French recommendations for the management of systemic sclerosis.

Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.

Patients and spouses coping with inflammatory arthritis: Impact of communication and spousal perceived social support and burden.

OBJECTIVES: To guide Therapeutic Patient Education (TPE) programs to improve patients' and spouses' perceived health, we aimed to analyze the effect of the spouse health state and patient-spouse relationship on functional impairment, mental health and self-efficacy of patients with RA or SpA. METHODS: In this observational cross-sectional study, inclusion criteria were age≥18years and diagnosis of RA or SpA. The outcome criteria were functional impairment and mental health measured by the Medical Outcomes Study Short-Form 12-item version (SF-12-MCS); mental health measured by the General Health Questionnaire (GHQ-12); and self-efficacy by the General Self-Efficacy Scale (GSE Scale). Caregiver-patient relationship was assessed by the Personal Assessment of Intimacy in Relationships (PAIR) questionnaire and the Dyadic Adjustment Scale (DAS); social support by the Social Support Questionnaire-6 and spousal burden by the Zarit questionnaire. RESULTS: A total of 88 patient-spouse couples were included. Patients were mostly female (n=68, 77%), with mean age 59 (SD 12.6) years; 69% had RA. On bivariate analyses, only spousal burden was associated with patients' functional impairment. Patients' mental health was associated with satisfaction with social support and the dyad relationship. Self-efficacy of patients was associated with spousal burden, satisfaction with social support, spousal anxiety/depression and the dyad relationship. On multivariate analysis, improved mental health and perceived self-efficacy of patients were associated with spousal satisfaction with social support (Beta=0.8, P=0.1 with GHQ-12) good communication in the couple (Beta=0.5, P=0.04 with SF-12-MCS and Beta=0.4, P=0.04 with GSE Scale) and low spousal-assessed burden (Beta=-0.2, P=0.003 with GSE Scale). CONCLUSION: This study has identified potential focus for intervention. It has highlighted the importance of recognizing the role of couple communication (experiencing an open and fluent exchange of ideas) in the patient's mental health and self-efficacy but also perceived satisfaction with social support of both members of the couple on the patient's mental health.

What Is Relatives' Role in Arthritis Management? A Qualitative Study of the Perceptions of Patient-Relative Dyads.

PURPOSE: The main aim of patient education is to maintain or improve quality of life. It is mostly focused on patients even if families might be included. The aim of this study was to explore patients' and relatives' perceptions and experiences about the role of relatives in disease management in chronic inflammatory arthritis in order to provide insight into how patient education programs might include relatives. METHODS: Individual semi-structured interviews were conducted with 20 patients (13 with polyarthritis; 7 with spondyloarthritis) and one of their relatives (N=40). A thematic analysis following an inductive approach was carried out using the QDA-Miner Software (inter-coder agreement 0.7). RESULTS: The analysis revealed three relevant themes. The first was their perception of relatives' general roles, which included technical skills, knowledge about the disease and interpersonal skills. The other two themes dealt with their specific relationship: the dyad relationship (including the usual relationship and in the context of the disease) and the help relationship (including practical assistance and emotional help). CONCLUSION: The results show the wide-ranging role of relatives in practical and emotional support, the complexity of patient-relative interactions regarding requests for help, their relationship and ability to share difficulties. This study gives indications about how to include relatives in TPE programs and emphasizes the importance of developing interventions for patient-relative dyads regarding the practical and emotional management of the disease, as well as interactions concerning help. Those interventions should enhance patients and relatives' quality of life.

Patients and relatives coping with inflammatory arthritis: Care teamwork.

