We describe a middle aged male with history of APLS on chronic warfarin presenting for subacute abdominal pain and dry cough found to have bilateral adrenal hemorrhage with hospital course complicated by acute hypoxic respiratory failure. Patient then had frank hemoptysis from DAH likely due to 1) catastrophic APLS and 2) ANCA Vasculitis (MPO+). Differential for DAH is as below. As we worked up massive hemoptysis leading to intubation, patient was ANA, ANCA and p-ANCA positive leading to ANCA vasculitis leading to DAH. At the same time, the bilateral adrenal hemorrhages led and DAH gave rise to concern for CAPS – catastrophic AntiPhospholipid Syndrome.
Classification for CAPS
- Evidence of involvement of three or more organs, systems and/or tissues
- Development of manifestations simulatenously or in less than a week
- Confirmation by histopathology of small vessel occlusion in at least one organ or tissue
- Lab confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin and anti-beta2-glycoprotein I antibodies)
All 4 criteria leads to a definite diagnosis of CAPS. Our patient met 3 of 4 criteria for CAPS leading to probable CAPS.
It is important to suspect DAH even when there is no frank hemotpysis but there is hypoxemia, anemia and diffuse GGO. Review differential fro DAH below. Patient received PLEX initially for possible CAPS and then received Rituximab IV + Methylprednisolone for ANCA associated vasculitis.