The role of ultrasound in systemic sclerosis: On the cutting edge to foster clinical and research advancement

Hughes, Michael; Bruni, Cosimo; Cuomo, Giovanna; Sedie, Andrea Delle; Gargani, Luna; Gutiérrez, Marwin; Lepri, Gemma; Ruaro, Barbara; Santiago, Tânia; Suliman, Yossra A; Watanabe, Shinji; Iagnocco, Annamaria; Fürst, Daniel E.; Bellando-Randone, Silvia

doi:10.1177/2397198320970394

Cited by 26 publications

(24 citation statements)

References 79 publications

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“…This may be due to the fact that MRI is capable of differentiating inflammation-predominant versus fibrosis-predominant lesions, offering information as to the choice for more anti-inflammatory or more anti-fibrotic targeting medications [ 68 ]. Moreover, ultrasound lung investigations are becoming widespread in the SSc-ILD field due to their potential for ILD screening [ 69 , 70 , 71 ]. Ultrasound correlates well with ILD extent and lung impairment and has a significant prognostic value in the evaluation of lung involvement, even if further studies are required to support the use of the technique [ 69 , 70 , 71 ].…”

Section: Imagingmentioning

confidence: 99%

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

et al. 2021

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Systemic sclerosis (SSc) is an autoimmune disease, characterized by the presence of generalized vasculopathy and tissue fibrosis. Collagen vascular disorder in SSc is due to fibroblast and endothelial cell dysfunctions. This leads to collagen overproduction, vascular impairment and immune system abnormalities and, in the last stage, multi-organ damage. Thus, to avoid organ damage, which has a poor prognosis, all patients should be carefully evaluated and followed. This is particularly important in the initial disease phase, so as to facilitate early identification of any organ involvement and to allow for appropriate therapy. Pulmonary disease in SSc mainly involves interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). High-resolution computed tomography (HRCT) and pulmonary function tests (PFT) have been proposed to monitor parenchymal damage. Although transthoracic echocardiography is the most commonly used screening tool for PAH in SSc patients, definitive diagnosis necessitates confirmation by right heart catheterization (RHC). Moreover, some studies have demonstrated that nailfold videocapillaroscopy (NVC) provides an accurate evaluation of the microvascular damage in SSc and is able to predict internal organ involvement, such as lung impairment. This review provides an overview of the correlation between lung damage and microvascular involvement in SSc patients.

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Section: Imagingmentioning

confidence: 99%

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

et al. 2021

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“…The assessment of skin involvement in SSc is not only pivotal in making an initial diagnosis, but also has a prognostic relevance, as there is an inverse correlation between the severity of skin involvement, survival and prognosis. [1][2][3][4][5][6] Indeed, quantifying skin impairment provides vital information, such as an assessment of disease activity and severity, an evaluation of therapy response and a management guide. [1][2][3][4][5][6] Systemic sclerosis skin impairment goes through three phases, ie, edematous, fibrotic and atrophic.…”

Section: Introductionmentioning

confidence: 99%

“…1,[7][8][9][10][11] The observation of skin manifestations is often used in clinical practice to classify the disease into two subsets, ie, limited or diffuse cutaneous skin involvement. [1][2][3][4][5] The damage observed in limited cutaneous skin involvement (lcSSc) is confined to the extremities, ie, the hands, forearms, feet, legs and face, whilst it is present on the arms, chest, abdomen and thighs in diffuse cutaneous skin involvement (dcSSc). [1][2][3][4][5] The mRSS is the only validated method able to recognise and study the skin manifestations and the skin involvement severity in SSc.…”

Section: Introductionmentioning

confidence: 99%

“…[1][2][3][4][5] The damage observed in limited cutaneous skin involvement (lcSSc) is confined to the extremities, ie, the hands, forearms, feet, legs and face, whilst it is present on the arms, chest, abdomen and thighs in diffuse cutaneous skin involvement (dcSSc). [1][2][3][4][5] The mRSS is the only validated method able to recognise and study the skin manifestations and the skin involvement severity in SSc. [1][2][3][4][5]12 Rodnan et al developed the original score in 1979 13 and it is used for the primary outcome in most clinical trials by the evaluation of skin thickness.…”

