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Chloe Comarmond,
Christian Pagnoux,
Mehdi Khellaf,
Jean-François Cordier,
Mohamed Hamidou,
Jean-François Viallard,
François Maurier,
Stéphane Jouneau,
Boris Bienvenu,
Xavier Puéchal, [......],
Olivier Fain,
Bertrand Godeau, Raphaèle Seror,
Bertrand Dunogué,
Alfred Mahr,
Philippe Guilpain,
Pascal Cohen,
Achille Aouba,
Luc Mouthon,
Loïc Guillevin
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ABSTRACT: OBJECTIVE: Earlier studies on eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome; EGPA), with limited patient numbers and follow-up durations, demonstrated that ANCA+ and ANCA- patients differed clinically at diagnosis but not in their outcomes. Our aims were to describe the main characteristics of a larger patient cohort and their long-term outcomes. METHODS.: Retrospective study on EGPA patients entered into the FVSG database and satisfying the American College of Rheumatology and/or Chapel Hill criteria. Patient characteristics and outcomes were compared according to ANCA status and year of diagnosis. RESULTS.: We identified 383 patients diagnosed between 1957 and June 2009 (128 [33.4%] diagnosed ≤1996) and followed for 66.8 ± 62.5 months. At diagnosis, mean age was 50.3 ± 15.7 years and 91.1% had asthma (since 9.3 ± 10.8 years). Main manifestations included peripheral neuropathy (51.4%), ENT signs (48.0%), skin lesions (39.7%), lung infiltrates (38.6%) and cardiomyopathy (16.4%). Among the 348 whose sera were tested at diagnosis for ANCA, the 108 (31.0%) ANCA+ patients had significantly more frequent ENT manifestations, peripheral neuropathy and/or renal involvement, but less frequent cardiac manifestations than ANCA- patients. Vasculitis relapses occurred in 35.2% of ANCA+ versus 22.5% of ANCA- patients (P = 0.01) and 5.6% versus 12.5%, respectively, died (P <0.05). Five-year relapse-free survival was 58.1% [95% CI; 45.6-68.6] for ANCA+ and 67.8% [95% CI; 59.8-74.5] for ANCA- patients (P = 0.35). Multivariable analysis identified cardiomyopathy, older age and diagnosis ≤1996 as independent risk factors for death, and lower eosinophil count at diagnosis as predictive of relapse. CONCLUSION.: EGPA patients differ according to their ANCA status, as do their long-term outcomes. Although EGPA relapses remain frequent, mortality has declined, at least since 1996. © 2012 American College of Rheumatology.
Arthritis & Rheumatism 10/2012; · 7.87 Impact Factor
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ABSTRACT: The beautification of data is the process of reporting results of a research in a way that does not correspond to reality, in order to present them in a more favorable or attractive way. The border between errors due to methodological ignorance, embellishment of reality and fraud is sometimes difficult to determine. It is the intentional nature and the repetition of these "arrangements" that are the real switch to deliberate fraud. The emergence of regulatory procedures, such as clinical trial registries, "reporting guidelines", improvement of "peer review" and data sharing, are some of the measures used to fight against and improve transparency of clinical research.
La Presse Médicale 07/2012; 41(9 Pt 1):835-40. · 0.67 Impact Factor
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Raphaèle Seror,
Hendrika Bootsma,
Simon J Bowman,
Thomas Dörner,
Jacques-Eric Gottenberg,
Xavier Mariette,
Manel Ramos-Casals,
Philippe Ravaud,
Elke Theander,
Athanasios Tzioufas,
Claudio Vitali
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ABSTRACT: Lymphocytic infiltration of different exocrine and non-exocrine epithelia is the pathological hallmark of primary Sjögren's syndrome, whereas involvement of salivary and lachrymal glands with the clinical counterpart of dry eye and dry mouth are the predominant features of the disease, together with fatigue and musculoskeletal pain. In addition, systemic manifestations, like arthritis, skin vasculitis, peripheral neuropathy, glomerulonephritis, may also be present in a consistent number of patients. As result, clinical features in SS can be divided into two facets: the benign subjective but disabling manifestations such as dryness, pain and fatigue, and the systemic manifestations. In the past decades, a core set of domains, which included sicca symptoms, objective measurements of tear and saliva production, fatigue, quality of life, disease activity and damage was indicated as essential for outcome assessment in this disorder. Afterwards, great efforts have been made to develop valid tools for the assessment of different domains. Specific questionnaires such as the Profile of Fatigue and Discomfort (PROFAD) and Sicca Symptoms Inventory (SSI) have been proposed as dedicated tools for the evaluation of patients symptoms, whereas different composite indexes have been suggested for the assessment of disease activity and damage. Some of these preliminary studies served as bases of an international project supported by EULAR, aimed at developing two consensus disease activity indexes: the EULAR Sjögren's Syndrome Patients Reported Index (ESSPRI), and the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI), a systemic activity index to assess systemic manifestations. A detailed and critical review of all these indexes is provided in this article. Both EULAR indexes showed, in recent studies, to be feasible, valid, and reliable instruments. After their final validation, which is currently in process, they could be used as consensus outcome criteria in therapeutic trials and in clinical practice.
