Catastrophic anti-phospholipid syndrome (CAPS) is characterized by microvascular thrombosis in multiple sites leading to multi-organ damage. It is a rare and fatal complication of antiphospholipid syndrome (APS). We present a rare case of CAPS that presented with bilateral (b/l) adrenal hemorrhage making the diagnosis challenging in this otherwise rare disease. A 51-year-old female was initially admitted with abdominal pain and found to have bilateral adrenal hemorrhage. Patient had a fulminant disease course in which she had thrombotic manifestations involving multiple organ systems. This case was especially challenging as the patient’s bilateral adrenal hemorrhage was the first manifestation of CAPS; the diagnosis of APS had to be made while treatment for presumed CAPS was emergently commenced for this life-threatening disease. Key to managing this condition is having a high index of suspicion for the diagnosis in patients presenting with multi-organ failure and multiple thromboses and hemorrhage.
Anti-phospholipid syndrome (APS) is a disease characterized by the presence of antiphospholipid antibodies (aPLs) with arterial or venous thrombosis with or without pregnancy complications.
Catastrophic anti-phospholipid syndrome (CAPS) is a rare life-threatening complication of APS (<1% of cases) with microvascular thrombosis that leads to multi-organ damage.
The typical presentation is with multi-organ dysfunction secondary to multi-organ thromboses with clinical manifestations that commonly overlap with thrombotic microangiopathies.
The clinical manifestations depend on the organ affected by the small vessel occlusions. The most common organs involved during presentation are lungs, kidney, brain, skin and mesentery. Below, we present a rare case of catastrophic antiphospholipid syndrome with unusual first clinical presentation of bilateral adrenal hemorrhage making the diagnosis of CAPS a challenge. This case also highlights the high mortality associated with CAPS despite multidisciplinary approach to the treatment.