Recommendations for the assessment and optimization of adherence to disease-modifying drugs in chronic inflammatory rheumatic diseases: A process based on literature reviews and expert consensus.

BACKGROUND: Adherence to treatment is a key issue in chronic inflammatory rheumatic diseases (CIRDs). OBJECTIVE: To develop recommendations to facilitate in daily practice, the management of non-adherence to disease-modifying drugs in patients with rheumatoid arthritis, spondyloarthritis, psoriatic arthritis, connective tissue diseases or other CIRDs. METHODS: The process comprised (a) systematic literature reviews of methods (including questionnaires) to measure non-adherence, risk factors for non-adherence and efficacy of targeted interventions; (b) development of recommendations through consensus of 104 rheumatologist and nurse experts; (c) assessment of agreement and ease of applicability (1-5 where 5 is highest) by the 104 experts. RESULTS: (a) Overall, 274 publications were analysed. (b) The consensus process led to 5 overarching principles and 10 recommendations regarding adherence. Key points include that adherence should be assessed at each outpatient visit, at least using an open question; questionnaires and hydroxychloroquine blood level assessments may also be useful. Risk factors associated to non-adherence were listed. Patient information and education, and patient/physician shared decision, are key to optimize adherence. Other techniques such as formalized education sessions, motivational interviewing and cognitive behavioral therapy may be useful. All health professionals can get involved and e-health may be a support. (c) The agreement with the recommendations was high (range of means, 3.9-4.5) but ease of applicability was lower (2.7-4.4). CONCLUSIONS: Using an evidence-based approach followed by expert consensus, this initiative should improve the assessment and optimization of adherence in chronic inflammatory rheumatic disorders.

Illness Identity in Adults with a Chronic Illness.

The present study examines the concept of illness identity, the degree to which a chronic illness is integrated into one's identity, in adults with a chronic illness by validating a new self-report questionnaire, the Illness Identity Questionnaire (IIQ). Self-report questionnaires on illness identity, psychological, and physical functioning were assessed in two samples: adults with congenital heart disease (22-78 year old; n = 276) and with multisystem connective tissue disorders (systemic lupus erythematosus or systemic sclerosis; 17-81 year old; n = 241). The IIQ could differentiate four illness identity states (i.e., engulfment, rejection, acceptance, and enrichment) in both samples, based on exploratory and confirmatory factor analysis. All four subscales proved to be reliable. Rejection and engulfment were related to maladaptive psychological and physical functioning, whereas acceptance and enrichment were related to adaptive psychological and physical functioning. The present findings underscore the importance of the concept of illness identity. The IIQ, a self-report questionnaire, is introduced to measure four different illness identity states in adults with a chronic illness.

EQ-5D studies in musculoskeletal and connective tissue diseases in eight Central and Eastern European countries: a systematic literature review and meta-analysis.

EQ-5D is becoming the preferred instrument to measure health-state utilities involved in health technology assessment. The objective of this study is to assess the state of EQ-5D research in musculoskeletal disorders in 8 Central and Eastern European countries and to provide a meta-analysis of EQ-5D index scores. Original research articles published in any language between Jan 2000 and Sept 2016 were included, if they reported any EQ-5D outcome from at least two musculoskeletal patients from Austria, Bulgaria, the Czech Republic, Hungary, Poland, Romania, Slovakia, or Slovenia. Risk of bias was assessed with the Cochrane Collaboration's tool. Twenty-nine articles (5992 patients) were included on rheumatoid arthritis (n = 7), osteoporosis (n = 5), chronic pain (n = 5), osteoarthritis (n = 4), ankylosing spondylitis (n = 2), psoriatic arthritis (n = 2), total hip replacement (n = 2), and scleroderma (n = 2). Low back pain was under-represented, while studies in neck pain, systemic lupus erythematosus, gout, and childhood disorders were lacking. EQ-5D index scores were reported in 24 studies, while the version of the instrument and the value-set was not specified in 41% and 46% of the articles, respectively. Meta-analysis was performed on 24 disease states involving 6876 observation points. Intervention effect was reported in 22 subgroups, out of which risk of bias was low in 41%. This review provides recommendations to improve reporting standards of EQ-5D results and highlights potential areas for future research. Coordinated research in conditions with greatest public health impact as well as a development of a regional value-set could provide locally relevant health-state utilities that are transferable among countries within the region.

