1. University of Colorado School of Medicine Department of Rheumatology
A case of PAPS: Bled with Anti-coagulation, Infected with Immunosuppression and Ineffective IVIG
What’s Next?
Abo Baker Ahmd Mosa, MD
University of Colorado School of Medicine
Department of Rheumatology
Introduction
Case
Discussion
Our patient developed bacteremia and endocarditis with immunosuppression, her DAH persisted despite IVIG. Several case reports have indicated that treatment with eculizumab (monoclonal antibody against complement component C5) may be effective6,7,8,9, but the risk of infection, particularly meningitis are still there.
Diffuse alveolar hemorrhage (DAH) is a rare manifestation of primary antiphospholipid syndrome (PAPS)1,2,3,4. The general approach for treatment of DAH in the setting of PAPS is similar to that of catastrophic antiphospholipid syndrome (CAPS), including high dose steroids and in severe cases: PLEX, IVIG and/or Rituximab3,4.
A 35-year-old woman with PMH of 3 consecutive miscarriages was admitted for a syncopal event. Evaluation showed nonbacterial thrombotic endocarditis and she received porcine mitral valve prosthesis. Her hospital stay was complicated by subclavian and axillary vein thromboses as well as a PE. Hypercoagulability evaluation revealed strongly positive anti-cardiolipin and anti-B2 glycoprotein IgG antibodies. She was discharged on warfarin. Two weeks later she was admitted with DAH. Her anticoagulation was held, and she was treated with pulse dose steroids followed by prednisone taper. Off of warfarin, she suffered a left MCA stroke. Anticoagulation was thus resumed and she received a total of 3g of rituximab, azathioprine maintenance (2 mg/kg) and 4 cycles of monthly IVIG with persistently positive APL antibodies. After the third rituximab infusion, she presented with hemoptysis, fever and syncope. She was found to have DAH and Streptococcal bacteremia with prosthetic valve infectious endocarditis. She underwent a second mitral valve replacement and received a prolonged course of antibiotics.
Pathogenesis
Clinical Question
Should we proceed with Eculizumab, vaccination and prophylactic antibiotics or just continue IVIG and monitor?
References
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The proposed mechanism for the pathogenic effects of antiphospholipid antibodies on tissue injury through complements activation5.