Uncontrolled Pain as Sign of Worsening Ischemia in Acute Catastrophic Anti-phospholipid Syndrome

Case Diagnosis: Ischemic pain in acute catastrophic anti-phospholipid syndrome

Case Description or Program Description: A 32 year old female with a past medical history of systemic lupus erythematosus (SLE) and end-stage renal disease on hemodialysis presented to the emergency department with acute chest and foot pain. Patient was admitted to the cardiac intensive care unit with sepsis and myocarditis. Pain medicine was consulted on day 26 of admission after intravenous hydromorphone failed to adequately control her pain overnight. Patient was noted to have black necrotic tissue on her bilateral toes. Hydromorphone patient controlled analgesia (PCA) pump controlled her pain during the day. Cyclically overnight she would develop acute hemolysis with rapid drops in her hemoglobin which manifested by worsening pain uncontrolled by the PCA pump. Pain uncontrolled by her PCA pump repeatedly signaled her hemoglobin dropped below 7.0 g/dL requiring transfusion. Extensive workup revealed a diagnosis of acute catastrophic antiphospholipid syndrome (CAPS). She developed necrosis which progressed proximally during hospitalization requiring left transfemoral amputation. Following amputation her pain was better controlled with oral pain regimen.

Setting: Acute care hospital

Assessment/Results: Hydromorphone PCA pump failed to control cyclical nighttime pain due to CAPS and was used to identify worsening ischemia and guide treatment.

Discussion (relevance): CAPS is a rare condition with associated hemolysis and vasculitis which generates ischemic pain, commonly in distal perfusion areas. Four definitive diagnostic criteria must be met for CAPS: involvement of 3 or more organs, manifestations in less than a week, histopathology of small vessel occlusion, and laboratory confirmation of antiphospholipid antibodies. Pain is often used as an indicator of new or worsening pathology and should clinically be used to guide diagnostic testing and treatment.

Conclusions: Loss of pain control from high dose opioids can clinically indicate worsening ischemia and alert providers further diagnostic testing and treatment may be indicated, especially in conditions involving hemolysis and/or vasculitis.