Journal of Autoimmunity 02/2012; 39(1-2):97-102. · 7.37 Impact Factor
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ABSTRACT: In primary Sjögren’s syndrome (SS), clinical features can be divided into two facets: the benign subjective but disabling
manifestations such as dryness, articular and muscular pain, and fatigue, and the systemic manifestations such as synovitis,
vasculitis, skin, lung, renal and neurological involvement, or lymphoma. Great efforts have been made to develop valid activity
indexes needed to assess the effectiveness of new therapies. First, for evaluation of patients’ symptoms: the Profile of Fatigue
and Discomfort (PROFAD) and Sicca Symptoms Inventory (SSI) then for systemic features: the SS Disease Activity Index (SSDAI)
and Sjögren’s Systemic Clinical Activity Index (SCAI). The development of these indexes served as bases of an international
collaborative project promoted by European League Against Rheumatism (EULAR). Thirty-nine primary SS experts were involved
in the development of these two consensus disease activity indexes: the EULAR Sjögren’s Syndrome Patients Reported Index (ESSPRI),
a patient-administered questionnaire to assess subjective features, and the EULAR Sjögren’s Syndrome Disease Activity Index
(ESSDAI), a systemic activity index to assess systemic complications. Both indexes have good correlations with existing scores
and also global evaluation of disease activity by physician for ESSDAI and by patient for ESSPRI. In addition, ESSDAI had
a good sensitivity to change and detects changes more accurately, when compared to other scores. These both indexes are simple
and aimed to be used for both clinical trials and clinical practice. They are currently being validated for that purpose.
KeywordsPrimary Sjögren’s syndrome-Disease activity index-Patient-reported outcome-Outcome assessment-Systemic activity
12/2011: pages 443-454;
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Loïc Guillevin,
Alice Bérezné, Raphaèle Seror,
Luis Teixeira,
Jacques Pourrat,
Alfred Mahr,
Eric Hachulla,
Christian Agard,
Jean Cabane,
Philippe Vanhille,
Jean-Robert Harle,
Isabelle Deleveaux,
Luc Mouthon
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ABSTRACT: Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence.
Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC-SSc patients taken as controls.
Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC-SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died.
Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence.
Rheumatology (Oxford, England) 11/2011; 51(3):460-7. · 4.24 Impact Factor
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Rheumatology (Oxford, England) 05/2011; 50(9):1723-5. · 4.24 Impact Factor
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Raphaèle Seror,
Philippe Ravaud,
Xavier Mariette,
Hendrika Bootsma,
Elke Theander,
Arne Hansen,
Manel Ramos-Casals,
Thomas Dörner,
Stefano Bombardieri,
Eric Hachulla, [......],
Valerie Devauchelle,
Salvatore Devita,
Cristina Vollenweider,
Thomas Mandl,
Athanasios Tzioufas,
Steven Carsons,
Alain Saraux,
Nurhan Sutcliffe,
Claudio Vitali,
Simon J Bowman
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ABSTRACT: To develop a score for assessment of patients' symptoms in primary Sjögren's syndrome (SS): the EULAR SS Patient Reported Index (ESSPRI).
Dryness, pain, somatic and mental fatigue were identified as the main symptoms of patients with primary SS, in studies developing the Profile of Fatigue and Discomfort (PROFAD) and Sicca Symptoms Inventory (SSI). It was suspected that a single 0-10 numerical scale for each domain was sufficient to assess these symptoms. These four scales were gathered to form the ESSPRI. 230 patients, from 12 countries completed the ESSPRI, SSI and PROFAD questionnaires and a 0-10 patient global assessment (PGA). Correlations between each symptom and PGA were obtained. Multiple regression modelling, using PGA as 'gold standard' was used to select domains and estimate their weights.
