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FRI0375 Clinical features of systemic sclerosis in patients with and without recurrent digital ulcers: findings from the duo registry
  1. M. Matucci-Cerinic1,
  2. T. Krieg2,
  3. L. Guillevin3,
  4. B. Schwierin4,
  5. M. Scott5,
  6. D. Rosenberg4,
  7. C. P. Denton6,
  8. The DUO Registry Group
  1. 1University of Florence, Florence, Italy
  2. 2University of Cologne, Cologne, Germany
  3. 3Hôpital Cochin, Paris, France
  4. 4Actelion Pharmaceuticals Ltd, Allschwil, Switzerland
  5. 5Numerus, Wokingham
  6. 6Royal Free & University College Medical School, London, United Kingdom

Abstract

Background Digital ulcers (DU) are painful and frequent in patients with systemic sclerosis (SSc), with ~30% of patients experiencing DU at least once a year.

Objectives This analysis describes SSc clinical features in patients with different types of DU recurrence.

Methods The DUO Registry is a European, prospective, observational cohort of SSc patients with DU. At enrolment, all have current and/or previous DU. Enrolled patients who had ≥2 follow-up (FU) visits and a 2-year observation period from Apr 2008 to Nov 2012 were categorised according to their DU recurrence pattern. Patients were categorized into 4 DU recurrence groups: non-active (no DU at FU visits and no new DU between visits), episodic (only 1 FU visit with a DU), recurrent (≥2 FU visits with DU and ≥1 visit with no DU) and chronic (DU present at every FU visit).

Results Of the 1273 patients with available data, 1056 (83%) were female, 459 (36.1%) were categorised as non-active, 274 (21.5%) as episodic, 398 (31.2%) as recurrent and 142 (11.1%) as chronic; the mean age (SD) at enrolment was 55.1 (13.8), 54.9 (13.5), 52.0 (14.3) and 51.8 (12.5) years in the non-active, episodic, recurrent and chronic groups, respectively. 465 (36.5%) patients had diffuse SSc: 140 (30.1%) non-active, 94 (20.2%) episodic, 161 (34.6%) recurrent, 70 (15.1%) chronic; 659 (51.8%) patients had limited SSc: 252 (38.2%) non-active, 150 (22.8%) episodic, 191 (29.0%) recurrent and 66 (10.0%) chronic. Anti-centromere antibodies were present in 50%, 41.1%, 40.1% and 29.9% of non-active, episodic, recurrent and chronic patients, respectively. 63.4% of the chronic and 51.1% of the recurrent patients had anti-Scl 70 antibodies, vs. 36.5% and 41.5% of non-active and episodic patients. Pulmonary arterial hypertension was present in 17.9% of non-active patients, vs. 10.6%, 15.1% and 7.7% of episodic, recurrent and chronic patients. Lung fibrosis was found in 36.8%, 36.1%, 41.7% and 52.1% of non-active, episodic, recurrent and chronic patients, respectively. Mean age at first DU (43.2 years, SD 14.6) and age at first Raynaud phenomenon (RP; 35.8 years, SD 14.0) were earliest in chronic patients. Median time from first RP to enrolment was 9.9, 10.6, 10.8 and 13.9 years in non-active, episodic, recurrent and chronic patients, respectively. Median time from first DU to enrolment was 4.2, 4.4, 4.3 and 6.4 years, respectively. At enrolment, 52.2% of the non-active patients had no DU, vs. 24%, 21.5% and 2.2% of the episodic, recurrent and chronic patients. Digital ischaemia, gangrene and soft tissue infection at enrolment were more frequent in recurrent and chronic patients.

Conclusions Frequency of SSc clinical features varies by type of DU recurrence; lung fibrosis prevalence increases from episodic to chronic. Patients with a chronic pattern of DU recurrence experience gangrene and infection most frequently. These data may imply that preventive management in SSc is needed to avoid DU complications and further analysis is needed.

Disclosure of Interest: M. Matucci-Cerinic Consultant for: Actelion Pharmaceuticals Ltd, Bristol-Meyers Squibb, Behring, Menarini, Speakers bureau: Britsol-Meyers Squibb, Abbot, Pfizer, Menarini, Actelion Pharmaceuticals Ltd, GlaxoSmithKline, T. Krieg Grant/research support from: Actelion Pharmaceuticals Ltd, Consultant for: Actelion Pharmaceuticals Ltd, L. Guillevin Consultant for: Actelion Pharmaceuticals Ltd, B. Schwierin Employee of: Actelion Pharmaceuticals Ltd, M. Scott Consultant for: Actelion Pharmaceuticals Ltd, D. Rosenberg Employee of: Actelion Pharmaceuticals Ltd, C. Denton Consultant for: Actelion Pharmaceuticals Ltd, Pfizer, GlaxoSmithKline, Digna, Sanofi-Aventis, Boehringer Ingleheim, Roche, CSL Behring

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