Catastrophic antiphospholipid syndrome (CAPS) – thrombotic microvasculopathy, characterized by involvement of
systems and organs with formation of their insufficiency. Therapy of glucocorticoids and immunosuppressants with plasmapheresis and intravenous immunoglobulin demonstrates lack of effectivity. Currently, there are reports of rituximab effectiveness in APS and CAPS, but they are not numerous, especially CAPS. Methods Assessment of the rituximab effectiveness in patients with resistant CAPS. We present the treatment results of two patients with CAPS, who were treated in our clinic

Klochko, V.

Savytskyi, V.

Tikhonchuk, N.

Vasylets, V.

Yakymenko, O.

Odessa National Medical University Institutional Repository

for reducing thrombotic risk and fatigue in jSLE patients. Fur-ther studies are needed to address this issue.P4 ANTIPHOSPHOLIPID SYNDROME IN PATIENTS WITHACUTE MYOCARDIAL INFARCTION1Mikel Jordhani, 2Dorina Ruci, 3Joana Seiti. 1Dept. Internal Medicine, Rheumatology Clinic,UHC Mother Teresa, Tirana; 2Dept. Internal Medicine, Rheumatology Clinic, UHC MotherTeresa, Tirana; 3Dept. Cardiovascular Diseases, Cardiovascular Reanimation Clinic, UHCMother Teresa, Tirana, Albania10.1136/lupus-2020-eurolupus.53Background Antiphospholipid Syndrome (APS) is a knowncause of thrombotic disorders, including Acute MyocardialInfarction (AMI). Although its incidence in AMI patients isnot known, it may be an important factor in precipitatinginfarction, especially in younger adults.Methods This is a case-control study consisting in 73 patientswith Acute Myocardial Infarction, hospitalized at Cardiovascu-lar Reanimation Clinic from 10 December 2015- 10 October2019. All patients included in the study were from 23 to 50years old. In the case-group were included 24 patients withAntiphospholipid Syndrome and Acute Myocardial Infarction,and 49 patients were included in the control group, whichconsisted only in patients with AMI, with no presence ofAPS. In every patient were gathered data such as completeblood count + ESR, Antinuclear Antibodies (ANA), Anti-cardi-olipin antibodies (ACA), C3 and C4 complement fraction,Anti-ds-DNA, CRP and Ejection Fraction (EF) in echocardiog-raphy. Female patients were asked about their abortion history.As recommended in the guidelines, in positive results for APS,ACA levels were repeated after 12 weeks in order to establishthe diagnosis. All data were gathered and statistically analyzedusing Excel 2010 and IBM SPSS.Results After comparing all gathered data, it was found thatthe patients with APS and AMI had a more significant ten-dency to have C3 hypocomplementemia (p=0.006), thrombo-cytopenia (p=0.002), a lower ejection fraction ontransthoracic echocardiography (p=0.04) and a more elevatednumber of abortions before acute myocardial infarction(p=0.03) in comparison to the controls.Conclusions From our study it was found that APS is not rarein young adults with AMI. It should be always suspected inyoung patients with no cardiovascular risk factors and theremay be a characteristic clinical and laboratory picture inpatients with AMI, which may suggest the APS diagnosis.Acknowledgements The authors declare no conflict of interest.P5 RECOGNIZING THE DISEASE, TREATING THE PATIENT: AMIXED-METHOD EVALUATION OF CARE FOR THEANTIPHOSPHOLIPID SYNDROME (APS) IN THENETHERLANDS1Mirthe J Klein Haneveld, 1Caro Lemmen, 1Tammo Brunekreef, 2Marc Bijl, 3Gerard Jansen,4Karina de Leeuw, 1Julia Spierings, 1Maarten Limper, on behalf of the ARCH study group.1Dept. of Rheumatology and Clinical Immunology, University Medical Centre Utrecht,Utrecht; 2Dept. of Internal Medicine and Rheumatology, Martini Hospital, Groningen; 3Dept.of Hematology, Erasmus MC, University Medical Center Rotterdam, Rotterdam; 4Dept. ofRheumatology and Clinical Immunology, University Medical Center Groningen, Groningen,The Netherlands10.1136/lupus-2020-eurolupus.54Purpose This study aims to gain insight into the care providedto patients with antiphospholipid syndrome (APS) in TheNetherlands and to identify areas for improvement from theperspective of both patients and clinicians.Methods APS care was evaluated using qualitative and quanti-tative methods. Perspectives on APS care were identified usingsemi-structured interviews with medical specialists, patientfocus groups and a cross-sectional, online patient survey. Inorder to examine differences in medical practice, medicalrecords were reviewed retrospectively regarding clinical andlaboratory manifestations, pharmacological treatment and man-agement of disease in six hospitals throughout TheNetherlands.Results Fourteen interviewed medical specialists, fourteen focusgroup participants and 79 survey respondents participated inthe study. 