PGA had good correlation with dryness, limb pain, fatigue and mental fatigue (r=0.49-0.59, all p<0.0001), but correlated less well with individual dryness features. In multivariate analysis, dryness, limb pain and fatigue, but not mental fatigue, were significantly associated with PGA; weights derived from the regression were identical for these three domains. Thus, ESSPRI was redefined as the mean of the three scales: dryness, limb pain and fatigue. Lastly, ESSPRI significantly correlated with PGA (r=0.70), PROFAD (r=0.73) and SSI (r=0.66).
ESSPRI is a very simple index designed to measure patients' symptoms in primary SS. It has good construct validity and is well correlated with SSI and PROFAD. ESSPRI should now be validated for use as an outcome measure in clinical trials.
Annals of the rheumatic diseases 02/2011; 70(6):968-72. · 8.11 Impact Factor
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ABSTRACT: Patients with antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis (AASV) commonly suffer from arthralgias and, sometimes, polyarthritis during disease flares. Although rheumatoid factor (RF) can be detected in up to 37-50% of AASV patients, anti-cyclic citrullinated peptide (anti-CCP) antibodies are rare. Herein, we describe the clinical features of five P-ANCA-positive vasculitis patients, who had persistent and/or high anti-CCP levels and, more importantly, suffered from remittent non-destructive arthralgias and polysynovitis, independently of the vasculitis course and without evidence of RA. Two were initially thought to have RA rather than microscopic polyangiitis and, at AASV diagnosis, all had kidney involvement and three had alveolar hemorrhages. With a median follow-up of 30 months, one suffered vasculitis relapses, preceded by polysynovitis, and others had remittent arthralgias and polysynovitis, while their vasculitides remained in remission. None of these patients had radiological destructive arthritis. We discuss the challenge of diagnosing these patients positive for anti-CCP and ANCA, and with dominant articular manifestations. AASV patients with anti-CCP antibodies may experience relapsing polysynovitis and non-erosive polyarthritis prior to vasculitis flares, but also independently of the vasculitis course, which is uncommon in AASV, and might represent a small subgroup of AASV patients.
Joint, bone, spine: revue du rhumatisme 12/2010; 77(6):604-7. · 2.25 Impact Factor
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Raphaèle Seror,
Christian Pagnoux,
Marc Ruivard,
Isabelle Landru,
Denis Wahl,
Sophie Rivière,
Solveig Aussant,
Alfred Mahr,
Pascal Cohen,
Luc Mouthon,
Loïc Guillevin
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ABSTRACT: To study the efficacy of rescue treatment strategies and outcomes in patients with Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) not achieving remission with first-line induction with corticosteroids (CS) and intravenous cyclophosphamide (CYC).
159 eligible patients in the Wegener's Granulomatosis-Entretien (WEGENT) trial newly diagnosed with systemic or renal WG or MPA with ≥ 1 poor prognosis factors were included in this prospective study. Rescue treatment strategies and outcomes in patients with induction-refractory disease were analysed and patient characteristics at diagnosis were compared with those of induction-responders.
Most patients (n=126, 79.2%) achieved remission; 1 stopped induction because of allergy and 32 were induction-refractory (24 WG and 8 MPA); 11 died rapidly within a median of 2.5 months, 6 of uncontrolled disease, 1 of an infectious complication and 4 of both. Treatment was discontinued in 1 patient with MPA with end-stage renal disease. Induction was switched to oral CYC in 20 patients, combined with infliximab in 1; 15 (75%) achieved remission or low disease activity state, 3 subsequently died of uncontrolled disease and 2 entered remission using several other agents including biological agents. Alveolar haemorrhage and a creatinine level >200 μmol/l were independently associated with induction-refractory disease. Among patients with induction-refractory disease, massive alveolar haemorrhage was associated with higher mortality.
Switching to oral CYC can be an effective rescue treatment for patients with systemic forms of WG or MPA who fail to achieve remission with first-line CS and intravenous CYC. However, a more rapidly effective regimen remains to be identified for most severely affected patients whose outcomes can be rapidly fatal.
