[Factors determining the quality of life in patients with Raynaud's syndrome]. / Az életminoséget meghatározó tényezok Raynaud-szindrómában.

INTRODUCTION: Raynaud's disease is a vasospastic phenomenon affecting acral areas, which manifests itself in characteristic color changes. Symptoms are affected by mundane things like stress or temperature. There are also differences in the presence and progression of the disease in terms of gender, age, health-damaging behaviors (e.g., smoking) and occupation. OBJECTIVE: The aim of our study was to examine how the risk factors assumed in the literature affect the quality of life of patients with Raynaud's disease. METHOD: 110 people diagnosed with Raynaud's disease completed a questionnaire on disease-specific quality of life and risk factors. RESULTS: There was a significant difference between the groups with good and less good quality of life in terms of age (p<0.001), education (p<0.01), type of diagnosis (p<0.001), duration of illness (p<0.001), headache frequency (p<0.01), the influence of cold (p<0.05) and emotions (p<0.01). The groups currently working (p<0.01), drinking coffee more often (p<0.05), attributing less influence to emotions (p<0.001) and cold (p<0.01) had a better quality of life. According to the regression analysis, the type of diagnosis, the duration of the disease, the influencing role of emotions and cold are the most important predictors of Raynaud-specific quality of life. CONCLUSION: Our results draw attention to the role of factors that potentially affect the long-term development of the quality of life, thereby identifying the possible focuses of prevention. Orv Hetil. 2022; 163(47): 1880-1885.

Alexithymia and emotion regulation in patients with Raynaud's disease.

OBJECTIVES: Besides cold, emotional distress is the most important trigger of Raynaud's disease (RD) attacks, although little is known about the factors that contribute to the effectiveness of coping with emotional distress. The aim of the present study was to explore alexithymia and emotion regulation and their relationship with depression and quality of life among patients with RD. METHODS: Total of 110 patients (mean age 53.65; 96 women) with RD completed self-report measures. RESULTS: Alexithymia was associated with adverse emotional regulation skills, depression, and quality of life impairment. Detailed analysis revealed that there are significant differences between alexithymic and nonalexithymic patients in emotion regulation. CONCLUSION: Alexithymia is a significant factor affecting health status in RD since it plays a significant role in emotion regulation. A multidisciplinary approach is essential to improve treatment outcome by identifying patients with high alexithymia, and to improve their emotional regulation skills.

Comparison of mental and physical health between patients with primary and secondary Raynaud's phenomenon Category: Article.

OBJECTIVE: To compare anxiety, depression, physical health and quality of life in patients with primary Raynaud's phenomenon (PRP) and patients with secondary Raynaud's phenomenon (SRP). METHOD: Adult patients with Raynaud's phenomenon (RP) were assessed for the severity of anxiety and depressive symptoms, physical health and quality of life by using the General Anxiety Disorder Scale (GAD-7), the Beck Depression Inventory (BDI), the 12-item Short Form Health Survey: Physical Component Scale (PCS-12), and the Raynaud Specific Quality of Life Questionnaire (RQLQ), respectively. Patients with PRP and SRP were recruited for comparison at a single clinical center in Debrecen, Hungary from September to December 2017. RESULTS: In total, 60 primary and 41 secondary patients with RP were studied. Gender distribution, family status, employment status and smoking were similar in the two groups. Significantly more patients with SRP had anxiety and depressive symptoms than patients with PRP. Patients with SRP had significantly higher GAD-7 and BDI; and lower overall PCS-12 and RQLQ scores than patients with PRP. CONCLUSION: Anxiety and depression is more common in patients with SRP than in patients with PRP. Patients with SRP have a lower physical health condition and RP specific quality of life than patients with PRP. Anxiety, depression and quality of life impairments should be taken into account when managing all patients with RP. Further study is needed to assess whether appropriately designed interventions have the potential to reduce the mental and physical health burdens of RP on quality of life (QOL), especially in patients with SRP.

The patient experience of Raynaud's phenomenon in systemic sclerosis.

