Innovations in the Assessment of Primary and Secondary Raynaud’s Phenomenon

Abstract

Objectives: Raynaud’s phenomenon (RP) is characterized by intense vasospasm of the digital arteries that causes characteristic color changes in fingers. There are two main types of RP: Primary RP (PRP) and Secondary RP (SRP). PRP is a benign condition. Whilst SRP is associated with several connective tissue diseases (CTD), in particular systemic sclerosis (SSc). The objectives of this report were: to present a short review on morphological (nailfold videocapillaroscopy, NVC) and functional techniques (laser tools and thermography) that allow for a correct diagnosis and treatment of RP and to investigate blood perfusion (BP) by laser speckle contrast analysis (LASCA) in different skin areas of hands and face in PRP, SRP to SSc, and healthy subjects (CNT). Methods: 31 PRP patients (LeRoy criteria), 70 SRP to SSc (ACR/EULAR criteria) and 68 CNT were enrolled. BP was assessed by LASCA at the level different areas of hands and face. NVC was performed to distinguish between PRP and SRP, and to detect the proper pattern of nailfold microangiopathy in SSc patients. Results: Both PRP and SRP showed a statistically significant lower BP than CNT at the level of fingertips ( p < 0.0001), periungual ( p < 0.0001), palmar aspect of 3rd finger ( p < 0.0001), and palm areas ( p < 0.0001). Moreover, BP was significantly lower in PRP than in SRP to SSc with the “Early” pattern of microangiopathy in the same areas as above ( p < 0.04). Conclusion: By considering a small cohort of patients, BP of hands was found lower in PRP than in SSc patients with the “Early” NVC pattern of microangiopathy.

Monitoring the microcirculation in the diagnosis and follow‐up of systemic sclerosis patients: Focus on pulmonary and peripheral vascular manifestations

“…Moreover, they reported a statistically significant negative correlation between the extent of the nailfold microangiopathy and the BP values at the level of the same skin areas in SSc patients. 3,45,59 Once again, peripheral BP negatively correlated to the extent of the microangiopathy, with the "Late" SSc NVC microangiopathy pattern, showing a lower blood flow than that observed in patients with "Active" or "Early" SSc patterns. 59 Lastly, LASCA may safely monitor DU evolution in SSc patients during standard treatment.…”

“…45,52 Laser speckle contrast analysis has been used in research studies on primary RP and SSc. 3,45 The authors of another study showed that LASCA assessed BP was significantly lower in SSc patients than in healthy subjects at the level of fingertips, periungual areas, and palm of hands. Moreover, they reported a statistically significant negative correlation between the extent of the nailfold microangiopathy and the BP values at the level of the same skin areas in SSc patients.…”

“…Several SSc studies have demonstrated that the microvascular involvement (assessed either qualitatively by NVC patterns or semiquantitatively by NVC scoring) is negatively correlated with skin blood perfusion (BP). 3,5,14,15,43,45 However, capillary blood flow/perfusion cannot be quantitatively measured by NVC under standard conditions, as only a qualitative evaluation, as regular, granulous, or stasis may be performed. 49 The direct/indirect assessment and the quantification of cutaneous BP in SSc may be performed by various laser techniques, thermography, and other emerging technologies (eg, optical Doppler tomography, spectroscopy, and photoplethysmography).…”

“…Systemic sclerosis (SSc) is a connective tissue disease with multi‐factorial etiology and autoimmune pathogenesis 1,2 . SSc is characterized by structural and functional alterations of the microcirculation, with serious clinical implications such as Raynaud's Phenomenon (RP), digital ulcers (DU) and pulmonary arterial hypertension (PAH), 1,3‐6 making morphological and functional assessment of the peripheral microvasculature a must for diagnosis, prognosis, and treatment response in SSc patients 7‐10 . Nailfold videocapillaroscopy (NVC) is a safe, noninvasive, and validated method capable of detecting morphological microvascular abnormalities and differentiating between secondary RP due to SSc, primary RP and healthy subjects 3,9,11,12 .…”

Recent advances in the workup and management of Raynaud’s phenomenon

“…45 Laser Doppler imaging, speckle contrast analysis, and speckle contrast imaging are characterized by a higher reliability, but they are more expensive and are not yet widely accessible. 46 The assessment of blood perfusion in RP may be also performed by hand perfusion scintigraphy. 47 This method was reported to play a role in the diagnosis of RP and evaluating the response to therapy.…”