Section: Introductionmentioning

confidence: 99%

“…[1][2][3][4][5] The mRSS is the only validated method able to recognise and study the skin manifestations and the skin involvement severity in SSc. [1][2][3][4][5]12 Rodnan et al developed the original score in 1979 13 and it is used for the primary outcome in most clinical trials by the evaluation of skin thickness. It is feasible, reliable, valid and responsive to change in multicenter clinical trials, especially in dcSSc.…”

Section: Introductionmentioning

confidence: 99%

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The Updated Role of Ultrasound in Assessing Dermatological Manifestations in Systemic Sclerosis

Ruaro¹,

Santiago²,

Hughes³

et al. 2021

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Systemic sclerosis (SSc), an autoimmune connective tissue disease, characterized by skin fibrosis, increased dermal thickness and microvascular involvement. Fibroblasts and myofibroblasts deposit excessive amounts of collagenous and non-collagenous extracellular matrix components in the skin. This leads to microvascular abnormalities and Raynaud’s phenomenon, with painful digital ulcers (DU) at the fingertips adding to patient discomfort. The skin involvement and severity in SSc was evaluated by the Modified Rodnan skin score (mRSS). Although high-frequency ultrasound (HUS) has been widely researched in the study of skin thickness and DU in SSc, its adoption into clinical practice is not yet common. However, novel insights into the still relatively unknown disease pathogenesis in SSc and its evaluation may be provided by HUS, including early (pre-clinical) skin involvement. It may also be useful in both the evaluation and follow-up of DU. Indeed, it is a non-invasive, safe, inexpensive and reproducible method able to assess not only SSc patients’ cutaneous structural changes, but also their vascular system changes. Moreover, several recent studies have reported that elastosonography (ES) is of use when investigating skin involvement in systemic sclerosis. This review aims at providing information as to role HUS and ES play in research advancements and the clinical perspectives in the evaluation of skin thickness and DU in SSc patients.

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A Practical Approach to the Management of Digital Ulcers in Patients With Systemic Sclerosis

et al. 2021

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IMPORTANCE Digital ulcers (DUs) occurring on the fingers in patients with systemic sclerosis (SSc) are associated with substantial pain and disability and are often challenging to treat. However, careful clinical assessment and prompt intervention (wound bed management and systemic pharmacologic treatment) may modify the clinical course.OBJECTIVES To provide a practical approach to the assessment and management of SSc-DUs and highlight unmet needs and research priorities.EVIDENCE REVIEW A narrative review of the extant literature was undertaken to provide a broad overview of current knowledge and augmented by expert opinion.FINDINGS Half of the patients with SSc have a history of DUs, and there is a point of prevalence of approximately 10%. Digital ulcers are often very painful and affect all aspects of physical, social, and family life as well as occupation. Digital ulcers are associated with a severe disease course. Systemic sclerosis DUs, particularly those occurring on the fingertips, represent a vascular ischemic complication, although other etiopathogenic factors play an important role. To guide management, a structured clinical approach is required, including DU definition, classification, and categorization. Digital ulcers require a multidisciplinary approach with close cooperation between physicians and specialist nursing and other allied health professionals to guarantee the appropriate treatment and provide patient education. Local wound bed management is necessary for all DUs and is combined with systemic (pharmacologic) treatments. When treating a DU, the clinician should actively review the therapeutic strategy to prevent further DUs, including the level of systemic disease control, and monitor closely for the development of DU complications, including infection and progression to gangrene. Despite a wide available therapeutic armory, a number of unmet needs and challenges remain that that require resolution to optimize DU management. CONCLUSIONS AND RELEVANCEA practical approach to DU management, including local wound bed management and systemic treatments, is useful. Digital ulcers are of interest to a broad range of dermatologists, rheumatologists, and other physicians providing care for patients with SSc. Careful clinical assessment and prompt intervention can substantially modify the clinical course of DUs in SSc.

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The role of ultrasound in systemic sclerosis: On the cutting edge to foster clinical and research advancement

Cited by 26 publications

References 79 publications

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

The Updated Role of Ultrasound in Assessing Dermatological Manifestations in Systemic Sclerosis

A Practical Approach to the Management of Digital Ulcers in Patients With Systemic Sclerosis

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