THU0561 Clinical practice guideline for diagnosis and management of catastrophic antiphospholipid syndrome

Abstract

BackgroundCatastrophic antiphospholipid syndrome (CAPS) is characterized by the rapid onset of widespread or multifocal large and/or small vessel thrombosis associated with multi-organ failure in patients meeting the serological criteria for antiphospholipid syndrome [1]. Mortality in CAPS approaches 50% [2].ObjectivesThe RARE-BestPractices project group identified CAPS as a rare disease condition of interest in which to develop a clinical practice guideline. The project was run in partnership with McMaster University, and used the GIN-McMaster Guideline Development checklist and Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology to develop guidelines on rare diseases [3].MethodsThe CAPS guideline was coordinated by a steering committee including representatives from RARE-BP and methodologists from McMaster University. The CAPS guideline panel consisted of 19 international members, including patient representation. The panel used the GradePro software to brainstorm and prioritize potential questions and outcomes. Systematic reviews were performed for each question. To supplement the published evidence, we compiled raw data for mortality from the CAPS Registry, and systematically elicited expert opinion from the panel members using a systematic observation form. For each question an evidence profile and evidence to decision table was generated and shared.ResultsThe question prioritization step generated 47 questions, which were ranked to identify the top priorities. The top 10 questions were chosen for guideline development, yielding 7 therapy and 3 diagnostic questions. The outcome generation step yielded 7 outcomes.The questions were addressed during an in-person panel meeting, held on April 27, 2016 in Barcelona, Spain, with follow-up via webinar on June 3, 2016, and with web-based voting completed July 31, 2016.Recommendations were developed for all questions and will be discussed in detail.ConclusionsTen recommendations were issued by the CAPS Guideline Panel to assist clinicians in diagnosis and management of suspected CAPS patients. Future research is needed to improve evidence quality in rare diseases such as CAPS. The GIN-McMaster Guideline Development Checklist and the GRADE methodology were effective in producing a rigorous guideline in this rare disease.References Asherson R. The catastrophic antiphospholipid syndrome. J Rheumatol 1992.Cervera R, Bucciarelli S, Plasín M, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009;32:240–5.Guyatt G, Oxman A, Vist G, et al. GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 2008;336:924–6. Disclosure of InterestNone declared

16th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome

“…The guideline recommendations were presented initially at the European League against Rheumatism European Rheumatology Congress in June 2017, and the guideline methodology was presented at the American College of Rheumatology annual meeting in November 2017. 13 , 14 The complete guideline was published in the Journal of Thrombosis and Haemostasis in 2018. 7…”

Biological therapy in systemic lupus erythematosus, antiphospholipid syndrome, and Sjögren’s syndrome: evidence- and practice-based guidance

“…For patients with CAPS, acute management is based on a combined therapy using anticoagulation, corticosteroids, plasma exchange, and/or intravenous immunoglobulin administration and, in the case of CAPS initiated by an infectious event, systemic antibiotics (110,(113)(114)(115). Some recommendations suggest the use of rituximab for refractory APS patients (116), and rituximab or eculizumab for refractory CAPS (113,114,117).…”

“…Acute treatment with anticoagulants, corticosteroids, and IVIG is the current SoC of CAPS. The CAPS registry has shown significantly lower mortality among CAPS patients receiving combination therapy, compared with those receiving other treatments (odds ratio [OR], 0.51; 95% confidence interval [CI], 0.27, 0.95) (117).…”

“…Even though the paucity and equivocal evidence retrieved from publications between 2014 and 2019, IVIG has been used in the treatment of APS patients with recurrent thrombosis (118), obstetric APS (119-122), and CAPS (123). Usually administered at doses of 0.4 g/kg/day for 5 days, IVIG may be more useful in patients with thrombocytopenia and has the advantage of being immunomodulatory rather than immunosuppressive (117,118). However, IVIG was also associated with both increased thrombotic risk and worsening renal function, especially in elderly patients (117).…”

“…Usually administered at doses of 0.4 g/kg/day for 5 days, IVIG may be more useful in patients with thrombocytopenia and has the advantage of being immunomodulatory rather than immunosuppressive (117,118). However, IVIG was also associated with both increased thrombotic risk and worsening renal function, especially in elderly patients (117).…”