Diffuse Alveolar Hemorrhage in Primary Versus Secondary Antiphospholipid Syndrome

Diffuse alveolar hemorrhage (DAH) occurs in patients with both primary and secondary antiphospholipid antibody syndrome (APS). We sought to determine the differences in clinical presentation, management, and outcomes of DAH in these patients.

We performed a medical records review study and reviewed 30 patients with DAH in the setting of primary and secondary antiphospholipid syndrome seen at our institution between January 1, 1997, and December 31, 2018. We analyzed their demographics, clinical presentation, laboratory values, imaging studies, lung pathology results, management, and outcomes.

The patients in the secondary APS cohort were younger (median age, 48.5 vs 58 years) and comprised more females (75% vs 17%) compared with those with primary APS (p < 0.05). Two thirds of patients in the secondary APS group were anemic compared with less than one fourth in the primary APS group (p = 0.005). At the time of the first episode of DAH, the patients in the secondary APS required invasive and noninvasive ventilation, antibiotics, and combination immunosuppressive therapy (includes a combination of glucocorticoids with immunosuppressants or intravenous immunoglobulins or plasma exchange) more often compared with those with primary APS. There was only one in-hospital death (3% in-hospital mortality). One-year and 5-year mortality rates were 20% and 27%, respectively, with no significant difference between the primary and secondary APS groups.

Diffuse alveolar hemorrhage in the setting of APS, especially secondary APS, can be severe. However, in-hospital mortality is uncommon with current management strategies.