RP is the most common manifestation of SSc and a major cause of disease-related morbidity. This review provides a detailed appraisal of the patient experience of SSc-RP and potential implications for disease classification, patient-reported outcome instrument development and SSc-RP clinical trial design. The review explores the clinical features of SSc-RP, the severity and burden of SSc-RP symptoms and the impact of SSc-RP on function, work and social participation, body image dissatisfaction and health-related quality of life in SSc. Where management of SSc-RP is concerned, the review focuses on the 'patient experience' of interventions for SSc-RP, examining geographic variation in clinical practice and potential barriers to the adoption of treatment recommendations concerning best-practice management of SSc-RP. Knowledge gaps are highlighted that could form the focus of future research. A more thorough understanding of the patient experience could support the development of novel reported outcome instruments for assessing SSc-RP.

Behaviour change interventions for the management of Raynaud's phenomenon: a systematic literature review.

OBJECTIVES: Raynaud's phenomenon (RP) is a significant cause of morbidity. Vasodilator medications cause unwanted adverse effects, with behavioural and lifestyle changes forming the mainstay of self-management; this is difficult to implement successfully. The objectives of this study were to evaluate the efficacy of behaviour change interventions for RP and identify learning points for future treatment development. DESIGN: Systematic literature review and narrative synthesis of findings. DATA SOURCES: EMBASE, MEDLINE, Cochrane and PsycINFO were searched for eligible studies on 22 August 2017. ELIGIBILITY CRITERIA: Randomised controlled trials (RCTs) of behaviour change interventions with at least one control comparator arm. DATA EXTRACTION AND SYNTHESIS: Study selection, data extraction and risk of bias were assessed independently by two reviewers, reaching consensus with a third when necessary. Primary outcomes of interest included severity/impact, frequency and duration of RP episodes, pain, disability, adverse events and study withdrawal. RESULTS: Of 638 articles retrieved, eight studies fulfilled criteria for inclusion. Biofeedback was the active behaviour change treatment arm for seven studies, with one study reporting a behavioural intervention. Studies were published 1978-2002; six were USA-based studies, one German and one Swedish. Using Cochrane Risk of Bias assessment, studies were assessed to be overall at high risk of bias, with the exception of one large RCT. The total sample included 495 participants (study median=29), with a median age of 39.5 years and preponderance towards females (73%). Five studies reported significant effects in primary outcomes of interest; however, due to missing data, relative efficacy of interventions could not be reliably assessed. CONCLUSIONS: There is no evidence to support or refute claims of the efficacy of behaviour change interventions for the management of RP. There remains a strong case for developing and testing behaviour change interventions that focus on self-management; however, theoretical development and advancement in trial quality is imperative to underpin future work. PROSPERO REGISTRATION NUMBER: CRD42017049643.

[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.

Multinational Qualitative Research Study Exploring the Patient Experience of Raynaud's Phenomenon in Systemic Sclerosis.

OBJECTIVE: Raynaud's phenomenon (RP) is the most common manifestation of systemic sclerosis (SSc). RP is an episodic phenomenon, not easily assessed in the clinic, leading to reliance on self-report. A thorough understanding of the patient experience of SSc-RP is essential to ensuring that patient-reported outcome (PRO) instruments capture domains important to the target patient population. We report the findings of an international qualitative research study investigating the patient experience of SSc-RP. METHODS: Focus groups of SSc patients were conducted across 3 scleroderma centers in the US and UK, using a topic guide and a priori purposive sampling framework devised by qualitative researchers, SSc patients, and SSc experts. Focus groups were audio recorded, transcribed, anonymized, and analyzed using inductive thematic analysis. Focus groups were conducted until thematic saturation was achieved. RESULTS: Forty SSc patients participated in 6 focus groups conducted in Bath (UK), New Orleans (Louisiana), and Pittsburgh (Pennsylvania). Seven major themes were identified that encapsulate the patient experience of SSc-RP: physical symptoms, emotional impact, triggers and exacerbating factors, constant vigilance and self-management, impact on daily life, uncertainty, and adaptation. The interrelationship of the 7 constituent themes can be arranged within a conceptual map of SSc-RP. CONCLUSION: We have explored the patient experience of SSc-RP in a diverse and representative SSc cohort and identified a complex interplay of experiences that result in significant impact. Work to develop a novel PRO instrument for assessing the severity and impact of SSc-RP, comprising domains/items grounded in the patient experiences of SSc-RP identified in this study, is underway.