Annals of the rheumatic diseases 12/2010; 69(12):2125-30. · 8.11 Impact Factor
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Achille Aouba,
Sophie Georgin-Lavialle,
Christian Pagnoux,
Nicolas Martin Silva,
Amédée Renand,
Françoise Galateau-Salle,
Sophie Le Toquin,
Henri Bensadoun,
Frederique Larousserie,
Stéphane Silvera,
Nicole Provost,
Sophie Candon, Raphaèle Seror,
Mathilde de Menthon,
Olivier Hermine,
Loïc Guillevin,
Boris Bienvenu
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ABSTRACT: Erdheim-Chester disease (ECD) pathophysiology remains largely unknown. Its treatment is not codified and usually disappointing. Interferon (IFN)-α therapy lacks efficacy for some life-threatening manifestations and has a poor tolerance profile. Because interleukin (IL)-1Ra synthesis is naturally induced after stimulation by IFN-α, we hypothesized that recombinant IL-1Ra (anakinra) might have some efficacy in ECD. We treated 2 patients who had poor tolerance or contraindication to IFN-α with anakinra as a rescue therapy and measured their serum C-reactive protein, IL-1β, IL-6, and monocytic membranous IL-1α (mIL-1α) levels before, under, and after therapy. Another untreated ECD patient and 5 healthy subjects were enrolled as controls. After treatment, fever and bone pains rapidly disappeared in both patients, as well as eyelid involvement in one patient. In addition, retroperitoneal fibrosis completely or partially regressed, and C-reactive protein, IL-6, and mIL-1α levels decreased to within the normal and control range. Beside injection-site reactions, no adverse event was reported. Therefore, our results support a central role of the IL-1 network, which seemed to be overstimulated in ECD. Its specific blockade using anakinra thereby opens new pathophysiology and therapeutic perspectives in ECD.
Blood 11/2010; 116(20):4070-6. · 9.90 Impact Factor
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ABSTRACT: To study how corticosteroid therapy is planned and described in reports of systemic disease trials and estimate the impact of the between-arm difference in corticosteroid dose on treatment effect.
We performed a systematic review of PubMed and Cochrane databases on all reports of randomized systemic disease trials with corticosteroids as a cointervention. Data were extracted on the trial characteristics and results, planning of corticosteroid use, and dose. Success rates were adjusted for corticosteroid use for studies with available data and a binary outcome. Because the exact impact of between-arm differences in corticosteroid dose on success rates is unknown, we tested different values for the impact of a difference of 1 unit (1 mg for daily dosage or 250 mg for cumulative dose at the end of the trial).
A total of 139 trials were identified, including 79 investigating lupus and 30 investigating vasculitis. Planned management of corticosteroid use was specified in 101 reports (72.7%), with a fully described tapering scheme in 33 (23.7%). Corticosteroid consumption for each arm was given in 60 reports (43.2%), with a comparison of daily or cumulative dosage at the end of the trial in 32 (23.0%). An attempt to adjust for corticosteroid use was described in 2 (1.5%). With a value of 2.5% for the impact of a 1-unit difference in corticosteroid dose, adjustment yielded changes in success rate differences exceeding 10% in 11 (46%) of the 24 reports analyzed.
For systemic disease trials, use of corticosteroids as a cointervention is often inadequately planned and reported and could affect treatment effect.
Arthritis care & research. 07/2010; 62(7):1002-8.
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ABSTRACT: To assess and compare the sensitivity to change of the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (ESSDAI) with that of other primary Sjögren's syndrome (SS) disease activity indexes.
We abstracted 96 patient profiles, including data on 3 successive visits (visits 1-3), from the medical charts of patients with primary SS. Patient profiles were scored with the ESSDAI, SS Disease Activity Index (SSDAI), and Sjögren's Systemic Clinical Activity Index (SCAI). Thirty-nine experts assessed 5 profiles for whether disease activity had improved, worsened, or remained stable at visits 2 and 3.
For improved patients, the standardized response means (SRMs) for all scores did not differ, and ranged from -1.08 to -1.38 between visits 1 and 2 and from -0.50 to -0.76 between visits 2 and 3. For patients with worsened activity, the SRMs between visits 1 and 2 and between visits 2 and 3 were +0.46 and +1.10 for the ESSDAI, -0.03 and +0.79 for the SSDAI, and +0.17 and +1.02 for the SCAI, respectively. For patients with stable activity, the SRMs between visits 1 and 2 and between visits 2 and 3 were 0.00 and -0.13 for the ESSDAI, -0.44 and -0.11 for the SSDAI, and -0.36 and +0.34 for the SCAI, respectively.