[Connective tissue diseases in adolescents]. / Kollagenosen bei Jugendlichen.

In this article we provide a brief review of systemic lupus erythematosus, juvenile dermatomyositis, systemic scleroderma, and mixed connective tissue disease in adolescents. As skin manifestations often belong to the presenting symptoms and may have a significant impact on the quality of life, dermatologists play an important role in the management of patients with connective tissue diseases. Early diagnosis and therapy onset are crucial for the patients' long-term outcome.

Quality of life in Mexican patients with primary neurological or musculoskeletal disabilities.

BACKGROUND: As the population with a disability increases both in number and life expectancy, so does its demand for health services. Very little original research has been done in Mexican populations regarding the effect of disability on quality of life. OBJECTIVES: 1) Assessing the subjective quality of life of patients with neuromusculoskeletal and movement-related function disabilities and, 2) Analyzing the possible effect of the medical condition (diseases of the nervous system vs. diseases of the musculoskeletal system and connective tissue) and its interaction with sex and age. METHODS: In a cross-sectional design 330 participants were recruited from a public institution in Mexico providing specialized outpatient rehabilitation. After signing informed consent they responded a brief sociodemographic questionnaire and the WHOQOL-BREF scale. RESULTS: In general quality of life levels were satisfactory. Female and elderly patients with diseases of the nervous system were particularly affected in the physical and psychological dimensions. Women with diseases of the nervous system also showed a negatively affected social quality of life. Elderly with diseases of the musculoskeletal system and connective tissue scored the lowest in general health perception. CONCLUSIONS: Even though most patients were quite satisfied with their quality of life, not all aspects or cases have such positive perceptions; both the physical and psychological dimensions were particularly negative in female and elderly patients with nervous system diseases. Clinicians would do well to thoughtfully assess patients in these groups and adjust treatment to achieve the greatest possible rehabilitation.

The Italian version of the Systemic Sclerosis Questionnaire: a comparison of quality of life in patients with systemic sclerosis and with other connective tissue disorders.

AIM: Aim of the present study was to measure disability among patients with systemic sclerosis or other connective tissue disorders, using the specific Systemic Sclerosis Questionnaire (SySQ) and the Skindex-17. METHODS: Cross-sectional survey on hospitalized and day-hospital female patients in a dermatological setting, during March-May 2013. Comparison of disability and quality of life scores between patients in the two diagnostic groups. RESULTS: The use of these questionnaires in a clinical setting was well accepted. The levels of disability were slightly greater among women with systemic sclerosis in terms of general and musculoskeletal symptoms, while women with other connective tissue disorders had higher cardiopulmonary scores. The correlation between SySQ and Skindex-17 scores was low-moderate, indicating that the instruments indeed measure related but distinct constructs. CONCLUSION: The Italian version of the SySQ may provide an additional tool for dermatologists, both in the research and clinical setting. Furthermore, its use may be extended to the medical as well as to the nursing clinical practice. Results from SySQ can be very useful for dermatological nursing-care for the implementation of educational plans targeted to patients, with the objective of enabling the patients to self-manage the disability of this severe chronic condition also outside of the strictly clinical setting.

Social persuasion in rheumatology: a randomized trial of testimonials on television in the rheumatology clinic waiting room to increase attendance for multidisciplinary education.