OBJECTIVE: To explore how patients and relatives experience and talk together about their life with inflammatory arthritis. DESIGN: Qualitative research. SETTING: A convenience sample was used. Participants were recruited in seven rheumatology departments in France. PARTICIPANTS: Patients with rheumatoid arthritis or spondyloarthritis, agreeing to participate in the study with a relative, age at least 18 years. DATA COLLECTION AND ANALYSIS: Two psychologists conducted face-to-face interviews with 20 patient-relative dyads (40 individuals). A thematic analysis followed a general inductive approach. RESULTS: Saturation was reached after interviews with 20 dyads. The analysis revealed four main themes: (a) disease 'lived' together: a new role for the relative (providing help in physical tasks, emotional support, acting as a driving force, having a role in medical care) and communication around the disease (not focusing on the disease); (b) impact of the disease on the relationship; (c) social impact of the disease on the dyad (social isolation); (d) difficulties and needs of the relative (need to better know the disease). CONCLUSION: This study has highlighted the importance of recognizing the role of the relative in the management of inflammatory arthritis disease, especially when medical decisions are shared with professionals. A joint approach to treatment is a basis for coping with the disease. This approach supposes (a) discussions about relatives' new roles to clarify them, (b) patients' and relatives' communication skills and (c) a good understanding of each other, which can be improved by providing information on the disease and coping strategies for both the patient and the relative.

A very rare cause of acro-osteolysis: Hajdu-Cheney syndrome.

Acro-osteolysis is not uncommon and occurs in several conditions. Additional clinical and paraclinical findings and sometimes the performance of molecular tests can help to clarify the diagnosis. Here, we report the case of a 36-year-old woman who was referred to our department because of acute pain in the extremity of the left index finger. However, subsequent clinical examination also revealed short digits with pseudo-clubbing related to acro-osteolysis. Furthermore, severe osteoporosis, a moderate dysmorphic face, joint hypermobility, biological variables within normal ranges and her clinical history led us to consider the diagnosis of Hajdu-Cheney syndrome. Molecular analysis confirmed the diagnosis with the identification of a mutation in the NOTCH2 gene. The patient received bisphosphonate therapy, which resulted in some clinical and biological improvement 12 months later.

Characteristics and development of therapeutic patient education in rheumatoid arthritis: analysis of the 2003-2008 literature.

OBJECTIVE: The aim of this study is to point out the recent characteristics and developments of therapeutic patient education (TPE) in rheumatoid arthritis through an analysis of the international articles published from 2003 to 2008. METHOD: Studies were selected from major databases, using the following keywords: rheumatoid arthritis, patient education, self-management, programs. Three authors independently reviewed each study and selected the data using the patient education research categories (PERC). Articles consistently related to patient education in rheumatoid arthritis (37 among 109) were included. RESULTS: The selected articles have been published in 23 scientific journals. The majority of them concern TPE for adult patients with rheumatoid arthritis. TPE is delivered in several structures and group education represents the most widespread educational strategy mainly provided by a multiprofessional team. There are two types of programs: educational, aiming to make the patient competent in the daily management of his disease and psycho-educational ones, aiming to improve coping and to decrease stress, anxiety and depression. Twenty-eight studies show the effectiveness of TPE on the basis of bio-clinical, educational, psychosocial, economical criteria, but the majority of these positive results are observed in short-term. Barriers to TPE are linked to cultural and socio-economic factors. CONCLUSION: A large number of studies still assess the positive effects of TPE. Nowadays, the problems of short-term efficacy of TPE and the cultural and social barriers to this practice have become a major issue for research.

Sonographic appearance of trigger fingers.

