Assessment, Definition, and Classification of Lower Limb Ulcers in Systemic Sclerosis: A Challenge for the Rheumatologist

Blagojević, Jelena; Piemonte, Guya; Benelli, Laura; Braschi, Francesca; Fiori, Ginevra; Bartoli, Francesca; Guiducci, Serena; Randone, Silvia Bellando; Galluccio, Felice; Cometi, Laura; Castellani, Sergio; Boddi, Maria; Pignone, Alberto Moggi; Rasero, Laura; Epifani, Francesco; Matucci‐Cerinic, Marco

doi:10.3899/jrheum.150035

Cited by 19 publications

(29 citation statements)

References 15 publications

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“…In this large multicentre study focusing on LLU in SSc patients, the most frequent LLU aetiology was venous insufficiency (49%); mixed arterial and venous aetiology accounted for 13% of cases, which is similar to what has already been reported in the literature (9,11,19).…”

Section: Discussionsupporting

confidence: 84%

“…One limitation of this study is its retrospective design and the recruitment of matched controls in a dermatology department, indicating a 12.8% LLU frequency in SSc patients. Another is the difficulty of classifying LLUs in SSc, notably microvascular LLUs that are defined in this study, as in previous ones, by the exclusion of other causes, without systematic assessment of toe pressure measurement (11).…”

Section: Discussionmentioning

confidence: 86%

“…Patients with mixed LLU (i.e. with both arterial and venous diseases) were classified as venous LLU in cases of non-significant arterial stenosis; (ii) LLU of predominant arterial macrovascular cause was defined by clinical findings consistent with peripheral arterial disease of the involved limb (intermittent claudication, rest pain or ankle-brachial pressure index (ABPI) < 0.7) and the presence of significant (> 50%) arterial stenosis in Doppler US and/or arteriography; (iii) LLU of microvascular origin was determined after excluding other causes, as already described in the literature (11,13): arterial macrovascular and venous causes were excluded by US Doppler; mechanical causes (traumatic or hyperkeratosis) were excluded by medical history; other causes were excluded by medical examination, with cutaneous biopsy if necessary (i.e. palpable or necrotic purpura) and electromyogram in cases of peripheral neuropathy symptoms; (iv) LLU related to other aetiologies (pyoderma gangrenosum, vasculitis, cancer) was defined by suggesting clinical findings with pathological confirmation.…”

Section: Significancementioning

confidence: 99%

“…Quantitative variables are expressed as medians (range) and qualitative variables as number (%). As we aimed to point out clinical factors relevant to severe prognosis, which has been shown in the literature to be related to ischaemia (9, 10), and as macrovascular LLU might also have a microvascular component (11), we pooled arterial macrovascular and microangiopathy-related LLUs into an "ischaemic LLU" group for comparative analyses that excluded the other LLU types.…”

Section: Statistical Analysesmentioning

confidence: 99%

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Lower-limb Ulcers in Systemic Sclerosis: A Multicentre Retrospective Case-control Study

Bohelay¹,

Blaise²,

Lévy³

et al. 2018

Acta Derm Venerol

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Lower-limb ulcers in systemic sclerosis patients are rarely reported. The aim of this study was to describe the main causes and outcomes of lower-limb ulcers in systemic sclerosis patients and to assess factors associated with ischaemic causes (arterial disease and/or microvascular impairment). A retrospective, multicentre, case-control study was conducted in 2013 and 2014, including 45 systemic sclerosis patients presenting lower-limb ulcers between 2008 and 2013. The estimated prevalence of lower-limb ulcers among systemic sclerosis patients was 12.8%. Ulcers were related to venous insufficiency in 22 cases (49%), ischaemic causes in 21 (47%) and other causes in 2 (4%). Complete healing was observed in 60% of cases in a mean time of 10.3 months; 59% relapsed during a mean follow-up of 22 months. Ischaemic lower-limb ulcer outcomes were poor, with a 28.6% amputation rate. Logistic-regression multivariate analyses between ischaemic lower-limb ulcer cases and matched systemic sclerosis-controls identified past or concomitant digital ulcer and cutaneous sclerosis of the feet as independent risk factors associated with ischaemic lower-limb ulcers.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 86%

Section: Significancementioning

confidence: 99%

Section: Statistical Analysesmentioning

confidence: 99%

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Lower-limb Ulcers in Systemic Sclerosis: A Multicentre Retrospective Case-control Study

Bohelay¹,

Blaise²,

Lévy³

et al. 2018

Acta Derm Venerol

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“…The etiology of lower extremity ulcers in scleroderma is often multifactorial(22). Scleroderma ulcers are bilateral in 70% of cases, and the most common histologic finding is fibrin occlusive vasculopathy with intimal thickening and some inflammation(21).…”

Section: Scleroderma and Mixed Connective Tissue Disease Associated Umentioning

confidence: 99%

Vasculitic and autoimmune wounds

Shanmugam

Angra

Rahimi

et al. 2017

Journal of Vascular Surgery: Venous and Lymphatic Disorders

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Chronic wounds are a major cause of morbidity and mortality. Approximately 20–23% of non-healing wounds that are refractory to vascular intervention have other etiologies including vasculitis, pyoderma gangrenosum and other autoimmune diseases. The purpose of this article is to review the autoimmune and coagulopathic diseases that are commonly associated with leg ulceration. OBJECTIVES To review the literature across medical and surgical specialties with regards to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions. METHODS An electronic search encompassing MEDLINE®, PubMed®, Cochrane Library and Scopus ® was completed using the following search terms: rheumatoid arthritis; systemic sclerosis; systemic lupus erythematosus; ANCA associated vasculitis; mixed connective tissue disease; antiphospholipid syndrome; pyoderma gangrenosum; thromboangiitis obliterans; cryoglobulinemia; hydroxyurea; sickle-cell; atrophie blanche; livedoid vasculitis; cholesterol emboli; calciphylaxis; antiphospholipid antibodies; prothrombotic; combined with the terms: chronic wound and leg ulcer. Full text articles published in English up to March 1, 2016 that investigated the clinical outcomes of chronic wounds associated with autoimmune diseases were included. Review articles and evaluations of management of chronic wounds were also reviewed. Primary outcomes included in the review were amputation, ulcer healing, reduction in wound size, overall survival and freedom from reintervention. Owing to the heterogeneity of data reporting among articles, qualitative analysis is also reported. CONCLUSIONS Autoimmune and vasculitic etiologies should be considered in patients with chronic wounds who do not respond to appropriate vascular intervention and standard local wound care. A multidisciplinary approach with the involvement of rheumatology allows investigation for underlying systemic disease and improves clinical outcomes for many of these challenging patients.

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Digital Ulcers

Hughes¹,

Bruni²

2020

In Clinical Practice

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Assessment, Definition, and Classification of Lower Limb Ulcers in Systemic Sclerosis: A Challenge for the Rheumatologist

Abstract: Results of our study indicate that lower limb ulcers in SSc often have a multifactorial pathogenesis that may be difficult to manage. Further studies are needed to validate the proposed classification and to assess the most appropriate management of lower limb ulcers in SSc.

Cited by 19 publications

References 15 publications

Lower-limb Ulcers in Systemic Sclerosis: A Multicentre Retrospective Case-control Study

Lower-limb Ulcers in Systemic Sclerosis: A Multicentre Retrospective Case-control Study

Vasculitic and autoimmune wounds

Digital Ulcers

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