Background Raynaud's phenomenon (RP) is a nearly universal manifestation of systemic sclerosis (SSc) inducing the development of digital ulcers (DUs) in about 30% of these patients each year. Moreover, DUs can further escalate, causing substantial morbidity and complications including gangrene.
Objectives To determine the characteristics of SSc-DU patients who have past and/or current gangrene, and the incidence of new gangrene in these patients.
Methods The DUO Registry is a European, prospective, multicentre, observational, registry of SSc patients with DU disease, irrespective of treatment regimen. Data collected included demographics, SSc duration, SSc subset, internal organ manifestations, autoantibodies, previous and ongoing interventions and complications related to DUs. We have compared patients who developed gangrene at the time of inclusion (current gangrene) or in the past (past gangrene) compared to those who have never had gangrene (no past/current gangrene).
Results From April 2008 to June 2014, a total of 4534 patients had been enrolled into the registry. Within this cohort, 243 (5%) had current gangrene at the time of inclusion, 808 (18%) had current/past gangrene and 3417 (75%) had no current/past gangrene. There was no difference regarding RP duration or SSc duration between these groups. The three groups did not differ with regards to cutaneous subsets, auto-antibody profile, smoking, or major organ involvement. Compared with patients with no past/current gangrene, a higher proportion patients with current gangrene had at least one DU (95% vs 57%) and also critical digital ischemia (78% vs14%). The onerous burden of gangrene is further illustrated in Table 1 providing data about hospitalization, medical and surgical procedures and patient need for help from others due to the ulcers of the fingers or related symptoms. Out of 3611 patients with at least one follow-up visit, 286 developed incident gangrene during follow-up; this preliminary analysis shows that past gangrene is associated with the development of incident gangrene (gangrene before enrolment in 53% of incident cases vs 15% in patients free of new gangrene episode).
Conclusions The DUO Registry is a post-approval observational study which has included the largest ever-assembled cohort of patients with SSc and DU disease. Among them, gangrene appears to be a not infrequent complication; it is not associated with a well-defined subset but leads to a very heavy burden often requiring hospital-based management. Further analyses are now ongoing to clarify the risk factors for recurrence of gangrene.
Disclosure of Interest Y. Allanore Grant/research support from: Actelion, Bayer, Biogen Idec, BMS, Genentech/Roche, Inventive, Medac, Pfizer, Sanofi/Genzyme, Servier and UCB, Consultant for: Actelion, Bayer, Biogen Idec, BMS, Genentech/Roche, Inventive, Medac, Pfizer, Sanofi/Genzyme, Servier and UCB, C. Denton Grant/research support from: Actelion, CSL Behring, Novartis, Consultant for: Actelion, GSK, Bayer, Inventiva, Takeda, Speakers bureau: Actelion, GSK, Bayer, Inventiva, Takeda, T. Krieg Grant/research support from: Actelion, P. Cornelisse Employee of: Actelion Pharamaceuticals Ltd, D. Rosenberg Employee of: Actelion Pharamaceuticals Ltd, B. Schwierin Employee of: Actelion Pharamaceuticals Ltd, M. Matucci-Cerinic Grant/research support from: Actelion, Pfizer, GSK, BMS, Abbot, Speakers bureau: Actelion, Pfizer, GSK, BMS, Abbot