Multidisciplinary self-management programs are important in inflammatory arthritis as adjunctive treatment. Patients often have excuses as to why they do not attend these programs. The purpose of this study was to determine whether an intervention of televised testimonials from rheumatologists and allied health professionals increases attendance at a multidisciplinary education day for rheumatology patients seen in a large university hospital clinic. This was an RCT of intervention: playing televised interviews in the waiting room where rheumatology patients were seen versus no TV. There was a total of 6 months (3 months with and 3 without the televised interview playing). All eligible patients who attended the rheumatology outpatient clinic were then tracked to determine whether they attended a subsequent education day over the next 10 months. The sample size was calculated to have a 15% increase in attendance at the education days. There was a 20% increase in attendees at the multidisciplinary education days for patients who saw the televised testimonials. Sixty-three patients who viewed the testimonials (2.17% of 2,908) attended the education day compared to 39 who did not receive the intervention (1.80% of 2,168); however, the increase was not statistically significant (p = 0.36). Attendance of eligible patients increased using televised testimonials; however, the increase was not significant as the rates of attendance were still very low in both groups. Many eligible patients did not attend the program. Other interventions are necessary to encourage attendance in a multidisciplinary program.

Quality of life as measured by the short-form 36 (SF-36) questionnaire in patients with early systemic sclerosis and undifferentiated connective tissue disease.

OBJECTIVE: To investigate health-related quality of life (HRQOL) in patients affected by early systemic sclerosis (eSSc) and to compare it with that of patients with undifferentiated connective tissue disease (UCTD). METHODS: At baseline, 31 eSSc and 35 UCTD patients underwent clinical evaluation, laboratory investigations, nailfold videocapillaroscopy, echocardiography, and lung function tests. All patients and 40 controls, matched for sex and age completed the Short Form-36 (SF-36) questionnaire and the Health Assessment Questionnaire Disability Index (HAQ-DI). RESULTS: SF-36 scores were significantly lower in eSSc and UCTD patients than in healthy controls as regards the following domains: physical component score (PCS), mental component score (MCS), physical functioning, role-physical, bodily pain, general health and mental health. PCS was negatively correlated to the HAQ-DI (rho -0.59; p=0.0004) and ESR >20 mm/h (rho -0.58; p=0.0006) in eSSc patients. No statistically significant correlation was found between PCS, MCS and HAQ-DI in UCTD patients. Age, sex, disease duration, history of arthritis, low levels of either C3 or C4, a low DLCO (carbon monoxide lung diffusion) and inversion of the E/A ratio were not correlated to PCS and MCS in either eSSc or UCTD patients. CONCLUSION: Many eSSc or UCTD patients perceive they have an impaired quality of life in both physical and mental domains. This condition has to be taken into account by the clinicians involved in the care of these patients.

Audiometric characteristics of two Dutch families with non-ocular Stickler syndrome (COL11A2).

OBJECTIVE: To evaluate hearing impairment and cochlear function in non-ocular Stickler syndrome. STUDY DESIGN: Multifamily study. PATIENTS & METHODS: Ten patients from two different families with non-ocular Stickler syndrome (Stickler syndrome type 3) were included. Six members of the first family and four members of the second family participated in this study. Otorhinolaryngologic examinations were performed. Pure-tone and speech audiograms were obtained. Longitudinal analysis was performed. Psychophysical measurements, including loudness scaling, gap detection, difference limen for frequency and speech perception in noise were administered to assess cochlear function at a deeper level. RESULTS: Affected individuals in the first family were carriers of a heterozygous splice donor mutation in the COL11A2 gene. Affected individuals in the second family were carriers of a novel heterozygous missense mutation in COL11A2. Both families showed bilateral, non-progressive hearing impairment with childhood onset. The severity of the hearing impairment exhibited inter- and intrafamilial variability and was mostly mild to moderate. The results of the psychophysical measurements were similar to those previously published for DFNA8/12 (TECTA) and DFNA13 (COL11A2) patients and thus consistent with an intra-cochlear conductive hearing impairment. This is in line with the theory that mutations in COL11A2 affect tectorial membrane function. CONCLUSION: Hearing impairment in non-ocular Stickler syndrome is characterized by non-progressive hearing loss, present since childhood, and mostly mild to moderate in severity. Psychophysical measurements in non-ocular Stickler patients were suggestive of intra-cochlear conductive hearing impairment.

Assessing dyspnea and its impact on patients with connective tissue disease-related interstitial lung disease.