Prediction and impact of attacks of Raynaud's phenomenon, as judged by patient perception.

OBJECTIVE: To evaluate whether patients can predict attacks of RP (if so, this would have implications for developing new treatments) and to evaluate the impact of RP attacks on quality of life (QoL). METHODS: Individuals with RP were invited through international patient associations to participate in an online survey. RESULTS: Responses from 443 subjects with self-reported RP from 15 countries were evaluable. The mean age of subjects was 41 years (91% female). Fifty-eight per cent of subjects reported they could predict at least 51% of RP attacks, and 57% could predict attack severity either fairly well or better [with 43% predicting severity only poorly (30%) or very poorly (13%)]. Sixty-four per cent of subjects reported a poor or very poor current ability to prevent/control RP attacks. One hundred and eighty-two subjects (41%) reported current or previous use of medications for RP: 82% reported at least one currently used medication being tolerated, but only 16% reported at least one current medication being effective. Most subjects (78%) reported making at least one life adjustment due to RP, with more in subjects with secondary RP compared with primary RP (87% vs 71%, P = 0.001). Current QoL with RP was impaired [mean = 6/10 (10 best imaginable)] and secondary RP subjects reported a greater absolute improvement when asked to imagine their QoL without RP (2.3 vs 3.3 P = 0.0002). CONCLUSION: Subjects' ability to predict RP attacks is limited. Treatments were generally considered tolerable but seldom fully effective. Our results confirm an unmet need for new treatments. RP significantly impacts on QoL in all subjects.

Autoimmune rheumatic disease associated symptoms in fibromyalgia patients and their influence on anxiety, depression and somatisation: a comparative study.

OBJECTIVES: In this study we evaluated the frequency of autoimmune rheumatic disease associated major symptoms in fibromyalgia (FM) patients, and the association between their presence and anxiety, depression and somatisation. METHODS: Two hundred and thirty-two FM, 78 systemic lupus erythematosus (SLE) patients and 70 healthy controls were included. All subjects were questioned face-to-face for the presence of autoimmune rheumatic disease-associated symptoms and antinuclear antibody (ANA) was determined. All FM patients were questioned for the severity of pain and symptoms of FM by using a visual analogue scale. In addition, all subjects were interrogated for anxiety, depression, somatic symptoms and neuropathic pain by using different validated questionnaires. RESULTS: FM patients had significantly higher frequency of photosensitivity (27.6% vs. 11.4%) and Raynaud phenomenon (22% vs. 10%) when compared to controls (p-values, 0.005 and 0.026). FM patients had significantly lower frequencies of photosensitivity, oral ulcers, xerostomia, and xerophthalmia than SLE patients (all p-values <0.001). ANA positivity was 11.8% in FM patients and 7.1% in healthy controls. ANA-positive and negative FM patients had similar frequencies of autoimmune rheumatic disease symptoms. FM patients with photosensitivity had higher anxiety (p=0.002), somatic symptoms (p=0.015) and neuropathic pain (p=0.03) scores than others. FM patients with Raynaud had higher anxiety (p=0.004), depression (p=0.001), somatic symptom (p<0.001) and neuropathic pain scores than others. CONCLUSIONS: The presence of which findings in FM seems to be associated with anxiety, depression, and somatization rather than ANA positivity and disease severity.

The minimally important difference and patient acceptable symptom state for the Raynaud's condition score in patients with Raynaud's phenomenon in a large randomised controlled clinical trial.

