AB0650 Thrombotic microangiopathy associated to anca-positives vasculitis: a french retrospective case control study and literature review. (12th June 2018)

• Record Type: Journal Article
• Authors: Dellal, A.; Boffa, Jean Jacques.; Rondeau, Eric.; Hatron, Pierre Yves.; Deligny, Christophe.; Bally, Stephane.; Fain, Olivier.; Coppo, Paul.; Mekinian, Arsène.
• Is Part Of: Annals of the rheumatic diseases. Volume 77(2018)Supplement 2
• Page Start: 1471
• Page End: 1471
• Publication Date: 2018-06-12
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-eular.7467
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objectives: In this large nationwide French case-control study, we describe the features of TTP and ANCA-positive vasculitis; compare to vasculitis without associated TTP; describe the outcome and treatments. Methods: We collected all cases with TTP and associated vasculitis. We conducted a literature review using PubMed, Web of Science, congress posters from January 2005 to August 2017 of PTT and ANCA-positive vasculitis. A control group of MPA without TTP during all the disease foillow-up was extracted from the Saint Antoine and Montfermeil Hospital patients with vasculitis. Firstly we compared our PTT cases and the literature review cases and secondary with a control group ANCA vasculitis without MAT. Results: 8 patients with MAT secondary to ANCA associated vasculitides were included in our French series 75% of Women with a median age 45 years, 21–76 positive ANCA in 50% and 37.5% is MPO, BVSA score at 16, FFS at 1. In 10 literature cases 90% Women, a median age 60 years [17–77], positive ANCA in 100% and 30% is MPO, BVSA score at 39, FFS at 1. The clinical features at the diagnosis of vasculitis were fever (n=5; 62%), ENT involvement (25%), kidney cresescnt glomerular involvment (n=6; 75%) with kidney failure in joint involvement (n=2; 25%) polyarthralgia type, with gastrointestinal involvement type mesenteric ischemia and pericarditis and lung involvement (nodules and alveolar haemorrhage) (n=1; 12.5%), in one case each Median C- reactive protein levels were at 49 mg/L [1–204], with creatininemia at 170 mg/dl [80–588]. ANCA were present in 4 patients (50%), MPO in 3 cases (37.5%). The time between the diagnosis of vasculitis and TTP was 9 months [0–51]. TTP features were: Hb at 7.8 g/dl [4.8–10], LDH 1658 [777–3110], platelets 33000 [3000–1 25 000], haptoglobin at 0 [0–0.05], creatinine at 205 [80–757]. ADAMTS 13 levels were at 10 [1–138], and x patients have normal ADAMTS13 levels, with anti ADAMTS 13 antibodies in 1 case. Plasma exchanges were done in all patients with median of 10 exchanges [4–23], 37.5% are dialyzed. Conclusions: In comparison to our cases, the literature patients have similar organs involvement, but median creatininemia levels and BVAS levels were higher in the literature cases. Considering TTP features, our cases have less frequent active vasculitis, less important creatinine levels and thus less recurs to kidney dialysis. In vasculitis associated with TTP, there was no significant differences in organ involvements, BVAS and FFS scales values, laboratory data and ANCA levels. Only creatininemia as expected was higer in vasculitis associated with TTP (225 Mmol vs 150 Mmol, p=0.0044). Disclosure of Interest: None declared