RATIONALE: Dyspnea is the cardinal symptom in patients with any type of interstitial lung disease (ILD); however, there are limited data on dyspnea among patients with connective tissue disease-related ILD (i.e., CTD-ILD). OBJECTIVES: To explore the utility of two dyspnea instruments (the University of California San Diego Shortness of Breath Questionnaire [UCSD] and the Dyspnea-12 [D-12]) and use their scores to examine the impact of dyspnea on the lives of patients with CTD-ILD. METHODS: Subjects were enrolled from the Autoimmune Lung Database (ALD) at National Jewish Health. Chronbach's alpha was used to assess the internal consistency reliability of the two dyspnea questionnaires. We used the Multi-Dimensional Health Assessment Questionnaire [MDHAQ] as a measure of health status and examined associations between health status and dyspnea by using Pearson product-moment correlation and linear regression. RESULTS: The internal consistency reliability of each of the two dyspnea questionnaires was excellent (alpha=0.9 for each). There were significant correlations between either of the two dyspnea measures and MDHAQ components. While controlling for ILD severity, dyspnea as assessed by the UCSD, was a significant predictor of physical function (p=0.04), psychological well-being (p=0.005), and fatigue (p=0.02); dyspnea as assessed the D-12, was a significant predictor of psychological well-being (p=0.01) and global status (p=0.03). CONCLUSION: Dyspnea significantly affects day-to-day functioning and global well-being in patients with CTD-ILD. The UCSD and D-12 yield meaningful information about these patients that measures of pulmonary physiology cannot. Future studies should examine other performance characteristics of these self-report measures in patients with CTD-ILD.

Curso de reumatologia: aula 7: manifestações neurológicas nas colagenoses / Course of rheumatology: class 7: neurologic manifestations in collagenosis

O propósito deste artigo é focar as principais manifestações neurológicas das doenças difusas do tecido conjuntivo, com ênfase nas suas manifestações clínicas. Os autores discutem as várias complicações do sistema nervoso central e periférico na doença muscular inflamatória (polimiosite e dermatomiosite), policondrite recidivante, esclerose sistêmica, artrite reumatoide, síndrome de Sjõgren, doença mista do tecido conjuntivo (doença de Sharp), lúpus eritematoso sistêmico e síndrome do anticorpo antifosfolipide.

The purpose of this article is to focus on the major nervous system manifestations in connective tissue diseases, with emphasis on their clinica findings. Authors discuss several complications in inflammatory muscle disease (polymyositis an dermatomyositis), relapsing polychondritis, systemic sclarosis, rheumatoid arthritis, Sjõgren syndorme, mixed connective tissue (Sharp disease), systemic lupus erythematosus and antiphospholipid syndrome.

Psychometric properties of a Swedish translation of the VISA-P outcome score for patellar tendinopathy.

BACKGROUND: Self-administrated patient outcome scores are increasingly recommended for evaluation of primary outcome in clinical studies. The VISA-P score, developed at the Victorian Institute of Sport Assessment in Melbourne, Australia, is a questionnaire developed for patients with patellar tendinopathy and the patients assess severity of symptoms, function and ability to participate in sport. The aim of this study was to translate the questionnaire into Swedish and to study the reliability and validity of the translated questionnaire and resultant scores. METHODS: The questionnaire was translated into Swedish according to internationally recommended guidelines for cross-cultural adaptation of self-report measures. The reliability and validity were tested in three different populations. The populations used were healthy students (n = 17), members of the Swedish male national basketball team (n = 17), considered as a population at risk, and a group of non-surgically treated patients (n = 17) with clinically diagnosed patellar tendinopathy. The questionnaire was completed by 51 subjects altogether. RESULTS: The translated VISA-P questionnaire showed very good test-retest reliability (ICC = 0.97).The mean (+/- SD) of the VISA-P score, at both the first and second test occasions was highest in the healthy student group 83 (+/- 13) and 81 (+/- 15), respectively. The score of the basketball players was 79 (+/- 24) and 80 (+/- 23), while the patient group scored significantly (p < 0.05) lower, 48 (+/- 20) and 52 (+/- 19). CONCLUSIONS: The translated version of the VISA-P questionnaire was linguistically and culturally equivalent to the original version. The translated score showed good reliability.