BACKGROUND: The Raynaud's condition score (RCS) is a validated outcome measure for Raynaud's phenomenon (RP). OBJECTIVE: To assess the minimally important difference (MID) and patient acceptable symptom state (PASS) for RCS in patients with RP. SUBJECTS: and methods Patients with active RP (n=162) (mean RCS >25 (0-100 visual analogue scale) participated in a placebo-controlled, crossover randomised clinical trial (RCT). Data from the two treatment groups were combined for this analysis. Retrospective and prospective anchors were administered during the RCT. MID groups were defined as the group who reported being somewhat better (anchor #1) and a one-step change from "unbearable" to "very severe", etc (anchor #2). Patients were considered to have achieved PASS if they rated their Raynaud's condition as "very mild" or "mild" at the last study visit. RESULTS: The mean age of participants was 48.9 years and the mean baseline RCS was 46.4 points. The RCS change score for the MID improvement group ranged from -13.9 to -14.3 points and PASS estimate was 34.0 points. CONCLUSION: The MID and PASS estimates for RCS are 14-15 points for improvement and 34 points, respectively, on a 0-100 scale in a large RCT of patients with active RP. This information can aid in interpreting RCS in future RP trials.

Comparison of patients with and without digital ulcers in systemic sclerosis: detection of possible risk factors.

BACKGROUND: Digital ulcers (DU) are a major complication in the course of systemic sclerosis (SSc). In recent years, efficacious, but expensive therapies (e.g. iloprost, sildenafil, bosentan) have been shown to improve healing or to reduce the recurrence of DU. For optimal management it would be useful to identify the risk factors for DU. Such statistical analyses have been rare because they require a high number of patients. OBJECTIVES: To identify potential risk factors for DU in patients with SSc. METHODS: We used the registry of the German Network for Systemic Scleroderma and evaluated the data of 1881 patients included by August 2007. We assessed potential risk factors for DU by comparing patients with (24.1%) and without active DU at time of entry (75.9%). RESULTS: Multivariate analysis revealed that male sex, presence of pulmonary arterial hypertension (PAH), involvement of the oesophagus, diffuse skin sclerosis (only when PAH was present), anti-Scl70 antibodies, young age at onset of Raynaud's phenomenon (RP), and elevated erythrocyte sedimentation rate (ESR) significantly impacted on the appearance of DU. Certain combinations increased the patients' probability of presenting with DU, with the highest probability (88%) for male patients with early onset of RP, ESR>30 mm h(-1), anti-Scl70 antibodies and PAH. Patients with DU developed RP, skin sclerosis and organ involvement approximately 2-3 years earlier than patients without DU. CONCLUSIONS: The results reveal possible risk factors for the occurrence of DU in SSc. As DU are prone to local complications, prophylactic vasoactive treatment for patients presenting with these factors may be justified.

[Psychometric investigation of Tyrolean patients with primary Raynaud's phenomenon]. / Psychometrische Tests bei primärem Raynaud-Phänomen--Untersuchungsergebnisse an einem Tiroler Patientenkollektiv.

Primary Raynaud's phenomenon (PRP) is provoked by digital vasospasm induced by cold and emotional strain. By use of established psychometric test instruments (Freiburger Aggressionsfragebogen (FAF), Fragebogen zur Abschätzung psychosomatischen Krankheitserlebens (FAPK), Stressverarbeitungsfragebogen (SVF 120)) we examined the patients' ways of dealing with aggression and distress as well as their experience of psychosomatic illness. In all psychometric tests performed there were no statistically significant differences between the patients and the controls, indicating that patients with PRP depict no specific ways of dealing with aggression and distress, and do not experience psychosomatic illness. The subgroup of patients with affective disorders and the subgroup of patients with impaired psychological well-being, displayed a 'depressive style' of dealing with life events. Patients with major physical complaints of symptoms of Raynaud's phenomenon showed either an inability or a propensity to deny aggression. Patients with impaired psychological well-being tended to complain more often about severe physical symptoms associated with Raynaud's phenomenon. Psychometric testing in patients with primary Raynaud's phenomenon cannot be recommended without additional psychiatric diagnostic. In patients with primary Raynaud's phenomenon and psychiatric comorbidity psychometric testing can additionally provide useful clinical information.

Iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis and the quality of life: a new therapeutic protocol.

