Raynaud’s phenomenon

Herrick, Ariane L.

doi:10.1177/2397198319826467

Cited by 22 publications

(23 citation statements)

References 93 publications

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“…Therefore, the optimal treatment frequency and duration is currently unknown. In current recommendations and expert reviews, TPE is not reported as a therapeutic modality (3, 16). This may be because TPE is not readily available at most centers and practitioners may be reluctant to consider it due to its invasiveness, sometimes requiring central venous access when peripheral access cannot readily be obtained.…”

Section: Discussionmentioning

confidence: 99%

Rheopheresis for Digital Ulcers and Raynaud's Phenomenon in Systemic Sclerosis Refractory to Conventional Treatments

et al. 2019

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Raynaud's phenomenon (RP) is almost universally present in patients with Systemic Sclerosis (SSc). RP represents a generalized vasculopathy and potentially lead to digital ulcers (DU), which may be complicated by superinfection, tissue necrosis, and limb loss. We report the analysis of an extracorporeal procedure in a 36-year-old female patient with diffuse SSc with refractory RP and DU despite treatment with diltiazem, candesartan, sildenafil, and intravenous iloprost. We performed rheopheresis (RheoP), a variant of double-filtration plasmapheresis, as a potential new treatment option for refractory patients despite optimal medical therapy. We performed two RheoP per week every 4 weeks for a total of 3 months. Clinical improvement in DU healing occurred with no adverse events directly related to the treatment. While there was no reduction in the number of Raynaud attacks with RheoP, a significant reduction of the duration of attacks from a median of 15 (5–45, 95% CI 10–15) to 7 (3–30, 95% CI 6–10) minutes with an improvement of the Raynaud Condition Score (RCS) improved from 4 to 2. In conclusion, RheoP is a feasible and potentially beneficial treatment modality in patients with refractory RP and DU. We propose that RheoP should be investigated in a larger number of patients in a clinical trial setting.

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Section: Discussionmentioning

confidence: 99%

Rheopheresis for Digital Ulcers and Raynaud's Phenomenon in Systemic Sclerosis Refractory to Conventional Treatments

et al. 2019

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“…Raynaud's phenomenon (RP), secondary to SSc, is the most frequent vascular manifestation in SSc patients. Secondary RP, the most common presenting feature of the disease, is observed in 95% of scleroderma patients and may precede diagnosis by many years [85][86][87][88]. During RP, the skin usually turns white (ischemia), blue (deoxygenation) and then red (reperfusion) [85][86][87][88].…”

Section: Peripheral Vascular Manifestationsmentioning

confidence: 99%

“…As there is an obliterative vasculopathy of the peripheral arteries and microcirculation in SRP, it often leads to critical ischemia in scleroderma. There is often a luminal narrowing of >75% of digital arteries due to underlying intimal fibrosis and luminal occlusion caused by thrombi [85][86][87][88]. Endothelial cell injury and activation lead to vascular dysfunction and vasospasm that may quickly obstruct the already limited blood flow of the vasculopathic digital arteries [41].…”

Section: Peripheral Vascular Manifestationsmentioning

confidence: 99%

“…Conversely, primary RP (PRP) is an isolated finding without underlying pathology (idiopathic). The suggested criteria for PRP include symmetric attacks, the absence of tissue necrosis, ulceration or gangrene, the absence of a secondary cause, negative tests for antinuclear antibodies and a normal erythrocyte sedimentation rate [85,86].…”

Section: Peripheral Vascular Manifestationsmentioning

confidence: 99%

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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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“…SSc and IIMs are characterized by the common presence of Raynaud's phenomenon (RP). This is a recurrent spasm of the terminal microcirculation, generally caused by a thermal cold shock, in which the fingers turn white (ischemic phase), blue (cyanotic phase) and red (revascularization phase) [6]. Nailfold videocapillaroscopy (NVC) is a useful, economic and rapid tool generally used for the diagnostic assessment of RP as the primary or associated with CTDs [7].…”

Section: Introductionmentioning

confidence: 99%

Nailfold Videocapillaroscopy Is a Useful Tool to Recognize Definite Forms of Systemic Sclerosis and Idiopathic Inflammatory Myositis in Interstitial Lung Disease Patients

Sambataro

Libra

et al. 2020

Diagnostics

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Nailfold videocapillaroscopy (NVC) is an easy tool used for the assessment of patients with Raynaud’s phenomenon (RP) as possibly associated with systemic sclerosis (SSc). Recent insights have also highlighted its role in the diagnostic assessment of idiopathic inflammatory myopathies (IIMs). The aim of this study is to describe the diagnostic role of NVC in a series of 361 consecutive patients with interstitial lung disease (ILD). All the patients were assessed by clinical pulmonary and rheumatic examinations, blood exams, high-resolution computed tomography and NVC. NVC was considered positive only in the presence of avascular areas or giant capillaries, but also, the presence of bushy capillaries (BCs) was recorded. NVC was positive in 17.7% of ILD patients and in 78.1% of ILD patients associated with a diagnosis of connective tissue disease (CTD). In 25% of SSc-ILD patients, NVC proved necessary for a correct diagnosis. The presence of BCs and/or NVC positivity in ILD patients with normal levels of creatine phosphokinase is associated with amyopathic IIM, regardless the presence of RP. In conclusion, NVC is useful for the diagnostic assessment of incomplete forms of CTD and in amyopathic IIMs. NVC should be considered in the diagnostic assessment of ILD patients regardless of the presence of RP.

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Raynaud’s phenomenon

Cited by 22 publications

References 93 publications

Rheopheresis for Digital Ulcers and Raynaud's Phenomenon in Systemic Sclerosis Refractory to Conventional Treatments

Rheopheresis for Digital Ulcers and Raynaud's Phenomenon in Systemic Sclerosis Refractory to Conventional Treatments

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

Nailfold Videocapillaroscopy Is a Useful Tool to Recognize Definite Forms of Systemic Sclerosis and Idiopathic Inflammatory Myositis in Interstitial Lung Disease Patients

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