For patients with improved activity, the 3 disease activity indexes showed similar, large sensitivity to change. However, the ESSDAI seemed to detect changes in activity more accurately than other disease activity indexes. Notably, for patients with stable activity, the ESSDAI did not show erroneous improvement.
Arthritis care & research. 04/2010; 62(4):551-8.
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ABSTRACT: Periostitis (periosteal new bone formation) is a rare manifestation of systemic necrotizing vasculitis.
We searched the French Vasculitis Study Group (FVSG) database, established in 1980 and containing the data on 1762 patients with a systemic necrotizing vasculitis, i.e., polyarteritis nodosa (PAN), microscopic polyangiitis, Churg-Strauss syndrome or Wegener's granulomatosis, for those with periostitis. Herein, we describe their characteristics and outcomes.
Only 4 patients with periostitis were identified. All had limited and localized PAN-like vasculitis, without poor prognosis factors. Periostitis was painful and localized to the lower limbs in all of them and associated with local myositis in 2. Pain and inflammation resolved under corticosteroids in all, but relapses or corticosteroid-dependence ultimately required the adjunction of an immunosuppressant for 3. With a median follow-up of 10 [range: 4-11] years, only 1 developed peripheral neuropathy and none progressed to a more systemic form of vasculitis, i.e., with severe visceral involvement.
Physicians should be aware that periostitis, usually localized, is a potential manifestation of systemic necrotizing vasculitis, especially polyarteritis nodosa. The overall long-term outcome of these patients is good, but relapses or corticosteroid-dependence is frequent.
La Presse Médicale 03/2010; 39(7-8):e165-73. · 0.67 Impact Factor
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ABSTRACT: Previous studies of polyarteritis nodosa (PAN) included patients with microscopic polyangiitis, because these entities were not distinguished prior to the Chapel Hill Consensus Conference (CHCC). This study was undertaken to describe the main characteristics of and long-term outcomes in patients with well-characterized PAN diagnoses.
We conducted a systematic retrospective study of 348 patients who were diagnosed as having PAN between March 1963 and October 2005, were registered in the French Vasculitis Study Group database, and satisfied the American College of Rheumatology and CHCC criteria. Patient characteristics and outcomes were analyzed and compared according to hepatitis B virus (HBV) status.
At diagnosis, the mean +/- SD age was 51.2 +/- 17.3 years. The most frequent findings were general symptoms (93.1%), neurologic manifestations (79%), skin involvement (49.7%), abdominal pain (35.6%), and hypertension (34.8%); 66.2% had renal artery microaneurysms; 70.1% had histologically proven PAN. Patients with HBV-related PAN (n = 123) had more frequent peripheral neuropathy, abdominal pain, cardiomyopathy, orchitis, and hypertension compared with patients with non-HBV-related PAN (n = 225). During a mean +/- SD followup of 68.3 +/- 63.5 months, 76 patients (21.8%) relapsed (63 with non-HBV-related PAN [28%] versus 13 with HBV-related PAN [10.6%]; P < 0.001); 86 patients (24.7%) died (44 with non-HBV-related PAN [19.6%] versus 42 with HBV-related PAN [34.1%]; P = 0.003). Five-year relapse-free survival rates were 59.4% (95% confidence interval [95% CI] 52.6-67.0) versus 67.0% (95% CI 58.5-76.8) for non-HBV-related PAN and HBV-related PAN, respectively. Multivariate analysis retained age >65 years, hypertension, and gastrointestinal manifestations requiring surgery or at least consultation with a surgeon as independent predictors of death, whereas patients with cutaneous manifestations or non-HBV-related PAN had a higher risk of relapse.
Our findings indicate that the rate of mortality from PAN remains high, especially for the elderly, and relapses do occur, particularly in patients with non-HBV-related PAN with cutaneous manifestations.
Arthritis & Rheumatism 02/2010; 62(2):616-26. · 7.87 Impact Factor
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ABSTRACT: To determine the frequency and risk factors of venous thromboembolic events (VTE) in Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and, the so far unstudied, Churg-Strauss syndrome (CSS) and polyarteritis nodosa (PAN).
Retrospective, systematic analysis and comparisons were made between the characteristics of patients in the VTE group and non-VTE group. 1130 patients with WG, MPA, CSS or PAN were identified from the French Vasculitis Study Group cohort.
