Publications

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216 Publications visible to you, out of a total of 216
Morbidity and mortality in adult polymyositis and dermatomyositis

Abstract (Expand)

Before the use of corticosteroids, the prognosis for polymyositis/dermatomyositis (PM/DM) was extremely poor. To date, although overall prognosis appears to be better, PM and DM are still considered … to be associated with increased morbidity, primarily related to severe muscle weakness and visceral involvement. Recent series underline that only 20% to 40% of treated patients will achieve PM/DM remission, whereas 60% to 80% will experience a polycyclic or chronic, continuous course of the disease. PM/DM further continues to have a great impact on life in medium- and long-term follow-up, as up to 80% of treated patients are still disabled (using Health Assessment Questionnaire scores). The overall mortality ratio in PM/DM patients also remains threefold higher compared with the general population, with cancer, lung, and cardiac complications and infections being the most common causes of deaths. Predictive factors for a poor prognosis in PM/DM patients are older age, involvement of lung and cardiac systems, dysphagia, cancer, and serum myositis-specific antibodies (including coexistent presence of anti-Ro52 and anti-Jo1 antibodies, anti-signal recognition particle antibody, anti-155/140, and anti-CADM-140 antibodies).

Author: I. Marie

Date Published: 2012

Publication Type: Journal

Predictors of survival in a cohort of patients with polymyositis and dermatomyositis: effect of corticosteroids, methotrexate and azathioprine

Abstract (Expand)

INTRODUCTION: The idiopathic inflammatory myopathies are rare diseases for which data regarding the natural history, response to therapies and factors affecting mortality are needed. We performed this … study to examine the effects of treatment and clinical features on survival in polymyositis and dermatomyositis patients. METHODS: A total of 160 consecutive patients (77 with polymyositis and 83 with dermatomyositis) seen at the University of Michigan from 1997 to 2003 were included. Medical records were abstracted for clinical, laboratory and therapeutic data, including initial steroid regimen and immunosuppressive use. State vital records were utilized to derive mortality and cause of death data. Survival was modeled by left-truncated Kaplan-Meier estimation and Cox regression. RESULTS: The 5- and 10-year survival estimates were 77% (95% CI = 66 to 85), and 62% (95% CI = 48 to 73), respectively, and the rates were similar for polymyositis and dermatomyositis. Survival between the sexes was similar through 5 years and significantly lower thereafter for males (10-year survival: 18% male, 73% female; P = 0.002 for 5- to 10-year interval). The sex disparity was restricted to the polymyositis group. Increased age at diagnosis and non-Caucasian race were associated with lower survival. Intravenous versus oral corticosteroid use was associated with a higher risk of death among Caucasians (HR = 10.6, 95% CI = 2.1 to 52.8). Early survival between patients treated with methotrexate versus azathioprine was similar, but survival at 10 years was higher for the methotrexate-treated group (76% vs 52%, P = 0.046 for 5- to 10-year interval). CONCLUSIONS: Patients treated initially with intravenous corticosteroids had higher mortality, which was likely related to disease severity. Both methotrexate and azathioprine showed similar early survival benefits as first-line immunosuppressive drugs. Survival was higher between 5 and 10 years in the methotrexate-treated group, but could not be confirmed in multivariable modeling for the full follow-up period. Other important predictors of long-term survival included younger age, female sex and Caucasian race.

Authors: E. Schiopu, K. Phillips, P. M. MacDonald, L. J. Crofford, E. C. Somers

Date Published: 2012

Publication Type: Journal

Current state of evidence on ’off-label’ therapeutic options for systemic lupus erythematosus, including biological immunosuppressive agents, in Germany, Austria and Switzerland–a consensus report

Abstract (Expand)

Systemic lupus erythematosus (SLE) can be a severe and potentially life-threatening disease that often represents a therapeutic challenge because of its heterogeneous organ manifestations. Only … glucocorticoids, chloroquine and hydroxychloroquine, azathioprine, cyclophosphamide and very recently belimumab have been approved for SLE therapy in Germany, Austria and Switzerland. Dependence on glucocorticoids and resistance to the approved therapeutic agents, as well as substantial toxicity, are frequent. Therefore, treatment considerations will include ’off-label’ use of medication approved for other indications. In this consensus approach, an effort has been undertaken to delineate the limits of the current evidence on therapeutic options for SLE organ disease, and to agree on common practice. This has been based on the best available evidence obtained by a rigorous literature review and the authors’ own experience with available drugs derived under very similar health care conditions. Preparation of this consensus document included an initial meeting to agree upon the core agenda, a systematic literature review with subsequent formulation of a consensus and determination of the evidence level followed by collecting the level of agreement from the panel members. In addition to overarching principles, the panel have focused on the treatment of major SLE organ manifestations (lupus nephritis, arthritis, lung disease, neuropsychiatric and haematological manifestations, antiphospholipid syndrome and serositis). This consensus report is intended to support clinicians involved in the care of patients with difficult courses of SLE not responding to standard therapies by providing up-to-date information on the best available evidence.

