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THU0415 Anti-pm/scl antibody clinical associations in patients with systemic sclerosis. analysis of the multicenter eustar cohort
  1. M.G. Lazzaroni1,
  2. A. Furloni1,
  3. I. Cavazzana1,
  4. S. Zingarelli1,
  5. F. Franceschini1,
  6. A. Tincani1,
  7. S. Bellando-Randone2,
  8. C. Caimmi3,
  9. E. Hachulla4,
  10. Y. Allanore5,
  11. P. Airo’1,
  12. on behalf of EUSTAR co-authors
  1. 1University and Spedali Civili of Brescia, Brescia
  2. 2University of Florence and Division of Rheumatology AOUC, Firenze
  3. 3Azienda Ospedaliera Universitaria Integrata, Verona, Italy
  4. 4University Lille Nord-de-France, Lille
  5. 5University Paris Descartes and Cochin Hospital, Paris, France


Background Antibodies to the PM/Scl complex are found in patients with Systemic Sclerosis (SSc), but also with Polymyositis, Dermatomyositis and overlap syndromes. Historically, the main clinical association of anti-PM/Scl antibodies in SSc, which include calcinosis, articular and muscle involvement, and interstitial lung diseases, were described by some large single-centre studies, or by multicenter studies which recruited a relatively small number of positive patients. Therefore, some unresolved issue deserves further research. In particular, scleroderma renal crisis was recently identified in a sizeable number of anti-PM/Scl+SSc patients in a large monocentric British cohort (4 out of 70; 5.7%), a somewhat unexpected finding since this antibody type is generally considered not to be associated with renal crisis.1

Objectives To evaluate clinical associations of anti-PM/Scl in patients with SSc in the large multicenter EUSTAR database, with specific focus on scleroderma renal crisis.

Methods Patients from the EUSTAR database were included when the item anti-PM/Scl was fulfilled in at least one visit; clinical data were collected from the last visit available.

Results Anti-PM/Scl status was available in 8,287 SSc patients from EUSTAR database: 295 were anti-PM/Scl +. After exclusion of 145 patients with multiple autoantibody positivity, 150 anti-PM/Scl+patients were compared with 7992 anti-PM/Scl -negative patients. Among these, 2530 were positive for anticentromere, 1933 for anti-topoisomerase I, 186 for anti-RNA polymerase III, and 220 for anti-U1Rnp antibodies.

Renal crisis was identified in 8 of 150 anti-PM/Scl+SSc patients (5.3%), and was significantly more frequent than in anti-PM/Scl-negative SSc patients (1.6%; p=0.0015). Positivity for anti-PM/Scl was also associated with male sex, diffuse cutaneous subsets, joint and muscle involvement, lung fibrosis at chest X-rays, heart conduction blocks, stomach and intestinal symptoms (table 1). However, in multivariate analysis, adjusted for age at disease onset, sex, and disease duration, the association of anti-PM/Scl with renal crisis was not significant, whereas the associations with joint and muscle involvement, lung fibrosis, and intestinal symptoms were confirmed (table 1).

Table 1 Results of the univariable and multivariable analysis adjusted on sex, age at disease onset and disease duration (n=8142 patients). Results are presented as number/number available data (%) unless stated otherwise.

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Conclusions In the largest series of anti-PM-Scl positive patients so far reported, well-known clinical associations were confirmed. Moreover, scleroderma renal crisis was more frequent than in the antibody-negative patient controls (which included a majority of anticentromere-positive patients, and a relatively small number of anti-RNA polymerase III-positive patients). However, this association was probably explained by covariates, such as joint and muscle involvement, or lung fibrosis. A possible role of corticosteroid therapy might therefore be suspected.

Reference [1] Bruni C, et al. Rheumatology (Oxford). 2017;56:317–8.

Acknowledgements Authors would like to thank the non-profit organisation ‘Gruppo Italiano Lotta alla Sclerodermia’ (GILS) for its substantial grant for this research project.

Disclosure of Interest None declared

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