The role of capillaroscopy and thermography in the assessment and management of Raynaud's phenomenon.

Most patients with Raynaud's phenomenon (RP) have "benign" primary RP (PRP), but a minority have an underlying cause, for example a connective tissue disease such as systemic sclerosis (SSc). Secondary RP can be associated with structural as well as functional digital vascular changes and can be very severe, potentially progressing to digital ulceration or gangrene. The first step in management is to establish why the patient has RP. This short review discusses the role of nailfold capillaroscopy and thermography in the assessment of RP. Nailfold capillaroscopy examines microvascular structure, which is normal in PRP but abnormal in most patients with SSc: the inclusion of abnormal nailfold capillaries into the 2013 classification criteria for SSc behoves clinicians diagnosing connective tissue disease to be familiar with the technique. For those without access to the gold standard of high magnification videocapillaroscopy, a low magnification dermatoscope or USB microscope can be used. Thermography measures surface temperature and is therefore an indirect measure of blood blow, assessing digital vascular function (abnormal in both PRP and SSc). Until now, the use of thermography has been mainly confined to specialist centres and used mainly in research: this may change with development of mobile phone thermography.

Successful treatment of Raynaud's phenomenon and digital ulcers in systemic sclerosis patients with botulinum toxin B injection: Assessment of peripheral vascular disorder by angiography and dermoscopic image of nail fold capillary.

We recently identified the efficacy and safety of a botulinum toxin (BTX)-A/B in Raynaud's phenomenon (RP) and digital ulcers (DU) in Japanese patients with systemic sclerosis (SSc). Detailed assessments of peripheral vascular disorder using angiography and dermoscopic images of nail fold capillaries have not been performed previously. This study aimed to evaluate the effect of BTX-B on SSc-associated peripheral vascular disorder. Two SSc patients who suffered with RP and DU were treated with a BTX-B injection, and thereafter the symptoms of RP were improved and DU healed in both patients. Furthermore, angiography showed an increased blood flow to the palm and fingers, and dermoscopic images of nail fold capillary changes showed improvement. These results suggest that a BTX-B injection may increase peripheral blood flow and improve RP and DU in SSc patients.

Assessment of treatment effects on digital ulcer and blood perfusion by laser speckle contrast analysis in a patient affected by systemic sclerosis.

Laser speckle contrast analysis (LASCA) is a good tool to evaluate the variation in peripheral blood perfusion during long-term follow-up and is able to safely monitor digital ulcer evolution in scleroderma patients. It evaluates blood perfusion in different areas within the skin lesions and surrounding them during standard treatment.

The Place of Nailfold Capillaroscopy Among Instrumental Methods for Assessment of Some Peripheral Ischaemic Syndromes in Rheumatology.

Micro- and macrovascular pathology is a frequent finding in a number of common rheumatic diseases. Secondary Raynaud's phenomenon (RP) is among the most common symptoms in systemic sclerosis and several other systemic autoimmune diseases including a broad differential diagnosis. It should be also differential from other peripheral vascular syndromes such as embolism, thrombosis, etc., some of which lead to clinical manifestation of the blue toe syndrome. The current review discusses the instrumental methods for vascular assessments. Nailfold capillaroscopy is the only method among the imaging techniques that can be used for morphological assessment of the nutritive capillaries in the nailfold area. Laser-Doppler flowmetry and laser-Doppler imaging are methods for functional assessment of microcirculation, while thermography and plethysmography reflect both blood flow in peripheral arteries and microcirculation. Doppler ultrasound and angiography visualize peripheral arteries. The choice of the appropriate instrumental method is guided by the clinical presentation. The main role of capillaroscopy is to provide differential diagnosis between primary and secondary RP. In rheumatology, capillaroscopic changes in systemic sclerosis have been recently defined as diagnostic. The appearance of abnormal capillaroscopic pattern inherits high positive predictive value for the development of a connective tissue disease that is higher than the predictive value of antinuclear antibodies. In cases of abrupt onset of peripheral ischaemia, clinical signs of critical ischaemia, unilateral or lower limb involvement, Doppler ultrasound and angiography are indicated. The most common causes for such clinical picture that may be referred to rheumatologic consultation are the antiphospholipid syndrome, mimickers of vasculitides such as atherosclerosis with cholesterol emboli, and neoplasms.

Comparison of laser Doppler imaging, fingertip lacticemy test, and nailfold capillaroscopy for assessment of digital microcirculation in systemic sclerosis.

INTRODUCTION: Laser Doppler imaging (LDI) is a relatively new method for assessing the functional aspect of superficial skin blood flow in systemic sclerosis (SSc) and Raynaud's phenomenon. The present study investigated the dynamic behavior of digital skin microvascular blood flow before and after cold stimulus (CS) in SSc patients and in healthy controls by means of a comprehensive approach of the functional (LDI), morphological (nailfold capillaroscopy (NFC)), and biochemical (fingertip lacticemy (FTL)) microcirculation components. METHODS: Forty-four SSc patients and 40 healthy controls were included. After acclimatization, all subjects underwent NFC followed by LDI and FTL measurement. NFC was performed with a stereomicroscope under 10× to 20× magnification in the 10 digits of the hands. Skin blood flow of the dorsum of four fingertips (excluding the thumb) of the left hand was measured using LDI at baseline and for 30 minutes after CS. The mean finger blood flow (FBF) of the four fingertips was expressed as arbitrary perfusion units. FTL was determined on the fourth left finger before (pre-CS-FTL) and 10 minutes after CS. RESULTS: LDI showed significantly lower mean baseline FBF in SSc patients as compared with controls (296.9 ± 208.8 vs. 503.6 ± 146.4 perfusion units; P < 0.001) and also at all time points after CS (P < 0.001). There was a significant decrease in mean FBF after CS as compared with baseline in SSc patients and in controls, followed by recovery of the blood flow 27 minutes after CS in healthy controls, but not in SSc patients. FBF tended to be lower in patients with digital scars and previous ulceration/amputation (P = 0.06). There was no correlation between mean baseline FBF and NFC parameters. Interestingly, there was a negative correlation between FTL and FBF measured by LDI in basal conditions and 10 minutes after CS in SSc patients. CONCLUSIONS: LDI showed lower digital blood flow in SSc patients when compared with healthy controls and correlated well with FTL both at baseline and after CS, allowing objective measurement of blood perfusion in SSc patients. The lack of correlation between functional and morphological microvascular abnormalities, measured by LDI and NFC, suggests they are complementary tools for evaluation of independent microangiopathy aspects in SSc patients.