Grupo de calidad de vida en enfermedades del tejido conectivo / Health quality life groups in connective diseases

Introduccion: la necesidad de un abordaje integral bio-psico-social de los pacientes con enfermedad cronica, presenta la dificultad de evaluar adecuadamente las intervenciones psicosociales que se deben realizar, como p. ej: la educacion del paciente basada en principios psiconeuroendocrinoinmunologicos, aplicados en reuniones grupales, llamadas de Calidad de Vida (CV), destinadas a crear y/o mejorar las estrategias de afrontamiento de los pacientes para el manejo de su enfermedad. Objetivos: 1) Evaluar a traves de tests psicometricos la influencia de un tratamiento medico-psicologico integrado 2) Ofrecer en un espacio transicional una instancia de psicoaprendizaje y de manejo del estres cronico. 3) Observar si esto influye en la evolucion de la enfermedad de base, su actividad y requerimientos. Material y metodos: se estudiaron 12 pacientes, de sexo femenino, con edad mediana de 36.8 años. Todas las pacientes habian estado internadas con diagnostico de: 1 paciente artitris reumatoidea (AR), 8 pacientes lupus eritematoso sistemico (LES), 2 pacientes esclerodermia (ESP), 1 paciente fibromialgia (FM). El estado de actividad de la enfermedad fue registrado con los respectivos metodos: AR con el core set del American College of Rheumatology (ACR), LES con SLEDAI, ESP con compromiso de organo, y FM con FIQ. Se evaluo impacto de la enfermedad en la CV de las pacientes con SF36, depresion con Beck Depresion Inventory (BDI), estres con Escala de Holmes modificada. Se registro ademas concurrencia al grupo, cumplimiento del tratamiento internaciones, desarrollo social y actividades fuera del hogar. Resultados: De 12 pacientes 3 (25 por ciento) realizaron el programa en sesiones individuales y 9 (75 por ciento) completaron las sesiones programadas; cinco (41.6 por ciento) pacientes retomaron su actividad laboral. En las 12 pacientes se ha registrado menor numero de reactivacion anual de la enfermedad y cumplen adecuadamente con el tratamiento. Cuatro de ellas han sobrellevado eventos traumaticos sin reactivar la enfermedad. El numero de hospitalizaciones de 12/12 en 1998 fue de 4/12 en los 4 años subsiguientes. La media estadistica del BDI antes del tratamiento fue de 28.92 (DS 3.75) y despues del tratamiento 20.83 (DS 3.66) p<0.005 (AU)

A longitudinal study of autoantibodies against central nervous system tissue and gangliosides in connective tissue diseases.

Our objective was to investigate longitudinally, antibodies against central nervous tissue (anti-CNS) derived from bovine brain and gangliosides GM 1, GD1a, GD1b, and GT1b in 91 patients with connective tissue diseases (systemic lupus erythematosus, n = 38; mixed connective tissue disease, n = 16; primary Sjogren's syndrome, n = 7; progressive systemic sclerosis, n= 13; polymyositis/dermatomyositis, n=4; overlap syndrome, n = 5; undifferentiated connective tissue disease, n = 8). Anti-CNS and anti-ganglioside antibodies, measured by enzyme-linked immunosorbent assay, were found in 73% and 63% of patients, respectively. Anti-CNS positive sera were also reactive in Western blotting in 74% of cases and recognized up to 14 different polypeptides from 29 to 130 kDa. Anti-CNS and anti-ganglioside antibodies reflected only in a limited extent the disease activity. In 27 of 58 patients, anti-CNS antibodies remained positive independently of disease activity and antibody levels did not correlate with the phases of exacerbations. A total of 36 of 60 anti-CNS-positive patients, in contrast to two of 22 anti-CNS-negative patients, had major neuropsychiatric manifestations (P < 0.001). Anti-ganglioside antibodies were not significantly associated with neuropsychiatric manifestations. In conclusion, our longitudinal data suggest that anti-CNS antibodies may be an important marker for the diagnosis of cerebral involvement in connective tissue diseases, but the pathogenic role of these autoantibodies remains to be determined.

