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Abo Baker Ahmd Mosa , MD University of Colorado School of Medicine Department of Rheumatology

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. Antiphospholipid Syndrome (APS). A systemic autoimmune disorder.

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Abo Baker Ahmd Mosa , MD University of Colorado School of Medicine Department of Rheumatology

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  1. PAPSBled with Anti-coagulation, Infected with Immunosuppression and Ineffective IVIG, What’s Next? Abo Baker AhmdMosa, MDUniversity of Colorado School of MedicineDepartment of Rheumatology

  2. Introduction

  3. Antiphospholipid Syndrome (APS) A systemic autoimmune disorder. Characterized by: Presence of antiphospholipid antibodies (aPL). Venous or arterial thrombosis. And/or pregnancy morbidity. Types: Primary Antiphospholipid Syndrome (PAPS). Secondary to other autoimmune disease (SLE). The mainstay of treatment is anticoagulation.

  4. CAPS(Catastrophic Antiphospholipid Syndrome) A rare, sudden onset and life-threatening complication of APLS1. Multiple thromboses of medium and small arteries occurring over a period of days2: Stroke, cardiac, hepatic, adrenal, renal, and intestinal infarctions. Peripheral gangrene.

  5. CAPS Treatment Early diagnosis is critical. Mortality rate 48%.  Addressing thrombosis and suppressing cytokine cascade. Treatment includes combination of 6: Anticoagulants (Possible bleeding complications). Systemic glucocorticoids. Plasma exchange and/or IVIG. In desperate situations: Cyclophosphamide or Rituximab. Not effectively studied due to it’s rarity.

  6. Case 35-year-old Hispanic woman. PMH of OSA, morbid obesity and hypertension . Also hx of brain aneurysm as incidental discovery during evaluation for MVA: Was told not to take blood thinners. She was on her usual state of health until: She developed worsening SOB and LL edema. She was found unconscious by her boyfriend.

  7. Fist Admission Echocardiogram: Mitral valve vegetations, likely bacterial endocarditis. Started on zosyn and vancomycin. Received porcine mitral valve prosthesis. Her recovery was complicated by: Pulmonary embolism. Subclavian and Axillary veins thrombosis. Infectious workup including Blood and valves cultures was negative.

  8. Pertinent work-up

  9. Fist Admission Cont. Thromboembolic work up revealed: Anti-phospholipid syndrome . No secondary cause identified. She was discharged on: Warfarin ( INR goal 2.5-3.5). Final diagnosis: Libman-Sacks endocarditis in the setting of PAPS.

  10. Second Admission Two weeks later, she developed: hemoptysis, SOB and low-grade fever. TEE: No acute abnormalities on prosthetic valve. CT-PE protocol: No embolism but left lower lob infiltrate. Bronchoscopy with BAL, consistent with DAH. She was started on pulse dose steroids followed by slow prednisone taper.

  11. Third Admission Two months later, she was admitted for: Persistent Hemoptysis and right side weakness. Wok up revealed left MCA stroke. Anticoagulation was resumed. Another pulse of steroids and prednisone taper. 2 g rituximab. 5 days of IVIG.

  12. Out-patient Management The patient received a total of: 3 g of rituximab. Azathioprine maintenance (2 mg/kg). 4 cycles of monthly IVIG. APL antibodies still positive.

  13. Fourth Admission After the third rituximab infusion. She developed: Hemoptysis, fever and syncope. She was found to have: DAH. Streptococcal bacteremia. Prosthetic valve endocarditis. Underwent a redo-mitral valve replacement. Received a prolonged course of Antibiotics.

  14. Summary A 35-year-old woman with PAPS: Recurrent bleeding with anticoagulation. Infection with immunosuppression: 3 rituximab infusions. Multiple rounds of high dose steroids. 3 round of monthly IVIG: Ineffective ?? High risk of thrombosis.

  15. Discussion 

  16. Diffuse Alveolar Hemorrhage (DAH) A rare manifestation of PAPS3,4,5,6. Treatment is generally similar to CAPS. Including high dose steroids. In severe cases: PLEX, IVIG and/or rituximab3,4.

  17. Pathogenesis (Complements involvement) The proposed mechanism of complements involvement in APLS pathogenesis7.

  18. Eculizumab (monoclonal antibody against C5) Several case reports have indicated the effectiveness of eculizumab in APLS8,9,10,11. In our patient: The risk of infection remains. Particularly meningitis with eculizumab.

  19. Clinical Question Should we proceed with eculizumab, vaccination and prophylactic antibiotics? OR Just continue IVIG, despite the higher risk of thrombosis?

  20. References Asherson RA, Cervera R, de Groot PG, et al: Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12: pp. 530-534.  Cervera R, Font J, Gomez-Puerta JA, et al: Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005; 64: pp. 1205-1209.  Koolaee RM, Moran AM, Shahane A. Diffuse alveolar hemorrhage and Libman-Sacks endocarditis as a manifestation of possible primary antiphospholipid syndrome. J ClinRheumatol. 2013 Mar; 19(2):79-83.  Bielski B, Shah NH, Westerveld D, Stalvey C. Diffuse alveolar hemorrhage and Libman-Sacks endocarditis: a rare presentation of antiphospholipid syndrome. BMJ Case Rep. 2018 May 7;2018.   Suzuki A, Asazuma N, Kikuchi E, Kawanobe T, Horimoto Y, Yokobari R, Kotake S, Okai T. Intern primary antiphospholipid syndrome" with concurrent diffuse alveolar hemorrhaging and Libman-Sacks endocarditis mimicking catastrophic antiphospholipid syndrome. Med. 2012; 51(7):813-6. Epub 2012 Apr. Yachoui R, Sehgal R, Amlani B, Goldberg JW. Antiphospholipid antibodies-associated diffuse alveolar hemorrhage. Semi Arthritis Rheum 2015 Jun; Epub 2014 Oct 31.    The antiphospholipid syndrome as a disorder initiated by inflammation: implications for the therapy of pregnant patients. Salmon JE, Girardi G, Lockshin MD. Nat clinRheumatol. 2007 Mar;3(3): 140-7; Quiz 1 p following 187.  Lonze BE, Singer AL, and Montgomery RA: Eculizumab and renal transplantation in a patient with CAPS. N Engl J Med 2010; 362: pp. 1744-1755.  Shapira I, Andrade D, Allen SL, et al: Brief report: induction of sustained remission in recurrent catastrophic antiphospholipid syndrome via inhibition of terminal complement with eculizumab. Arthritis Rheum 2012; 64: pp. 2719-2723. 14th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome. Cervera R, Rodríguez-PintóI, Colafrancesco S, Conti F, Valesini G, Rosário C, Agmon-Levin N, Shoenfel Y, Ferrão C, Faria R, Vasconcelos C, Signorelli F, Espinosa G. Autoimmun Rev. 2014 Jul;13(7):699-707. Epub 2014 Mar 20.   The Role of Complement Inhibition in Thrombotic Angiopathies and Antiphospholipid Syndrome. Erkan D, Salmon JE. Turk J Haematol. 2016;33(1):1.

  21. Thank you

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