Assessment of digital ischaemia and evaluation of response to therapy by 99mTc sestamibi limb scintigraphy after local cooling of the hands in patients with vasospastic Raynaud's syndrome.

AIM: Cold induced arteriolar constriction in patients with vasospastic Raynaud's syndrome (VRS) produces temporary digital ischaemia. The aim of this study was to ascertain whether 99mTc sestamibi scintigraphy is useful in the diagnosis and the monitoring of treatment in VRS. METHODS: Fifteen patients with VRS and 20 matched normal controls underwent examination. Twelve patients with VRS received therapy. For each patient, one hand was immersed in iced water for 30s while the other hand served as a control. Ten minutes after cooling, 99mTc sestamibi was injected and imaging was performed 60min later. The per cent decrease of the perfusion (%DP) was calculated by semiquantitative analysis to determine the severity of hypoperfusion. RESULTS: In all patients with VRS, moderate or marked hypoperfusion were seen in 99mTc sestamibi images after exposure to the iced water, while there was minimal or mild hypoperfusion in the control groups. Values for %DP were 46.86 +/- 19.04 and 7.85 +/- 4.53 for the VRS group and normal subjects, respectively. The difference between both groups was statistically significant (P = 0.0000). In 12 treated patients with VRS, pre-treatment and post-treatment %DP values were 51.16 +/- 18.42 and 33.58 +/- 17.83, respectively, and a significant difference was seen between both values (P = 0.001). However, there was still a statistically significant difference between control subjects and post-therapy values (7.85 +/- 4.53 vs. 33.58 +/- 17.83, P = 0.0000). The +/- 95% confidence interval of DP for control subjects was 5.7-10% (chi-squared, P = 0.000). When a DP of 10% was used as a cut-off point, sensitivity, specificity and diagnostic accuracy were 100%, 70% and 83%, respectively, for the 99mTc sestamibi scan. There was also a strong correlation between %DP and the duration of the disease (r = 0.80, P = 0.0003). CONCLUSION: The results of this study indicate that a 99mTc sestamibi scan is a valuable imaging method for the determination of digital ischaemia in vasospastic Raynaud's syndrome, and may play a role in evaluating the response to therapy.

Assessment of microvascular changes in Raynaud's phenomenon and connective tissue disease using colour doppler ultrasound.

OBJECTIVE: We used colour Doppler ultrasound (CDU) to differentiate primary from secondary Raynaud's phenomenon (pRP and sRP, respectively) and to assess digital vascular damage in patients with connective tissue disease (CTD). METHODS: Vascularity in the nailbeds of 15 healthy controls and 35 patients with CTD (systemic sclerosis or systemic lupus erythematosus) was quantified using a multi-D array transducer before and after cold and warm challenge, respectively. The results were compared with the clinically evaluated initial skin lesions. Vascularity was compared similarly between 10 pRP and 22 sRP patients. RESULTS: Vascularity at ambient temperature differed between healthy subjects and sRP patients as well as between healthy subjects and CTD patients without initial skin lesions. Patients with pRP had normal vascularity at ambient temperature but differed from healthy controls in response to a dynamic temperature challenge. CDU confirmed the clinical evaluation in 89.4% of the patients with RP and in 78.0% of the skin lesions. CONCLUSION: The novel CDU technique presented here makes it possible to discriminate between pRP and sRP and to quantify vascular changes in CTD patients.

Assessment of esophageal abnormalities in progressive systemic sclerosis using radionuclide transit.

Radionuclide transit (RT) is a noninvasive test of esophageal function with a sensitivity equivalent to manometry. Using RT, 34 patients with progressive systemic sclerosis (PSS), 15 with diffuse scleroderma and 18 with the CRST variant, were studied and compared to 22 patients with other connective tissue diseases and 20 normal volunteers. Abnormalities were present in 87% of patients with diffuse scleroderma, 72% with CRST, 38% with other connective tissue diseases and in none of the controls. The most frequent abnormality in PSS was that of adynamic transit. Abnormalities correlated with disease duration in the diffuse scleroderma group but not the CRST group. There was a positive association between abnormal RT and the presence of esophagitis in the PSS patients (p = 0.003).

The assessment of digital vessel disease by dynamic hand scanning.

A method of assessing hand and digital vessel perfusion is described. It involves an intravenous injection of Tc99m pertechnetate with monitoring of the isotope uptake in the hands and fingers by a gamma camera equipped with a small computer. The technique allows visual evaluation of the arterial supply and a means of quantifying digital perfusion. An evaluation of the technique in normal hands, in patients with digital vessel disease from vibration trauma, Raynaud's and embolism from an ulnar artery aneurysm is described. The potential of the technique in quantifying response to treatment is illustrated by sequential isotope hand scans in a patient treated for obliterative disease of the fingers.