Psychological and rheumatic symptoms of women requesting silicone breast implant removal.

In this case series, we describe the psychological and rheumatic symptoms of 52 women requesting breast implant removal. Main outcome measures include psychological evaluation with the Brief Symptom Inventory and the Beck Depression Inventory. Clinical evaluation was performed using a rheumatic symptom survey. Results noted elevation of all Brief Symptom Inventory subscales (highest in Somatization and Obsessive-Compulsive), and the Beck Depression Inventory showed mild depression. Rheumatic survey noted a mean of 12 of 23 symptoms. In conclusion, psychological distress and mild depression are noted in this group, who also may experience rheumatic symptoms of connective tissue disease. There was a moderate positive correlation between rheumatic symptoms and psychological distress.

[Quality of life and functional indices in osteoarthritis]. / Les indices de qualité de vie et les indices fonctionnels dans l'arthrose.

The main Indices of Quality of Life used in rheumatology and their aims and applications are surveyed. Their use in osteoarthritis implies a few issues that are addressed in a critical manner. Indices of Quality of Life are useful to measure deficiency resulting from osteoarthritis by comparison with other rheumatic diseases, as rheumatoid arthritis, connective tissue diseases, fibromyalgia... They are sensitive enough for apraising the domains improved by a radical treatment such as joint replacement. However, their scores are only slightly changing in most of the drug trials, in which the more simple functional indices seem demonstrative enough. Last but no least, they are notably time consuming for both the patient and the physician.

Psychiatric disorders among medical in-patients in an Indian hospital.

Psychiatric symptoms among medical in-patients in an Indian hospital were assessed: the SRQ was used as a screening instrument, and those with probable psychiatric disorders were given the PSE and MSE, for further, detailed assessment. The prevalence of psychiatric disorders was 34%, with a further 15% reporting distressing psychiatric symptoms only. The most frequent complaints were delirium and adjustment disorders. They were largely associated with connective tissue, as well as cardiovascular and endocrine, disorders, and were characterised by depression, worrying and irritability. The reliability of the SRQ varied with the cut-off score, which gave optimal specificity and sensitivity when set at 9.

Alteraciones psiquiátricas observadas en pacientes con enfermedades del tejido conjuntivo que asisten a un centro dermatológico / Psychiatric chianges observed in patients with connective fissue diseases attended in a dermatologic center

Entre las afecciones dermatológicas, las enfermedades del tejido conjuntivo, y en especial el Lupus Eritematoso Sistémico, representan un área importante en la interacción con las disciplinas neuropsiquiátricas. Se han reportado pocos estudios sistemáticos de las alteraciones psiquiátricas en estos pacientes. La presente investigación se realizó con el objeto de estudiar las alteraciones psiquiátricas que se presentan en pacientes con enfermedades del tejido conjuntivo en comparación con un grupo control, seleccionados ambos de entre la población que es atendida en el Centro Dermatológico "Ladislao de la Pascua", de la ciudad de México. La información clínica fue recabada a través de la entrevista psiquiátrica estandarizada, aplicando las reglas del programa ID-CATEGO para integrar sindromáticas y evaluar la concordancia en el diagnóstico clínico. Se encontraron diferencias estadísticamente significativas entre ambos grupos de estudio. Los síndromes más frecuentes en pacientes con colagenopatías fueron; depresión simple, ansiedad general, preocupaciones, irritabilidad, disminución del interés y de la concentración, hipocondriasis y síntomas orgánicos. No se presentaron alteraciones psicóticas en la muestras estudiada, probablemente por el tipo de centro hospitalario donde se verificó el trabajo. El acuerdo diagnóstico entre el juicio clínico y el programa ID-CATEGO mostró un valor medio de Kappa de 0.75. Los autores analizan los hallazgos clínicos, y subrayan la conveniciencia de realizar estudios similares en otras poblaciones