Scleroderma and Raynaud Phenomenon: The Cold Truth Regarding the Use of Operative Management.

BACKGROUND: Raynaud phenomenon, with and without scleroderma, is a common vasospastic condition that manifests with extremity pain and skin discoloration. When conservative management fails, complications such as ischemia, ulceration, and gangrene may warrant surgical intervention. The purpose of this study was to determine the risk factors and use of surgical intervention in this population. METHODS: A national insurance claims-based database with patient records from the Centers for Medicare and Medicaid Services was used for data collection. Patients with first diagnoses of Raynaud phenomenon, scleroderma, or both between 2005 and 2014 were identified. Primary outcomes included the presence of upper extremity amputation or vascular procedure, and history of amputation within 5 years of a vascular procedure. Secondary outcomes included hospital admissions, upper extremity wounds, and amputation within 1 year of diagnosis. RESULTS: The Raynaud phenomenon, scleroderma, and Raynaud phenomenon with scleroderma cohorts consisted of 161,300, 117,564, and 25,096 patients, respectively. A diagnosis of both Raynaud phenomenon and scleroderma increased the odds of upper extremity amputation by 5.4-fold, vascular procedure by 4.8-fold, and amputation within 5 years of a vascular procedure by 1.5-fold. Patients with Raynaud phenomenon or scleroderma alone were 3.1 and 5.6 times less likely to undergo amputation within 5 years of a vascular procedure, respectively. CONCLUSIONS: Patients with both Raynaud phenomenon and scleroderma have higher likelihoods of having upper extremity amputations, vascular procedures, and amputations following vascular procedures compared to each diagnosis alone. Vascular procedures are rarely being performed. Further research is necessary to establish a standard of care and determine whether early and more frequent intervention with vascular procedures can decrease amputation rates in this patient population. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

[Quality of life of patients with Raynaud's disease]. / Raynaud-szindrómás betegek életminoségének jellemzoi.

INTRODUCTION: Raynaud's disease is characterized by episodic vasospastic attacks and digital ischemia usually followed by pain, numbness and cold. Despite the severity of the symptoms, the investigation of the quality of life in this disease received less attention yet. AIM: The aim of the study was to examine how the disease affects the patients' quality of life. METHOD: Semi-structured interviews were made with 28 patients diagnosed with Raynaud's disease. RESULTS: Almost every domain of quality of life is negatively affected. The somatic symptoms cause significant suffering, they are accompanied by loss of functionality; frequent preventive actions are needed; furthermore they affect job performance, commuting and sleep quality. Emotional and cognitive burdens and negative changes in interpersonal relationships were found. CONCLUSION: The findings of this study show that the disease is present as significant hardship in every aspect of daily life. Because of the decrease in the quality of life and the psychological burdens caused by this chronic disease, not only the basic medical care, but psychological treatment is also indicated. Orv Hetil. 2018; 159(16): 636-641.

Symptoms of Raynaud's phenomenon (RP) in fibromyalgia syndrome are similar to those reported in primary RP despite differences in objective assessment of digital microvascular function and morphology.

Symptoms of Raynaud's phenomenon (RP) are common in fibromyalgia syndrome (FMS). We compared symptom characteristics and objective assessment of digital microvascular function using infrared thermography (and nailfold capillaroscopy where available) in patients with FMS (reporting RP symptoms) and primary RP. We retrospectively reviewed the outcome of microvascular imaging studies and RP symptom characteristics (captured using patient-completed questionnaire at the time of assessment) for patients with FMS (reporting RP symptoms) and patients with primary RP referred for thermographic assessment of RP symptoms over a 2-year period. Of 257 patients referred for thermographic assessment of RP symptoms between 2010 and 2012, we identified 85 patients with primary RP and 43 patients with FMS. There were no differences in RP symptom characteristics between FMS and primary RP (p > 0.05 for all comparisons). In contrast, patients with FMS had higher baseline temperature of the digits (32.1 vs. 29.0 °C, p = 0.004), dorsum (31.9 vs. 30.2 °C, p = 0.005) and thermal gradient (temperature of digits minus temperature of dorsum; +0.0 vs. -0.9 °C, p = 0.03) compared with primary RP. Significant differences between groups persisted following local cold challenge. In primary RP, patient reporting "blue" digits, bi-phasic and tri-phasic RP was associated with lower digital perfusion. In contrast, no associations between skin temperature and RP digital colour changes/phases were identified in FMS. Our findings suggest that symptoms of RP in FMS may have a different aetiology to those seen in primary RP. These findings have potential implications for both the classification of RP symptoms and the management of RP symptoms in the context of FMS. Digital colour changes reported by patients might reflect the degree of digital microvascular compromise in primary RP.

[Assessment of ECG and holter parameters among patients with systemic sclerosis].

Introduction: The aim of our study was to assess electrocardiographic abnormalities on the standard ECG and Holter monitoring in patients with systemic sclerosis. Materials and methods: 70 patients with systemic sclerosis and 20 healthy individuals were included in the study. Mean disease duration was 100,8 ± 95,79 months. The following electrocardiographic parameters were assessed: driving rhythm, P wave, PQ interval, atrio-ventricular and intraventricular conduction disturbances, ST-T complex abnormalities, mean heart rate, presence of conduction disturbances, supraventricular and ventricular arrhythmias. We evaluated if there could be any relationship between the Raynaud's phenomenon or disease duration and electrocardiographic abnormalities. Results: We found that patients with systemic sclerosis had higher burden of supraventricular and ventricular arrhythmias than individuals in the control group. The longer duration of the Raynaud's phenomenon or systemic sclerosis the higher incidence of these arrhythmias could be found. Patients with supraventricular arrhythmias were more often in NYHA class I/II heart failure whilst patients with ventricular arrhythmias were in NYHA class II. Conclusions: Patients with the longstanding Raynaud's phenomenon and systemic sclerosis are at a high risk of developing cardiac arrhythmias. It seems that a vigilant electrocardiographic monitoring could increase patients safety.

