PMID- 30146007 OWN - NLM STAT- MEDLINE DCOM- 20191002 LR - 20191007 IS - 1095-9157 (Electronic) IS - 0896-8411 (Linking) VI - 93 DP - 2018 Sep TI - Catastrophic antiphospholipid syndrome: Lessons from 14 cases successfully treated in a single center. A narrative report. PG - 124-130 LID - S0896-8411(18)30278-6 [pii] LID - 10.1016/j.jaut.2018.07.001 [doi] AB - The study aimed to evaluate the clinical significance of laboratory findings in patients with catastrophic antiphospholipid syndrome (CAPS) and to report the effects of a well-defined treatment protocol in 14 consecutive cases. Thirteen patients (12 presenting one and one presenting two episodes of CAPS) were consecutively treated and monitored between 1986 and 2017. Antiphospholipid antibody (aPL) characteristics of the patients were compared with those of 64 matched controls (45 antiphospholipid syndrome patients and 19 aPL carriers) who did not develop CAPS during the same mean follow-up period (12 years +/- 9.9 SD). Triple aPL positivity (IgG/IgM anticardiolipin + IgG/IgM anti-beta2Glycoprotein I + lupus anticoagulants) significantly prevailed in the CAPS patients with respect to the controls (p = 0.003). IgG anticardiolipin and IgG anti-beta2Glycoprotein I mean antibody titers of the CAPS patients were significantly higher than those of the controls (p = 0.0018 and p = 0.003, respectively). Triple therapy (anticoagulation + plasma exchange + steroids) was administered to all the CAPS cases except for one. Beginning in 2009, intravenous immunoglobulin infusion has also been included in the triple therapy protocol (six patients). All the patients recovered from CAPS; five showed renal failure and one a I-II class New York Heart Association (NYHA) dilated cardiomyopathy. Long-term outcomes of CAPS included a gradual worsening of renal failure in one patient who required hemodialysis 30 years after the acute episode. Renal function improved in the other four patients. The patient affected with dilated cardiomyopathy worsened to a II class NYHA over a five year period. Currently all the patients are alive. A specific antiphospholipid antibody profile could be considered a risk factor associated to CAPS. Early use of a defined treatment protocol based on triple therapy either or not associated with IVIG was associated with recovery in all CAPS patients. CI - Copyright (c) 2018 Elsevier Ltd. All rights reserved. FAU - Ruffatti, Amelia AU - Ruffatti A AD - Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy. Electronic address: amelia.ruffatti@unipd.it. FAU - De Silvestro, Giustina AU - De Silvestro G AD - Apheresis Unit, Blood Transfusion Service, University-Hospital of Padua, Padua, Italy. FAU - Marson, Piero AU - Marson P AD - Apheresis Unit, Blood Transfusion Service, University-Hospital of Padua, Padua, Italy. FAU - Tonello, Marta AU - Tonello M AD - Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy. FAU - Calligaro, Antonia AU - Calligaro A AD - Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy. FAU - Favaro, Maria AU - Favaro M AD - Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy. FAU - Del Ross, Teresa AU - Del Ross T AD - Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy. FAU - Hoxha, Ariela AU - Hoxha A AD - Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy. FAU - Mattia, Elena AU - Mattia E AD - Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy. FAU - Pengo, Vittorio AU - Pengo V AD - Cardiology Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy. LA - eng PT - Journal Article DEP - 20180707 PL - England TA - J Autoimmun JT - Journal of autoimmunity JID - 8812164 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Anticoagulants) RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin M) RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Lupus Coagulation Inhibitor) RN - 0 (beta 2-Glycoprotein I) SB - IM MH - Adolescent MH - Adrenal Cortex Hormones/*therapeutic use MH - Adult MH - Antibodies, Anticardiolipin/blood MH - Antibodies, Antiphospholipid/blood MH - Anticoagulants/*therapeutic use MH - Antiphospholipid Syndrome/diagnosis/immunology/pathology/*therapy MH - Case-Control Studies MH - Catastrophic Illness MH - Female MH - Follow-Up Studies MH - Humans MH - Immunoglobulin G/blood MH - Immunoglobulin M/blood MH - Immunoglobulins, Intravenous/*therapeutic use MH - Lupus Coagulation Inhibitor/blood MH - Male MH - Middle Aged MH - Plasma Exchange/*methods MH - Risk Factors MH - Treatment Outcome MH - beta 2-Glycoprotein I/antagonists & inhibitors/genetics/immunology OTO - NOTNLM OT - Anticoagulant drugs OT - Antiphospholipid antibodies OT - Catastrophic antiphospholipid syndrome OT - Corticosteroids OT - Intravenous immunoglobulins OT - Plasma exchange EDAT- 2018/08/28 06:00 MHDA- 2019/10/08 06:00 CRDT- 2018/08/28 06:00 PHST- 2018/05/17 00:00 [received] PHST- 2018/06/30 00:00 [revised] PHST- 2018/07/01 00:00 [accepted] PHST- 2018/08/28 06:00 [entrez] PHST- 2018/08/28 06:00 [pubmed] PHST- 2019/10/08 06:00 [medline] AID - S0896-8411(18)30278-6 [pii] AID - 10.1016/j.jaut.2018.07.001 [doi] PST - ppublish SO - J Autoimmun. 2018 Sep;93:124-130. doi: 10.1016/j.jaut.2018.07.001. Epub 2018 Jul 7.