FRI0683 Causes of death in 350 patients with systemic autoimmune rheumatic diseases (SARD)

Abstract

BackgroundThe major SARD have an increased mortality compared to the general population. It is well known that the main causes of death in Systemic Lupus Erythematosus (SLE) are infections (INF), cardiovascular events (CV), neoplasia (NEO) and disease activity. However, the compared mortality of Mixed Connective Tissue Disease (MCTD), Systemic Sclerosis (SSc), Poly/Dermatomyositis (PM/DM), overlap syndromes (OS), Sjögren's syndrome (SS), Antiphospholipid syndrome (APS), systemic vasculitis (SV), and undifferentiated or incomplete Connective Tissue Disease (UCTD) is poorly described.ObjectivesTo analyze the causes of death and the autoantibodies (AAB) profile among the SARD.MethodsThis was a single center, prospective and observational study. Mortality by all causes and relationship with AAB profile were analyzed in patients diagnosed of SLE, MCTD, SSc, PM/DM, OS (simultaneous or sequential criteria of 2 or more SARD), SS, APS, SV and UCTD or incomplete SARD (at least one clinical criterion of the classification criteria and a related antibody of any of the SARD). Data were obtained from the “Systemic Autoimmune Rheumatic Diseases Registry” of a tertiary referral hospital from 1986 to 2016. Patients with rheumatoid arthritis were excluded. The SARD registry counts with the institutional review board approval.Results1750 patients were included, of whom 1453 (83%) were women. Five hundred fifty six SLE, 125 SSc, 111 PM/DM, 91 OS, 90 MCTD, 250 SS, 71 APS, 211 SV, 117 UCTD and 128 losses to follow-up, the global follow up rate was 92.7%. A global mortality of 350 (20%) cases was observed: 101 INF (28,8%), 89 CV (25,4%), 51 NEO (14,5%), 45 due to disease activity (12,8%), 41 other causes (11,7%) and 23 from unknown causes (6,5%). Table 1 shows detailed mortality causes compared by diseases. A higher mortality was associated (p<0,05) with older patients (71 years, 20–96), SV (OR 3,65), male patients (OR 1,95), SSC/PM/DM (OR 1,76), MCTD (OR 1,6) and OS (OR 1,43). AAB to pANCA (OR 4,43), anti-topoisomerase I (OR 3,64), myositis-specific AAB (OR 3.0), cANCA (OR 2,19) and anticardiolipin (OR 1,89) were associated with poorer survival. A higher survival rate was observed in patients with SLE (OR 1,7), SS (OR 1,69) and UCTD (OR 15,57) (p<0,05).CAUSESSLE, %SSC, %MCTD, %SV, %PM/DM, %OS, %SS, %APS, % CV 30,86 27,02 42,3 25,5518,1816,1217,6421,4INF27,1621,623,84 36,66 33,33 45,16 23,5221,4NEO14,8113,5115,388,8818,189,67 26,47 14,28ACTIVITY9,8718,9119,2311,1112,1219,3511,76 28,57 OTHER8,6413,5111,5315,559,093,228,820.00UNKNOWN8,645,407,692,229,096,4511,7614,28ConclusionsThe main causes of death among SARD patients are CV (MCTD, SLE, and SSC), severe infections (OS, SV, and PM/DM), disease activity (APS) and neoplasia (SS). A higher mortality is observed among ANCA positive SV, anti-topoisomerase I positive SSC, MCTD, OS, anticardiolipin and myositis-specific positive patients.Disclosure of InterestNone declared

Practical approach to monitoring and prevention of infectious complications associated with systemic corticosteroids, antimetabolites, cyclosporine, and cyclophosphamide in nonmalignant hematologic diseases

“…However, publications about patients with ITP or autoimmune rheumatologic disease undergoing immunosuppressive therapies report infection as a major cause of morbidity, treatment interruption, and/or discontinuation. 1,2 When appraising the patient's risk of infection, the interactions of various endogenous and exogenous risk factors have to be considered: (1) nonmalignant immune-mediated hematologic diseases are themselves chronic conditions driven by an already dysfunctional immune system; (2) a patient's individual characteristics, such as advanced age (>65 years old), presence of preexisting comorbidities, polypharmacy (ie, ≥5 medications used daily), and risk of drug-drug interaction; the patient's compliance with the immunosuppressive therapy and dose adjustments; compliance and accessibility to clinical and laboratory monitoring; and a patient's rapid response to suspected infections; (3) specific immunosuppressive agents have particular mechanisms of causing immunosuppression, leading to an increased risk for infection with certain pathogens (eg, antimetabolites and risk for progressive multifocal leukoencephalopathy [PML]); and (4) higher risk for infection is seen in combination immunosuppressive therapy as opposed to singleagent therapy (Table 1). [2][3][4][5][6] It is a challenge for the clinician to estimate the contribution of each of these risk factors to the overall infection risk because no study has addressed this clinical issue.…”