Digital ulcers predict a worse disease course in patients with systemic sclerosis

Mihai, Carina; Landewé, Robert; Heijde, Desirée van der; Walker, Ulrich A.; Constantin, Paul I; Gherghe, Ana Maria; Ionescu, Ruxandra; Rednic, Simona; Allanore, Yannick; Avouac, Jérôme; Czirják, László; Hachulla, É.; Riemekasten, Gabriela; Cozzi, Franco; Airò, Paolo; Cutolo, Maurizio; Müeller-Ladner, Ulf; Matucci‐Cerinic, Marco

doi:10.1136/annrheumdis-2014-205897

Cited by 131 publications

(105 citation statements)

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“…A recent study by D’Alto et al [16] reported a significant worsening of sPAP (from 26.1 ± 6.0 to 28.8 ± 6.3) after a 3-year follow-up in a group of 74 consecutive SSc patients, in which IV iloprost was administered in 19% of cases. In the EUSTAR cohorts, where the treatment with IV iloprost is reported in 15.6% of cases [17], overall survival at 5 years is generally comprised between 80 and 90% [15, 18, 19]. Therefore, our results can be considered encouraging.…”

Section: Discussionmentioning

confidence: 57%

Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen

et al. 2016

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Intravenous iloprost is a first-line option for the treatment of scleroderma-related digital vasculopathy, and some studies have suggested its favourable role on disease progression. The aim of our study is to evaluate the disease progression, specifically in terms of cardiopulmonary function, in a group of consecutive patients chronically treated with intravenous iloprost. Our retrospective study enrolled 68 scleroderma patients (68 F, 54.4 ± 12.3 years) treated with iloprost for 7.1 ± 2.9 years, with a schedule of 5–6 consecutive daily infusions per month (6 h/day, 0.5–2.0 ng/kg/min). In all patients, modified Rodnan skin score (4.7 ± 5.3 vs. 3.7 ± 5.3, p < 0.0001), systolic pulmonary arterial pressure (sPAP) (30.9 ± 6.4 vs. 24.0 ± 3.2 mmHg, p < 0.0001), tricuspid annular plane systolic excursion (22.1 ± 2.4 vs. 23.8 ± 3.5 mm, p = 0.0001), pro-brain natriuretic peptide (97.2 ± 69.3 vs. 65.8 ± 31.7 pg/ml, p = 0.0005) showed statistically significant improvement from baseline. In the subgroup of patients with baseline sPAP ≥36 mmHg (n = 17), a significant sPAP reduction was observed (from 39.5 ± 3.8 to 25.1 ± 4.5 mmHg, p < 0.0001) after 7.6 ± 2.5 years of follow-up. The number of patients with digital ulcers (DUs) at follow-up was reduced from baseline (42.6 vs. 11.8%, p < 0.001), and none of the free-DU patients at baseline presented DUs at follow-up. An intensive and chronic regimen of IV iloprost administration seems to stabilize and potentially improve the long-term development of disease in SSc patients, as suggested by stabilization or significant improvement of cardiopulmonary parameters and vasculopathy.

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Section: Discussionmentioning

confidence: 57%

Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen

et al. 2016

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“…Nevertheless, the persistence of defined skin sclerosis is not consistent with inactive disease. Digital ulcers are clearly related to vascular disease activity and have been recently found to predict the occurrence of new digital ulcers during follow-up and to be associated with cardiovascular morbidity and decreased survival 35. A decreased DLCO can depend on both vascular and interstitial lung disease.…”

Section: Discussionmentioning

confidence: 99%

The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Valentini

Iudici

Walker³

et al. 2016

Ann Rheum Dis

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“…Digital ulcers are the consequence of an ischemic injury leading to necrosis of skin and subcutaneous tissues. The presence of DU is associated with increased burden and disability, reduced quality of life, impairment of daily activities, and decreased survival [5–7]. …”

Section: Introductionmentioning

confidence: 99%

Swift and Complete Healing of Digital Ulcers after Macitentan Treatment

Serret

2016

Case Reports in Rheumatology

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Digital ulcers are a burdensome and painful condition with sparse options of treatment. We report the case of a 78-year-old female patient with limited cutaneous systemic sclerosis that sequentially developed digital ulcers. After the appearance of digital ulcers in the soles of her feet she was successfully treated with bosentan. The report of two new digital ulcers in her hands 9 months later alongside with elevated transaminase levels led to a switch to macitentan treatment. A swift and complete healing of both digital ulcers was observed after 3 months, with the restoration of normal biochemical values.

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Digital ulcers predict a worse disease course in patients with systemic sclerosis

Abstract: In patients with SSc, HDU at presentation predicts the occurrence of DUs at follow-up and is associated with cardiovascular worsening and decreased survival.

Cited by 131 publications

References 17 publications

Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen

Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen

The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Swift and Complete Healing of Digital Ulcers after Macitentan Treatment

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