Authors: M. Aringer, H. Burkhardt, G. R. Burmester, R. Fischer-Betz, M. Fleck, W. Graninger, F. Hiepe, A. M. Jacobi, I. Kotter, H. J. Lakomek, H. M. Lorenz, B. Manger, G. Schett, R. E. Schmidt, M. Schneider, H. Schulze-Koops, J. S. Smolen, C. Specker, T. Stoll, A. Strangfeld, H. P. Tony, P. M. Villiger, R. Voll, T. Witte, T. Dorner

Date Published: 2012

Publication Type: Journal

Finalisation and validation of the rheumatoid arthritis impact of disease score, a patient-derived composite measure of impact of rheumatoid arthritis: a EULAR initiative

Abstract (Expand)

OBJECTIVE: A patient-derived composite measure of the impact of rheumatoid arthritis (RA), the rheumatoid arthritis impact of disease (RAID) score, takes into account pain, functional capacity, fatigue, … physical and emotional wellbeing, quality of sleep and coping. The objectives were to finalise the RAID and examine its psychometric properties. METHODS: An international multicentre cross-sectional and longitudinal study of consecutive RA patients from 12 European countries was conducted to examine the psychometric properties of the different combinations of instruments that might be included within the RAID combinations scale (numeric rating scales (NRS) or various questionnaires). Construct validity was assessed cross-sectionally by Spearman correlation, reliability by intraclass correlation coefficient (ICC) in 50 stable patients, and sensitivity to change by standardised response means (SRM) in 88 patients whose treatment was intensified. RESULTS: 570 patients (79% women, mean +/- SD age 56 +/- 13 years, disease duration 12.5 +/- 10.3 years, disease activity score (DAS28) 4.1 +/- 1.6) participated in the validation study. NRS questions performed as well as longer combinations of questionnaires: the final RAID score is composed of seven NRS questions. The final RAID correlated strongly with patient global (R=0.76) and significantly also with other outcomes (DAS28 R=0.69, short form 36 physical -0.59 and mental -0.55, p<0.0001 for all). Reliability was high (ICC 0.90; 95% CI 0.84 to 0.94) and sensitivity to change was good (SRM 0.98 (0.96 to 1.00) compared with DAS28 SRM 1.06 (1.01 to 1.11)). CONCLUSION: The RAID score is a patient-derived composite score assessing the seven most important domains of impact of RA. This score is now validated; sensitivity to change should be further examined in larger studies.

Authors: L. Gossec, S. Paternotte, G. J. Aanerud, A. Balanescu, D. T. Boumpas, L. Carmona, M. de Wit, B. A. Dijkmans, M. Dougados, M. Englbrecht, F. Gogus, T. Heiberg, C. Hernandez, J. R. Kirwan, E. M. Mola, M. M. Cerinic, K. Otsa, G. Schett, M. Scholte-Voshaar, T. Sokka, G. von Krause, G. A. Wells, T. K. Kvien

Date Published: 2011

Publication Type: Journal

Patients with polymyositis or dermatomyositis have reduced grip force and health-related quality of life in comparison with reference values: an observational study

Abstract (Expand)

OBJECTIVES: The aims of this study were to investigate hand function in PM and DM patients and compare this with reference values in healthy individuals and also to investigate if hand function correlated … with activity performance and health-related quality of life. METHODS: An observational cross-sectional study was performed in 18 women and 13 men with PM or DM with established disease. Grip force and hand mobility were assessed by Grippit and Escola Paulista de Medicina-Range of Motion scale. Activity performance was measured with myositis activities profile and health-related quality of life by short form-36 (SF-36). RESULTS: Women and men with PM and DM with mean disease duration of 6.8 (5.5) years had a significantly lower grip force than gender- and age-matched healthy individuals (women 71% and men 60%). They also had significantly lower mean values in all dimensions of the health-related quality of life instrument SF-36 compared with the Swedish population. In patients with PM and DM, the grip force correlated significantly with the ability to perform domestic activities. In women with PM and DM, the grip force correlated significantly with the health-related quality of life dimensions vitality and mental health. There were no significant differences between patients with PM and DM regarding grip force, hand mobility, activity performance or health-related quality of life. CONCLUSIONS: Patients with PM or DM have reduced grip force that could influence activity performance and health-related quality of life.

Authors: M. Regardt, E. Welin Henriksson, H. Alexanderson, I. E. Lundberg

Date Published: 2011

Publication Type: Journal

Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), physician global damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI)

Abstract

Not specified

Authors: L. G. Rider, V. P. Werth, A. M. Huber, H. Alexanderson, A. P. Rao, N. Ruperto, L. Herbelin, R. Barohn, D. Isenberg, F. W. Miller

Date Published: 2011

Publication Type: Journal

How well do patient reports reflect adverse drug reactions reported by rheumatologists? Agreement of physician- and patient-reported adverse events in patients with rheumatoid arthritis observed in the German biologics register

Abstract (Expand)

OBJECTIVE: To analyse the validity of patient reports on adverse drug reactions (ADRs) compared with the reports given by the treating physician. METHODS: Patients with RA enrolled in the German biologics … register rheumatoid arthritis observation of biologic therapy (RABBIT) between May 2001 and September 2006 were included in the study. We investigated concordance of reporting and level of agreement between physician- and patient-reported ADRs, taking the physician as gold standard. RESULTS: Data from 4246 patients were analysed. Patients reported on average 1.2 ADRs per patient-year (PY) compared with 0.8 ADRs reported by the physicians (P<0.001). Gastrointestinal disorders were the most frequently reported ADRs by patients (277.8/1000 PYs) and physicians (137.8/1000 PYs), infections were reported with considerably higher frequency by physicians (124/1000 PYs) than by patients (72/1000 PYs). Agreement between patients and physicians (same or similar event reported at the same time) differed according to the nature of the reported ADR. High agreement was found for easily observable, known ADRs (such as alopecia, agreement 76.7%) In contrast, even for some serious ADRs, many patients did not see a connection between the event and the drug taken (e.g. pneumonia, agreement 37.7%). CONCLUSIONS: Patient reports on ADRs are a useful source of information on the safety of new therapies. However, drug surveillance cannot rely on patient reports only, since even life-threatening events were not reported as ADRs by the patients who failed to associate them with the therapy. When coding patient reports on ADRs to a standard coding system, the differences in language and terminology between patients and physicians should be taken into account.

Authors: L. Gawert, F. Hierse, A. Zink, A. Strangfeld

Date Published: 2011

Publication Type: Journal

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