237 patients were included in medical recordreview. Only one-third of patients were diagnosed with APSwithin three months after entering specialist care. Diagnosisand management varied between centres and specialists.Almost 10% of triple positive patients did not receive anytreatment at the time of medical record review. Major chal-lenges according to specialists and patients were poor recogni-tion of APS by health care professionals, fragmentation ofcare and lack of accessible, reliable patient education and psy-chosocial support.Conclusion This study describes delayed diagnosis, variabilityin management strategies and a burden placed on patients toorchestrate their own care in APS care in The Netherlands.Of note, almost 10% of triple positive patients did not receiveany treatment, despite this group of patients being at thehighest risk of recurrent thrombosis.Acknowledgements This work was supported by the ArthritisResearch and Collaboration Hub (ARCH) Foundation.P6 RITUXIMAB EFFICACY IN CAPSOlena Yakymenko, Viktoria Vasylets, Viktor Klochko, Vladimir Savytskyi,Natalya Tikhonchuk. Rheumatological Dept., Multifield Medical Center of Odessa nationalmedical university, Odesa, Ukraine10.1136/lupus-2020-eurolupus.55Background Catastrophic antiphospholipid syndrome (CAPS) -thrombotic microvasculopathy, characterized by involvement ofsystems and organs with formation of their insufficiency. Ther-apy of glucocorticoids and immunosuppressants with plasma-pheresis and intravenous immunoglobulin demonstrates lack ofeffectivity. Currently, there are reports of rituximab effective-ness in APS and CAPS, but they are not numerous, especiallyCAPS.Methods Assessment of the rituximab effectiveness in patientswith resistant CAPS. We present the treatment results of twopatients with CAPS, who were treated in our clinic.Results Patient V., 21, was admitted in the early postpartumperiod. Diagnosis: Systemic lupus erythematosus, positivity forANA: ds-DNA, Sm. Secondary antiphospholipid syndrome(positivity for b2-glycoprotein-1, cardiolipin, lupus anticoagu-lant), catastrophic APS with multiorgan failure: epilepsy, sopor,psychosis; acute renal injury, signs of acute respiratory distresssyndrome and hypocoagulation. SLEDAI score was 56 points,GAPSS – 17 points. Pulse therapy with methylprednisolone,cyclophosphamide, plasmapheresis and intravenous immunoglo-bulin wasn’t effective enough. Rituximab course 375 mg/m2 1time per week for 4 weeks was administered. ManifestationsAbstractsLupus Science & Medicine 2020;7(Suppl 1):A1–A131 A31 on December 11, 2020 by guest. Protected by copyright.http://lupus.bmj.com/Lupus Sci Med: first published as 10.1136/lupus-2020-eurolupus.55 on 23 March 2020. Downloaded from of CAPS in multiorgan failure form regressed 14 days afterthe first administration, SLEDAI score decreased to 32 points,GAPSS to 4 points. After 4 weeks complete B-cell depletionwas achieved. A month after the course of rituximab sheachieved remission, which lasts 4 years already. ANA and anti-phospholipid antibodies weren’t detected.Patient E., 20, with primary APS, cardiolipin positivity, b2-glycoprotein-1 and lupus anticoagulant, thrombocytopenia,livedo reticularis; CAPS-like thrombotic microangiopathy typewith damage to cerebral vessels, lung vessels, recurrent pulmo-nary embolism for six months, deep leg vein thrombosis.GAPSS activity before treatment was 17 points. Pulse therapywasn’t performed. Therapy rituximab 375 mg/m2 1 time perweek, 4 weeks was administered. Multiple organ failure alsoregressed 10–14 days after 1 administration of rituximab.GAPSS score decreased to 10 points. Incomplete B-cell deple-tion was achieved.Conclusion Thus, rituximab demonstrated high effectiveness inCAPS in both cases. Rituximab allowed to reach multipleorgan failure regression and a persistent effect was achieved.P7 RIVAROXABAN MAY TRIGGER CATASTROPHICANTIPHOSPHOLIPID SYNDROME1Romain Stammler, 1Paul Legendre, 2Patrice Cacoub, 1Philippe Blanche, 2JeanCharles Piette, 1Nathalie Costedoat-Chalumeau. 1Internal Medicine Dept., AP-HP CochinHospital, Paris; 2Internal Medicine Dept., AP-HP La Pitié-Salpêtrière Hospital, Paris, France10.1136/lupus-2020-eurolupus.56Background Catastrophic antiphospholipid syndrome (CAPS)is the most severe complication of antiphospholipid syndrome(APS). Vitamin K antagonists (VKAs) are the reference treat-ment for preventing relapsing thrombotic complications inpatients with APS, but direct oral anticoagulants (DOACs),such as rivaroxaban, are nonetheless sometimes used inpatients with antiphospholipid antibody profiles or APS.Recent studies showed that DOACs were associated withmore arterial thromboses among patients with APS. Thepotential role of DOACs as a trigger factor of CAPS is notknown.Methods We describe two patients who developed a CAPS inthe week following the institution of rivaroxaban.Results We report the onset of definite CAPS in the weekfollowing introduction of