorrhage (DAH), and had a higher probability of previous thyroid disease. Common presentations of this disease are chronic sinusitis and perforation of the nasal septum, hematuria with red cell casts, cough, hemoptysis, and dyspnea. Renal involvement occurs in 77-85% throughout the disease course, while DAH occurs in 5-45% of patients. DAH is characterized by bleeding into the alveoli. It has a high mortality rate and requires supportive treatment and aggressive treatment of GPA.

We present the case of a 69-year-old male, who presented to the emergency department with generalized weakness and hematuria. The weakness progressed over several months to the point where the patient was fatigued and dyspneic with minimal exertion. The patient was found to have atrial fibrillation and renal insufficiency (creatinine of 3.6 with unknown baseline) and was admitted for further workup. Over the next few days, his renal function deteriorated (creatinine maximum: 8.2). The patient developed progressive dyspnea associated with hemoptysis and acute hypoxic respiratory failure. Chest x-ray (CXR) demonstrated pulmonary vascular congestion. The patient was started on broad-spectrum antibiotics on 1/1/24 and transferred to the ICU and hemodialysis was initiated. Repeat CXR showed no improvement after dialysis. The patient’s status deteriorated requiring intubation, vasopressors, and amiodarone. Workup revealed c-ANCA positivity and he was transferred to a referral center. Bronchoalveolar lavage revealed DAH secondary to GPA. The patient was treated with high-dose pulsed steroids (1 g Solumedrol, 5-day course) and transitioned to 80 mg prednisone. He was also started on cyclophosphamide. After 9 days the patient was extubated. Hemoglobin counts rebounded to 8.4-9 from previous levels of 6.9.

DAH is a rare but serious consequence of GPA associated with a mortality rate of 35-50%. Nearly two-thirds of patients require mechanical ventilation, and kidney involvement was not predictive of respiratory failure. Treatment focuses on supportive measures as well as treating the GPA. Current treatment includes high-dose pulsed steroids and rituximab or cyclophosphamide. Plasma exchange may be necessary. Long-term rituximab has also been shown to achieve higher remission rates than cyclophosphamide. Early recognition and initiation of treatment provide the best chances for survival.