OBJECTIVE: The purpose of this study was to describe the sonographic appearance of the first annular (A1) pulley-flexor tendon complex in patients with trigger fingers. METHODS: Thirty-three trigger fingers in 33 patients were examined with a 7- to 15-MHz probe. A control group consisted of 20 patients without trigger fingers. The study included systematic measurement of the thickness of the A1 pulley and a power Doppler assessment of the pulleys, tendons, and tendon sheaths. RESULTS: Thickening and hypoechogenicity of the A1 pulley were found in all patients with trigger fingers. Measurements of A1 pulley thickness were significantly different (P < .0001) between the groups without trigger fingers (mean, 0.5 mm; range, 0.4-0.6 mm) and with trigger fingers (mean, 1.8 mm; range, 1.1-2.9 mm). Hypervascularization of the A1 pulley on power Doppler imaging was found in 91% of the trigger fingers but was never found in the healthy control group. Flexor tendinosis was found in 48% of the trigger fingers; tenosynovitis was found in 55%; and both were found in 39%. In the control group, tenosynovitis and tendinosis were not found. CONCLUSIONS: Thickening and hyper-vascularization of the A1 pulley are the hallmarks of trigger fingers on sonography. Other frequently observed features include distal flexor tendinosis and tenosynovitis.

Influence of patient education on exercise compliance in rheumatoid arthritis: a prospective 12-month randomized controlled trial.

OBJECTIVE: To determine the effect of education on the exercise habits of patients with rheumatoid arthritis (RA) after 6 and 12 months. METHODS: We studied 208 outpatients recruited between June 2001 and December 2002. This was a prospective controlled randomized trial. The active group received a multidisciplinary education program, including training in home-based exercises and guidelines for leisure physical activity (PA). The control group received a booklet added to usual medical care. Compliance with home-based exercises was defined as a practice rate >or= 30% of the prescribed training. Compliance with leisure PA was defined as >or= 20% increase in Baecke questionnaire score. Additional assessments involved possible predictors of compliance and changes with regard to the compliance. RESULTS: At 6-month followup, home-based exercise and leisure PA compliance were significantly higher [13.5% vs 1%, respectively (p = 0.001); and 28.2% vs 13.8% (p = 0.02)], but were not at 12 months. Predictors of leisure PA compliance at 6 months included participating in the active group (odds ratio 2.74, 95% CI 1.17 to 6.38) and previous low leisure PA (OR 6.01, 95% CI 2.47 to 14.61), with decreased fatigue (FACIT-F mean -2.94 +/- 8.04 vs -0.1 +/- 7.25 for noncompliant subjects; p = 0.04) and improved psychological status (Arthritis Impact Measurement Scale mean -1.25 +/- 3.12 vs 0.11 +/- 3.39; p = 0.03). CONCLUSION: Education of patients with RA may increase compliance especially with leisure PA, particularly when it is poor at baseline, but these effects are limited and short-term.

Effect of a collective educational program for patients with rheumatoid arthritis: a prospective 12-month randomized controlled trial.

OBJECTIVE: To evaluate the effect on health and functional status of an 8-week group-education program for rheumatoid arthritis (RA) in addition to usual medical care. METHODS: All consecutive inpatients and outpatients with RA (ACR criteria) were asked to participate in this randomized, prospective, controlled trial. The educational intervention consisted of 8 weekly ambulatory sessions, each lasting 6 hours. Followup was undertaken after 1 year. The primary criterion for judging effectiveness was the Health Assessment Questionnaire (HAQ) score; secondary criteria consisted of coping, medical knowledge, patient global satisfaction, and quality of life scores before the intervention and after 1 year. RESULTS: We asked 1242 inpatients and outpatients to participate in the study: 208 (16.75%) agreed (104 in each group). At baseline, there was no statistically significant difference between the 2 groups. After 1 year, no statistically significant difference was observed between the 2 groups in change in HAQ score: -0.04 +/- 0.46 (education group) vs -0.06 +/- 0.47 (control group) (p = 0.79). Statistically significant differences were found in 3 domains: patient coping (-1.22 +/- 5.55 vs -0.22 +/- 3.81; p = 0.03), knowledge (3.42 +/- 4.73 vs 0.73 +/- 3.78; p < 0.0001), and satisfaction (10.07 +/- 11.70 vs 5.72 +/- 13.77; p = 0.02), all of which were better for the group attending the education sessions. CONCLUSION: Despite improvements in patient coping, knowledge, and satisfaction, the education program was not found to be effective at 1 year. There may have been methodological problems relating to the sensitivity of questionnaires and patient selection, and tailored educational interventions should be considered.

