Case Presentation: A 45 yo African American woman with lupus nephritis and a previous episode of myocarditis presents with 5 days of abdominal pain, headache, decreased appetite and acute shortness of breath. She was found to be afebrile but tachycardic, tachypneic and hypoxic. Her physical exam was notable for severe bullous changes over the skin of her right hand. Her labs showed a profound metabolic acidosis, severe coagulopathy, acute renal failure, transamonitis, hemolytic anemia and thrombocytopenia. Echocardiogram demonstrated an ejection fraction of 25%. Dopplers showed clots in the following vessels: Right common femoral vein, right basilic and cephalic veins, left cephalic vein, right brachial right axillary vein, right radial and ulnar arteries. Bronchoscopy revealed diffuse alveolar hemorrhage. The following labs were sent and were all negative: DS DNA, Cardiolipin, Beta 2 glycoprotein, Phosphatidylserine, Smith AB, Myeloperoxidase AB, SS-A, SS-B, Atypical pANCA, Neutrophil Cytoplasmic Antibody, HIT Ab, Seratonin release assay, Viral Panel, Blood cultures. The patient was diagnosed with seronegative catastrophic antiphospholipid antibody syndrome. She improved significantly with anticoagulation, high dose steroids and renal replacement therapy. She continued to have vascular thrombosis events, and will need anticoagulation for life.
Discussion: Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare variant of a well known disease, and is a likely underreported cause of multiorgan failure. It is imperative for hospitalists to be aware of this disease entity as early recognition can prevent escalation to a higher level of care and decrease overall mortality. There is an international CAPS registry that has documented approximately 380 cases since its inception in 2000. CAPS has a multifactorial pathogenesis involving both innate and adaptive immunity, as well as a genetic predisposition for thrombosis. 58% of patients have primary Antiphospholipid syndrome, 27% have SLE, and 9% of CAPS patients have other underlying autoimmune diseases. Additionally a “second hit” (infectious, trauma, etc) is needed to incite the inflammatory reaction. The clinical guidelines for diagnosis of CAPS were recently updated in 2014. Diagnostic criteria include: Evidence of involvement of 3 organ systems, development of manifestations simultaneously, laboratory confirmation of the presence of Antiphospholipid antibody; and lastly, exclusion of other diagnosis. Definitive CAPS is defined as all 4 criteria. Probable CAPS is defined as: All 4 criteria, except for involvement of only 2 systems; meeting all criteria 1, 2, and 4 (seronegative CAPS) (the case in our patient) and meeting all criteria except having 3 organ systems affected over a one month period despite anticoagulation. Treatment is directed at control of the inflammatory response and management of thrombosis. Patients with combined therapy of anticoagulation, steroids, plasma exchange and IVIG have improved outcomes.
Conclusions: CAPS is a rare, rapid onset, severe disease and raising awareness among hospitalists can improve early recognition, and ultimately mortality. Additionally, hospitalists should be aware of the new 2014 CAPS diagnostic criteria as CAPS is likely underdiagnosed.
To cite this abstract:Donohue K, Tupe C. Catastrophic Antiphospholipid Antibody Syndrome. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 509. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/catastrophic-antiphospholipid-antibody-syndrome/. Accessed April 9, 2020.