rivaroxaban treatment in twopatients, one with APS and the other with antiphospholipidlaboratory findings only. Both were triple positive for anti-phospholipid antibodies. The affected organs were the heart,kidneys, skin, and liver for Patient 1, and the heart, kid-neys, skin, adrenal gland, and central nervous system forPatient 2. The causative role of rivaroxaban is highly prob-able given that (1) CAPS occurred rapidly after this treat-ment was started, (2) an alternative trigger factor was foundin Patient 1 only (a colonoscopy), and (3) Patient 1 hadbeen clinically stable for 18 years with VKA as anticoagu-lant treatment, while Patient 2 did not have APS and hadhad no symptoms for 4 months (rivaroxaban had beenintroduced because at a scheduled visit, she reported neuro-logical symptoms that occurred four months before andwere retrospectively compatible with a brain transient ische-mic accident). One similar case was reported in 2017, alsofollowing the introduction of rivaroxaban, in a patient withtriple positive venous thrombotic APS who was stable foryears on warfarin and who developed definite CAPS(involvement of the myocardium and adrenal glands as wellas a pulmonary embolism) in the week after rivaroxaban 20mg daily replaced warfarin to meet the patient‘s desire fora less burdensome treatment.1 Finally, in the randomizedstudy published by Ordi-Ros et al, one of the patientstreated with rivaroxaban developed a CAPS.2Conclusions These two cases, as well as two previous reportedcases, underline the importance of avoiding DOACs inpatients with APS, especially those triple positive for antiphos-pholipid antibodies. VKAs must remain the reference anticoa-gulation treatment in this setting.REFERENCES1. Crowley MP, Cuadrado MJ, Hunt BJ. Catastrophic antiphospholipid syndrome onswitching from warfarin to rivaroxaban. Thromb Res 2017; 153; 37–9.2. Ordi-Ros J, Saez-Comet L, Pérez-Conesa M, Vidal X, Riera-Mestre A, Castro-Sal-omo A, et al. Rivaroxaban versus vitamine K antagonist in antiphospholipid syn-drome: a randomized noninferiority trial. doi: 10.7326/M19-0291. [Epub ahead ofprint]; 2019P8 REFRACTORY THROMBOCYTOPENIA IN A PATIENT WITHSYSTEMIC LUPUS ERYTHEMATOSUS AND SECONDARYANTIPHOSPHOLIPID SYNDROME1,2Rita Torres, 1Maria João Gonçalves, 1,2Nuno Gonçalves, 1,2Santiago Manica,1,2Agna Neto, 1,2José Marona, 1Manuela Costa, 1,2Fernando M Pimentel-Santos, 1,2JaimeC Branco. 1Rheumatology Dept., Centro Hospitalar Lisboa Ocidental, Hospital de EgasMoniz, Lisboa; 2NOVA Medical School, Universidade NOVA de Lisboa, Lisboa, Portugal10.1136/lupus-2020-eurolupus.57Background Thrombocytopenia is a frequent hematologicalmanifestation in patients with systemic lupus erythematosus(SLE), usually treated with glucocorticoids, immunosuppres-sants (such as azathioprine and cyclophosphamide), intravenousimmunoglobulin (IVIG) or splenectomy.Case report A 44-year-old woman diagnosed with SLE andsecondary antiphospholipid antibody syndrome was admittedin our hospital, in July 2018, presenting severe renal and hae-matological involvement. She underwent treatment with meth-ylprednisolone, followed by prednisolone, and mycophenolatemofetil, with no response. In August 2018, the patient startedhaemodialysis, human immunoglobulin (transient response) andplasmapheresis due to evidence of secondary thromboticmicroangiopathy. The refractory severe thrombocytopaenia andlupus nephritis, justify two cycles of rituximab - platelet countrising lasted for five months. Seven months later, the patientpresented pancytopenia and due to high hemorrhagic risk,there was a switch from warfarin to LMWH (prophylacticdosing). One month later, anti-coagulation was suspended dueto spontaneous intracranial bleeding. In August 2019, thepatient had an ischemic stroke of the right occipital lobe, sec-ondary to APS, under prednisolone and hydroxychloroquine,and with platelet count of 24.000/uL. Because of the bleedingrisk, no anti-aggregation was instituted, and she started tacroli-mus - the initial good response disappeared two weeks later.After consulting a rheumatology board, tacrolimus was keptand rituximab repeated, regardless of the hypogammaglobuli-nemia and no lymphocytes CD19+ count.Conclusion We experienced a severe case of secondary throm-bocytopenia, that was refractory to multiple therapeutic agents.A reasonable response was obtained under rituximab. Tacroli-mus is kept due to the reported cases of late effect. Ischaemicstroke is a challenging condition in patients thrombocytopenicand further clinical guidance is warranted.AbstractsA32 Lupus Science & Medicine 2020;7(Suppl 1):A1–A131 on December 11, 2020 by guest. Protected by copyright.http://lupus.bmj.com/Lupus Sci Med: first published as 10.1136/lupus-2020-eurolupus.55 on 23 March 2020. Downloaded from 

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