| | Author | Background | Result | Conclusion |
| | Aguiar and Erkan [4] | A stepwise approach for clinicians and researchers in the diagnosis of patients with CAPS | Development of algorithms for CAPS diagnosis in patients with and without history of APS or persistent APL positivity | It is critical to diagnose CAPS urgently when symptoms present, even without confirmatory APL tests | | Asherson et al. [7] | Consensus criteria for definition and classification of CAPS | Formulation of the preliminary criteria for classifying CAPS | Guidelines to define CAPS and guide multimodal treatment therapy; anticoagulation with survival benefit in treatment | | Cervera et al. [10] | Validation of the preliminary criteria for the classification of CAPS | Sensitivity, specificity, and positive and negative predictive values greater than 90% in classifying definite and probable CAPS | Use of the preliminary criteria for CAPS classification recommended | | Collier et al. [2] | Retrospective study of liver transplant recipients with postoperative hepatic vessel thrombosis from APS | The comparison of anticardiolipin IgG presence in recipients with hepatic vessel thrombosis and recipients without did not show statistical significance | The presence of IgG anticardiolipin is not associated with increased risk of hepatic vessel thrombosis in liver transplant recipients with APS. Routine screening is not warranted | | Erkan et al. [11] | Long-term follow-up for patients with CAPS after treatment | No recurrence of CAPS in patients on long-term high-intensity warfarin at 5.5 years; recurrence rate of 44-55% in untreated patients after first event | Long-term warfarin anticoagulation is recommended for long-term CAPS management | | Gologorsky et al. [12] | Lethal multisystem organ failure following CAPS after liver transplantation | Clinical manifestation likely from previously undiagnosed APS complicated by use of antifibrinolytic therapy during liver transplantation | Caution with use of synthetic antifibrinolytics in liver transplantation especially in patients with viral hepatitis and APS | | Kazzaz et al. [9] | Review of current approaches to diagnosis and treatment of CAPS | Anticoagulation and corticosteroids, IVIG, or plasma exchange recommended; cyclophosphamide use in patients with SLE | Triple therapy is marginally supported by retrospective data but recommended by most expert reviews | | Lockshin et al. [15] | Validation of the Sapporo criteria for the classification of APS | Sensitivity, specificity, and positive and negative predictive values were 0.71, 0.98, 0.95, and 0.88, respectively | The Sapporo criteria for APS are usable for clinical studies | | Miyakis et al. [16] | Update of the Sapporo criteria for the classification of APS | The laboratory criteria now require positive APL no less than 12 weeks apart vs. 6 weeks in previous criteria | Laboratory criteria was revised, use of “primary” and “secondary” APS was advised against, and CAPS was not discussed on here | | Obed et al. [24] | Case of ACLF and BCS treated with LDLT from donor with APS | Improvement in multisystem organ failure after LDLT and anticoagulation | In cases of APS+ donors with no clinical manifestation, LDLT is safe and feasible | | Reshetnyak et al. [5] | Liver transplantation in a patient with primary APS and BCS | Favorable outcomes following long-term use of dabigatran etexilate | Anticoagulation recommended for APS treatment | | Rodriguez-Pinto et al. [21] | Review of the current management approach for CAPS | Recommended treatment for CAPS is anticoagulation, glucocorticoids, and plasma exchange or IVIG. Rituximab and eculizumab for severe and refractory CAPS | Triple therapy is the current best therapeutic approach | | Rodriguez-Pinto et al. [22] | Clinical and immunologic manifestations of patients with CAPS | CAPS is majorly triggered by an event; kidneys most affected in multiorgan failure. Mortality rate is 37% | There are differences in CAPS patient presentation depending on age and presence of underlying chronic disease | | Steckelberg et al. [23] | Complication of HAT after liver transplant in a patient with APS and BCS | Favorable outcomes following retransplantation and long-term anticoagulation | Prophylactic anticoagulation may be beneficial in liver transplant candidates with history of previous thrombotic event