Early systemic sclerosis: assessment of clinical and pre-clinical organ involvement in patients with different disease features.

OBJECTIVE: To assess internal organ involvement in early SSc at presentation. METHODS: One hundred and fifteen patients admitted to a tertiary centre because of RP, who did not present any routinely detectable scleroderma-related internal organ involvement, were investigated for ANA and videocapillaroscopy, and underwent history and physical examination to detect symptoms/signs suggestive of SSc. Patients were then subdivided into three groups: (i) early SSc, constituted by patients without clinical manifestations other than RP, but with scleroderma marker autoantibodies and/or typical capillaroscopic abnormalities; (ii) probable SSc, constituted by patients with the same autoantibody and/or capillaroscopic status as early SSc patients, but with any of the following manifestations: digital ulcers/scars, puffy fingers, arthritis, telangiectasia, dysphagia/heartburn, shortness of breath; (iii) UCTD, constituted by patients with a specific (i.e. disease antibody marker) ANA and capillaroscopic findings plus any disease manifestation. All patients were investigated by lung functional study and B-mode echo-Doppler-cardiography. Patients who consented underwent oesophageal manometry. RESULTS: An inverted mitral E : A ratio (i.e. early scleroderma cardiac involvement) and/or a diffusing lung capacity for CO <80% of the predictive value (i.e. early lung involvement) and/or basal low oesophageal sphincter pressure <15 mmHg (i.e. early oesophageal involvement) were detected in 37/51 probable SSc patients (72%), 8/19 early SSc patients (42%) and 12/45 UCTD patients (27%). CONCLUSION: A scleroderma-related internal organ involvement was detected in patients from each group and, more importantly, was pre-clinical in a number of cases.

Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis.

OBJECTIVE: To correlate microvascular abnormalities, evaluated by nailfold videocapillaroscopy (NVC), with the duration of both Raynaud's phenomenon (RP) and systemic sclerosis (SSc) from the date of diagnosis, in a large number of patients with SSc. METHODS: Ninety-seven consecutive patients were recruited and distributed into 3 groups on the basis of the morphological NVC patterns observed: "early" (E), "active" (A), and "late" (L). In each group the age of patients, age at onset, and the duration of RP as well as of overt SSc were investigated and correlated with the different NVC pattern variables. RESULTS: The early appearance of giant capillaries and hemorrhages (E pattern) is of great relevance for the early diagnosis of SSc. Therefore, these alterations are more evident in the active phase of the disease (A pattern). Conversely, the NVC observation of loss of capillaries and vascular architectural disorganization and the presence of ramified/bushy capillaries (L pattern) represents the clearest aspect of advanced SSc microvascular damage. These morphological alterations were found to correlate significantly with the duration of both RP and SSc, as well as with age of patients (p = 0.0001). No significant differences were observed when the variables were analyzed in the patients classified as having limited cutaneous SSc or diffuse cutaneous SSc. CONCLUSION: Classification of defined major nailfold patterns may be useful in assessing the appearance and progression of sclerodermic microangiopathy. As well, nailfold changes might represent a morphological reproduction of the evolution of SSc.

Comparative assessment of the effects of vasodilators on peripheral vascular reactivity in patients with systemic scleroderma and Raynaud's phenomenon: color Doppler flow imaging study.

The aim of the present study was assessment of peripheral vascular reactivity during cold test effects of different types of vasodilators on vascular resistance in patients with progressive systemic sclerosis and Raynaud's phenomenon with use of color Doppler flow imaging of upper extremity.

Use of prostaglandin E1 (lipo-PGE1) to treat Raynaud's phenomenon associated with connective tissue disease: thermographic and subjective assessment.

Four patients with connective tissue disease who had been suffering from severe Raynaud's phenomenon were treated with an intravenous infusion of prostaglandin E1 (PGE1) and its effectiveness was assessed by thermography and a questionnaire approach. PGE1 in a lipid microsphere formulation (lipo-PGE1) produced a significantly greater increase in lesional skin temperature compared with treatment with PGE1 clathrated in alpha-cyclodextrin 12 months previously in the same group of patients. These results were supported by the subjective assessment obtained from the patients by questionnaire.

Finger systolic pressure during local cooling in normal subjects aged 20 to 60 years: reference values for the assessment of digital vasospasm in Raynaud's phenomenon of occupational origin.

Measurements of finger systolic pressure (FSP) during a standardized cold test (finger cooling to 30, 15 and 10 degrees C during 5 min ischemia) were performed on 103 healthy subjects divided into four subgroups according to age: 20-29 years (n = 22), 30-39 years (n = 32), 40-49 yr (n = 27), 50-60 years (n = 22). Among the age groups there was no difference in the reduction of FSP (FSP%) and in the digit-to-brachial systolic pressure index (DBI%) after cold provocation at 15 and 10 degrees C. Cold-induced digital vasoconstriction was more intense in smokers than in non smokers (P = 0.025). In the entire study population the lower normal limits of FSP% were 74% at 15 degrees C and 63% at 10 degrees C. Using an FSP%10 of less than 60% as a discriminating threshold between normals and patients with vibration white finger, the sensitivity of the cold test was 87% and the specificity was 100%. The findings of this study suggest the following considerations: (a) age does not seem to influence the digital arterial response to cold in healthy subjects; (b) smoking has a harmful effect on finger cold reaction in normal individuals; (c) the measurement of FSP during cooling is an accurate method of objectively diagnosing Raynaud's phenomenon of occupational origin.