OBJECTIVES: To evaluate the clinical efficacy and the effects on the quality of life of iloprost, a prostacyclin analogue, used according to a new protocol in patients with Raynaud's phenomenon secondary to systemic sclerosis. METHODS: In this randomized study, we treated 30 patients with iloprost, given by intravenous infusion, at progressively increasing doses (from 0.5 to 2 ng/kg/min) over a period of 6 h each day for 10 days in two consecutive weeks, with repeated cycles at regular intervals of 3 months for 18 months. The results were compared with those obtained in 30 other patients who received the same drug but with different dosing schemes. RESULTS: The total average daily duration of the attacks, the average duration of a single attack and the average daily frequency of the attacks were reduced significantly in all treatment groups, but the comparison between the groups demonstrated significant differences between patients treated with the new protocol and the others at later times (12 and 18 months). The effects on the quality of life in the group treated with the new protocol, evaluated with the Short Form-36, demonstrated a marked improvement regarding both the scale relating to the physical aspect of the illness and, especially, the scale relating to the mental aspect. CONCLUSIONS: In patients with systemic sclerosis, cyclic intravenous iloprost infusion is efficacious in the treatment of Raynaud's phenomenon. The protocol that we used, compared with others, not only has favourable clinical effects but also leads to a marked improvement in the quality of life.

Migraine aura without headache compared to migraine with aura in patients with affective disorders.

The characteristics of psychiatric comorbidity in migraine have been studied in migraine with aura (MA) and migraine without aura (MO). Little information is available concerning patients with migraine aura without headache. In a study of 201 patients with major affective disorders (DSM-IV) we have described the clinical characteristics of patients with these three sub-types of migraine (IHS criteria) and compared the MA and migraine aura without headache groups. Compared to patients having MA (n=57), the group with migraine aura without headache (n=18) had a higher age of onset of migraine (28.5 vs. 19.2, p=0.001), significantly lower prevalences of affective temperaments (28% vs. 56%, p=0.036), suicide attempts (17% vs. 53%, p=0.013) and Raynaud's syndrome (0% vs. 25%, p=0.017). These results indicate that there seem to be differences in the clinical characteristics found in patients with migraine with aura when compared to those having the migraine subtype without a headache phase. This may convey new information concerning the comorbid expression of migraine and affective disorders or concerning the processes that differentiates the migraine types with and without a subsequent pain attack.

Daily occupations and well-being in women with limited cutaneous systemic sclerosis.

OBJECTIVE: This study investigated occupational performance, well-being (operationalized as general life satisfaction, domain-specific life satisfaction, and self-rated health), and perceived symptoms in women with limited scleroderma and healthy controls. METHODS: Interview-based and self-administered questionnaires were used with 36 women with limited scleroderma and 40 healthy women. RESULTS: In the scleroderma group, most of occupation problems were perceived in work and household chores. The women with scleroderma were mainly satisfied with self-care and least satisfied with household chores. Regarding domain-specific life satisfaction, the women were least satisfied with physical health and leisure. General life satisfaction showed the strongest relations to performance of self-defined occupations and satisfaction with leisure, whereas the strongest association with self-rated health was found for satisfaction with work. Fatigue was perceived as a dominant problem and was significantly associated with well-being. Furthermore, the women with scleroderma felt lower satisfaction with daily occupations and well-being than the healthy women. CONCLUSION: Loss of occupations, low satisfaction with leisure, perceived fatigue, shortness of breath, and pain indicated poorer well-being in women with scleroderma and need to be focused on in occupational therapy interventions.

[Psychiatric investigation of Tyrolean patients with primary Raynaud's phenomenon]. / Psychiatrische Untersuchungen bei Tiroler Patienten mit primärem Raynaud-Phänomen.

Raynaud's phenomenon is provoked by digital vasospasm, mostly induced by cold and emotional strain. While studies dealing with other vasospastic disorders, e. g. migraine, described an increased comorbidity with affective and anxiety disorders, only little evidence has been reported for such an association in Raynaud's phenomenon. 70 Tyrolean patients (55 females and 15 males) with primary Raynaud's phenomenon presented more often with psychiatric morbidity on DSM-IV axis-I during their life-time than prevalence studies in the general population of North America and Europe would have led to expect. No psychotic (0%) and fewer somatoform disorders (2.9%) were found whereas anxiety disorders (77.1%), affective disorders (48.6%), and eating disorders (14.3%) were clearly overrepresented. We would therefore recommend a psychiatric evaluation in primary Raynaud's phenomenon along with the vascular diagnostic assessment to ensure that any psychiatric co-morbidity can be identified and treated.