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Abstract Number: 802

Prognostic Significance of Bicaudal D2 Antibodies in Sistemic Sclerosis (SSc) Patients

Giulia Segatto1, Chiara Bellocchi2, Gaia Montanelli1, Silvia Casas3, Karl Norvell4, Michelle Amino3, Fabrece Roup4, Michael Mahler4 and Lorenzo Beretta2, 1Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milano, Italy, 2Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 3Inova Diagnostics, San Diego, CA, 4Research and Development, Inova Diagnostics, San Diego, CA

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Autoantibodies and systemic sclerosis

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Session Information

Date: Sunday, October 21, 2018

Title: Systemic Sclerosis and Related Disorders – Clinical Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

Antibodies (Ab) toward Bicaudal D homolog 2 (BlCD2) can be found in 25% of Systemic Sclerosis (SSc) sera, mostly in association with anti-centromere (ACA) or anti-topoisomerase I (ATA) abs, but also in isolation. Anti-BlCD2 single specificity has been associated with myositis and interstitial lung disease (ILD), however its prognostic significance is unclear. A retrospective-prospective study was conducted to evaluate disease evolution in anti-BICD2+ patients.

Methods:

Serum samples from 288 SSc (ACR/EULAR 2013 criteria) collected between 2012 and 2014 were stored at -80° C and sent for Ab determination to Inova Diagnostics (San Diego, CA). Samples were tested using a novel particle-based multi-analyte technology (PMAT) and with CTD Essential (which includes DFS70, dsDNA, RNP, Sm, Ro60, Ro52, SS-B, Jo-1, Scl-70, Centromere, Ribo-P) and CTD Comprehensive (RNA Pol lll, Th/To, Ku, BICD2, PM/Scl; both test research use only).

Clinical data regarding major organ complications were retrospectively collected; time of evolution from baseline to new clinical manifestations was recorded. For dcSSc subjects evolutions included death, new digital ulcers, worsening of lung function (forced vital capacity, FVC loss > 10% or diffusing capacity for carbon monoxide, DLCO loss > 15%), progression of ILD on high resolution computed tomography, HRCT, development of pulmonary hypertension (PAH); evolution for lcSSc included progression of skin thickening as well as dcSSc categories; evolution for definite SSc without skin fibrosis included the appraisal of skin fibrosis and the above categories. Survival analysis for interval-censored data with 1.000-fold permutations was used.

Results:

Our cohort included: 30 (10.4%) definite non-cutaneous SSc, 216 (75%) lcSSc and 42 (14.5%) dcSSc, mostly females (95.5%), aged 60 ± 12.7 years and with disease duration of 14 ± 13 years. Anti-BlCD2 Ab were found in 49 subjects (17%), in 10 (3.5%) as a single-specificity, in 8 (2.8%) in associations with ATA, in 31 with ACA (10.8%). Patients were followed for a median of 4.0 (interquartile: 2 – 5.1) yrs until the first sign of evolution. Single anti-BlCD2 Ab positivity was associated with myositis (3/10 vs. 11/278, p=0.0089) and weakly with dcSSc (4/10 vs. 38/278, p=0.045). Ab specificity was associated with different pattern of progression (Figure): Anti-BlCD2+ patients had shorter evolution times compared to anti-BlCD2- patients (p=0.03), ACA+ subjects (p=0.007) but were similar to ATA+ subjects (p=0.2). Crude mortality trended to be higher in anti-BlCD2+ than anti-BlCD2- patients  (3/10 vs. 27/278, p=0.074).

Conclusion:

We confirm the association between anti-BlCD2 Ab and myositis in SSc and its overall prevalence. Anti-BlCD2+ patients have a worse prognosis and higher rates of disease progression compared to ACA+ subjects and behave similarly to ATA+ subjects.

 

 


Disclosure: G. Segatto, None; C. Bellocchi, None; G. Montanelli, None; S. Casas, Inova Diagnostics, 3; K. Norvell, Inova Diagnostics, 3; M. Amino, Inova Diagnostics, 3; F. Roup, Inova Diagnostics, 3; M. Mahler, Inova Diagnostics, 3; L. Beretta, EFPIA, 2.

To cite this abstract in AMA style:

Segatto G, Bellocchi C, Montanelli G, Casas S, Norvell K, Amino M, Roup F, Mahler M, Beretta L. Prognostic Significance of Bicaudal D2 Antibodies in Sistemic Sclerosis (SSc) Patients [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/prognostic-significance-of-bicaudal-d2-antibodies-in-sistemic-sclerosis-ssc-patients/. Accessed .
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