Case Presentation: A 44-year-old woman with a past medical history of systemic lupus erythematosus (SLE) not on maintenance therapy presented with joint pain, nausea, abdominal pain, and fever for one week. At the time of admission, the patient was found to have elevated ESR 72, CRP 30, and ferritin 4075. Admission labs were also notable for positive lupus anticoagulant, anti-cardiolipin and anti-nuclear antibodies, anemia, thrombocytopenia, hematuria, proteinuria, acute kidney injury, and low complement. A computed tomography scan (CT) of the abdomen pelvis demonstrated pancreatic and gastroduodenal inflammation, hydronephrosis, retroperitoneal fibrosis and lymphadenopathy. A kidney biopsy revealed thrombotic microangiopathy. The patient was diagnosed with anti-phospholipid syndrome (APLS) and transferred to the intensive care unit for pulse-dose steroids, intravenous immunoglobulin (IVIG), and heparin drip.  The patient was stabilized and transferred to the step down unit. Treatment was initiated with hydroxychloroquine and mycophenolate, however on hospital day 25 she developed severe abdominal pain and generalized tonic-clonic seizure requiring intubation. The patient suffered cardiac arrest and was revived after 2 cycles of ACLS. Repeat CT showed pancreatitis with fluid collections. The patient was transferred back to ICU for vasopressors and plasma exchange. Blood cultures grew streptococcus sanguinous that was treated with antibiotics. The patient’s clinical status gradually improved and she was discharged to acute inpatient rehab.

Discussion: APLS is characterized by arterial and venous thromboses in the context of circulating antiphospholipid antibodies (APLAs) including lupus anticoagulant, anticardiolipin, and/or anti-beta-2 glycoprotein antibodies. Some patients with APLS remain asymptomatic while others develop recurrent pregnancy loss and/or thrombocytopenia. Catastrophic APLS  (CAPS) is an APLS variant that manifests as multi-organ dysfunction and a systemic inflammatory response. Disease activity is triggered by bacterial or viral infections, SLE flare, withdrawal of anticoagulants, neoplasia, and/or obstetric complications. The diagnosis of CAPS is clinical but additional studies are helpful. A study comparing patients with APLS to healthy controls found elevated ferritin levels in 9% vs. 0% of subjects respectively (p<0.001). Of note, elevated ferritin was found in 71% of patients with CAPS, of which 36% had ferritin levels >1000 ng/ml, as in our patient. Treatment of CAPS requires anticoagulation (AC) and elimination of pathologic antibodies. CAPS has a 50% mortality rate, and prompt diagnosis and treatment are required to optimize patient outcomes.

Conclusions: CAPS is a highly morbid and deadly disease mediated by circulating APLAs. The diagnosis of CAPS clinical however extremely elevated ferritin levels may be suggestive of CAPS in the proper clinical context. The cornerstone of treatment includes AC and elimination of APLAs.