Development and testing of a specific quality-of-life questionnaire for knee and hip osteoarthritis: OAKHQOL (OsteoArthritis of Knee Hip Quality Of Life).

OBJECTIVE: To design a quality-of-life (QOL) instrument specific for patients with knee or hip osteoarthritis and to assess its validity and reproducibility. METHODS: One-on-one or group interviews were conducted with 79 patients and 28 healthcare professionals. Of the 80 potential items identified from the results, 46 were selected based on their content and were used to develop version 1 of the OsteoArthritis of Knee and Hip Quality of Life Scale (OAKHQOL). The psychometric characteristics of the scale were evaluated in patients who met Altman's criteria for knee or hip osteoarthritis. RESULTS: Based on the results of psychometric analyses in 263 patients, three items were excluded, leaving 43 items in the final version (2.3) of the OAKHQOL. Principal components analysis identified four domains: physical activities, mental health, social functioning, and social support. A pain domain was individualized later. Construct validity, reproducibility, and discriminating power of the domains were satisfactory. Standardized response means after joint replacement surgery were close to 1 for the pain and physical functioning domains and equal to 0.7 for the mental health domain, indicating good sensitivity to change. CONCLUSIONS: The OAKHQOL is the first QOL tool specifically dedicated to lower-limb osteoarthritis. It captures specific aspects of QOL in patients with knee or hip osteoarthritis and exhibits psychometric properties consistent with use in longitudinal studies.

OAKHQOL: a new instrument to measure quality of life in knee and hip osteoarthritis.

OBJECTIVE: To develop a questionnaire with which to measure quality of life (QoL) in patients with knee and hip osteoarthritis (OA). STUDY DESIGN AND SETTING: Thirty-two caregivers and 96 OA patients were interviewed individually (using cognitive and face-to-face techniques) and in focus groups. A group of experts working independently at first and then consensually used the interview transcripts to generate a 46-item questionnaire. RESULTS: Analysis of questionnaires completed by 263 patients with hip or knee OA resulted in the exclusion of three items (two because of low reliability and one because of a low response rate). Principal component analysis revealed four factors: physical activity, mental health, social functioning, and social support. A pain dimension was individualized. Preliminary testing showed the reliability of the five dimensions to be satisfactory (intraclass correlation coefficients: 0.70-0.85), construct validity was adequate when correlated with the SF36 (Spearman correlation coefficients: 0.43-0.75), and discrimination was satisfactory. The osteoarthritis knee and hip quality of life questionnaire (OAKHQOL) consists of 43 items in five dimensions and three independent items. CONCLUSION: The OAKHQOL is the first specific knee and hip OA quality of life instrument. Its development followed an a priori structured strategy to ensure content validity. It meets psychometric requirements for validity and reliability.

Positive effect of patient education for hip surgery: a randomized trial.

The current trial compared patient education before total hip arthroplasty with the usual verbal information. A randomized, controlled 24-month prospective single-center study was done. Patients scheduled for a first elective total hip arthroplasty for primary hip osteoarthritis were enrolled. All patients were given the usual information and an information leaflet and completed a self-evaluation questionnaire (Spielberger State and Trait Anxiety Inventory). The patients were assigned randomly to two groups: Group 1 attended a collective multidisciplinary information session 2 to 6 weeks before surgery and the control group did not attend. All patients completed another State Anxiety Inventory just before surgery and then 1 and 7 days after surgery. One hundred patients were randomized. Forty-eight attended the collective information session. Patients receiving education were significantly less anxious just before surgery than patients in the control group, in linear regression after adjustment for gender, trait and state anxiety at baseline, depression score, and health assessment questionnaire score. They experienced less pain before surgery and were able to stand sooner. However, the trend toward lower anxiety scores was not statistically significant after surgery. Patient education decreases preoperative anxiety and pain in patients having hip surgery.