TY - JOUR
T1 - Diffuse Alveolar Hemorrhage in Primary Versus Secondary Antiphospholipid Syndrome
AU - Sangli, Swathi S.
AU - Ryu, Jay H.
AU - Baqir, Misbah
N1 - Publisher Copyright:
© Wolters Kluwer Health, Inc. All rights reserved.
PY - 2021/12/1
Y1 - 2021/12/1
N2 - Background 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. Methods 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. Results 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. Conclusions Diffuse alveolar hemorrhage in the setting of APS, especially secondary APS, can be severe. However, in-hospital mortality is uncommon with current management strategies.
AB - Background 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. Methods 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. Results 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. Conclusions Diffuse alveolar hemorrhage in the setting of APS, especially secondary APS, can be severe. However, in-hospital mortality is uncommon with current management strategies.
KW - acute hypoxic respiratory failure
KW - antiphospholipid syndrome
KW - diffuse alveolar hemorrhage
KW - diffuse alveolar infiltrates
KW - systemic lupus erythematosus
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U2 - 10.1097/RHU.0000000000001358
DO - 10.1097/RHU.0000000000001358
M3 - Article
C2 - 32195850
AN - SCOPUS:85120380204
VL - 27
SP - E297-E301
JO - Journal of Clinical Rheumatology
JF - Journal of Clinical Rheumatology
SN - 1076-1608
IS - 8
ER -