to prevent posttransplant HAT | | Villamil et al. [6] | CAPS complicating orthotopic liver transplantation | CAPS causes multisystem organ failure. Anticoagulation plus steroid, plasmapheresis, and gamma globulin recommended | CAPS can be diagnosed when all other causes of multiorgan failure after transplant are ruled out | | Wilson et al. [14] | Consensus criteria for definition and classification of APS | Formulation of the Sapporo criteria for classifying APS | Definite APS is considered present when at least 1 of the clinical criteria and at least 1 of the laboratory criteria are met | | Yasutomi et al. [1] | APS-induced BCS in a 10-year-old child requiring liver transplant | Favorable outcomes following transplant and treatment with anticoagulation, steroids, and immunosuppression | Anticoagulation, steroids, and immunosuppression can be used in treatment of APS marked by a reduction in anticardiolipin antibody levels |
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CAPS: catastrophic antiphospholipid syndrome; APL: antiphospholipid antibodies; APS antiphospholipid syndrome; IgG: immunoglobulin G; IVIG: intravenous immunoglobulin; SLE: systemic lupus erythematosus; ACLF: acute on chronic liver failure; BCS: Budd-Chiari syndrome; LDLT: living donor liver transplant; HAT: hepatic artery thrombosis. Note: the data for Aguiar and Erkan is from Aguiar, C.L., and D. Erkan, Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease. Ther Adv Musculoskelet Dis, 2013. 5(6): p. 305-14. The data for Asherson et al. is from Asherson, R.A., et al., Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus, 2003. 12(7): p. 530-4. The data for Cervera et al. is from Cervera, R., et al., Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis, 2005. 64(8): p. 1205-9. The data for Collier et al. is from Collier, J.D., et al., Graft loss and the antiphospholipid syndrome following liver transplantation. J Hepatol, 1998. 29(6): p. 999-1003. The data for Erkan et al. is from Erkan, D., et al., Long term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis, 2003. 62(6): p. 530-3. The data for Gologorsky et al. is from Gologorsky, E., et al., Devastating intracardiac and aortic thrombosis: a case report of apparent catastrophic antiphospholipid syndrome during liver transplantation. J Clin Anesth, 2011. 23(5): p. 398-402. The data for Kazzaz et al. is from Kazzaz, N.M., W.J. McCune, and J.S. Knight, Treatment of catastrophic antiphospholipid syndrome. Curr Opin Rheumatol, 2016. 28(3): p. 218-27. The data for Lockshin et al. is from Lockshin, M.D., L.R. Sammaritano, and S. Schwartzman, Validation of the Sapporo criteria for antiphospholipid syndrome. Arthritis Rheum, 2000. 43(2): p. 440-3. The data for Obed et al. is from Obed, A., A. Bashir, and A. Jarrad, A case of live donor liver transplantation in acute-on-chronic liver failure with Budd-Chiari syndrome: donor and recipient with antiphospholipid antibody syndrome. Am J Case Rep, 2018. 19: p. 767-772. The data for Reshetnyak et al. is from Reshetnyak, T.M., et al., Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome. World J Hepatol, 2015. 7(19): p. 2229-36. The data for Rodriguez-Pinto et al. is from Rodriguez-Pinto, I., G. Espinosa, and R. Cervera, Catastrophic antiphospholipid syndrome: the current management approach. Best Pract Res Clin Rheumatol, 2016. 30(2): p. 239-249. The data for Rodriguez-Pinto et al. is from Rodriguez-Pinto, I., et al., Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev, 2016. 15(12): p. 1120-1124. The data for Steckelberg et al. is from Steckelberg, R.C., Z.D. Antongiorgi, and R.H. Steadman, Liver transplantation in a patient with antiphospholipid syndrome: a case report. A A Case Rep, 2017. 9(5): p. 148-150. The data for Villamil et al. is from Villamil, A., et al., Catastrophic antiphospholipid syndrome complicating orthotopic liver transplantation. Lupus, 2003. 12(2): p. 140-3. The data for Wilson et al. is from Wilson, W.A., et al., International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum, 1999. 42(7): p. 1309-11. The data for Yasutomi et al. is from Yasutomi, M., et al., Living donor liver transplantation for Budd-Chiari syndrome with inferior vena cava obstruction and assoiciated antiphospholipid antibody syndrome. J Pediatr Surg, 2001. 36(4): p. 659-62.
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