During a mean follow-up of 58.4 (45.8) months, 83 VTE occurred in 74 (6.5%) patients, with a median vasculitis-VTE diagnosis interval of 5.8 months (-3 to +156). VTE occurred in seven of 285 (2.5%) patients with PAN, 19 of 232 (8.2%) with CSS, 30 of 377 (8%) with WG and 18 of 236 (7.6%) with MPA. Multivariate analysis retained age, male sex or previous VTE or stroke with motor deficit as being associated with a higher VTE risk. The adjusted odds ratio (95% confidence interval) for VTE was 2.88 (1.27 to 6.50) for patients with WG, MPA or CSS compared with PAN (p = 0.01).
Our results suggest that, like WG and MPA, patients with CSS are at a greater risk of VTE, than those with PAN. The reasons for this difference remain to be elucidated.
Annals of the rheumatic diseases 12/2008; 68(4):564-7. · 8.11 Impact Factor
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ABSTRACT: OBJECTIVE: Recommended outcome measures in osteoarthritis are standardised scales identical for each patient. As patient-specific scales are of increasing interest when considering patient priorities in outcome assessment, this study aims to validate individualised forms of the Western Ontario and McMaster Universities osteoarthritis index (WOMAC) function subscale. PATIENTS AND METHODS: WOMAC function subscale data were prospectively obtained from 1218 outpatients with hip or knee osteoarthritis requiring non-steroidal anti-inflammatory drugs. Patients also rated the importance to remove disability in each activity of the WOMAC function subscale, and selected the five activities they considered the most important to be improved upon. After treatment, patients again completed the WOMAC function subscale. Several individualisation methods were evaluated: methods whereby the score of each item is multiplied by, or added to, its importance, and methods based on the five most important activities (WOMAC top 5). Psychometric properties of individualised scales were compared to those of the WOMAC function subscale. RESULTS: The missing data rate was 11%, 13% and 2% for the WOMAC function, its individualised forms and the WOMAC top 5, respectively. Combining severity and importance of each item did not improve the properties of the scales. The WOMAC top 5 was the most responsive scale (standardised response mean: 0.96 vs 0.80, p<0.001). CONCLUSION: Because of its better responsiveness, ease of use, low missing data rate and ability to highlight patient priorities, the WOMAC top 5 could be an interesting tool in therapeutic evaluation in hip or knee osteoarthritis.
Annals of the rheumatic diseases 04/2008; · 8.11 Impact Factor
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ABSTRACT: Rheumatoid arthritis (RA) is the most common inflammatory joint disease. Even though, physiopathology of rheumatoid arthritis remains unclear, the presence of circulating immune complexes and rheumatoid factors had led to the use of plasma exchange (PE). Even though PE procedures have evolved over the last decades, their indications and use in RA must be replaced in the context of the evolution of disease-modifying anti-rheumatic drugs. Results of PE and leukapheresis were disappointing in patients with severe and resistant RA. Conversely, immunoadsorption, and particularly over a Staphylococcus aureus protein A column, has resulted in some good responses in refractory RA patients. But, the emergence of effective biologics has clearly restrained their use and limited their indications to some rare patients with refractory and severe RA.
Transfusion and Apheresis Science 05/2007; 36(2):195-9. · 1.25 Impact Factor
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Raphaèle Seror,
Christelle Sordet,
Loic Guillevin,
Eric Hachulla,
Charles Masson,
Marc Ittah,
Sophie Candon,
Véronique Le Guern,
Achille Aouba,
Jean Sibilia,
Jacques-Eric Gottenberg,
Xavier Mariette
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ABSTRACT: To investigate the safety and efficacy of rituximab (RTX) for systemic symptoms in patients with primary Sjögren's syndrome (pSS), and changes in B cell biomarkers.
The records of 16 patients with pSS according to the American European consensus group criteria were reviewed retrospectively.
Patients, all women, had a median age of 58.5 (range 41-71) years and a disease duration of 9.5 (range 0-25) years. RTX was prescribed for lymphoma (n = 5), refractory pulmonary disease with polysynovitis (n = 2), severe polysynovitis (n = 2), mixed cryoglobulinaemia (n = 5), thrombocytopenia (n = 1) and mononeuritis multiplex (n = 1). The median follow-up duration was 14.5 (range 2-48) months. Three patients experienced adverse events, including one mild serum sickness-like reaction with the presence of human antichimeric antibodies. Efficacy of treatment was observed in 4 of 5 patients with lymphomas and in 9 of 11 patients with systemic involvement. Dryness was improved in only a minority of patients. Corticosteroid dose was reduced in 11 patients. RTX induced decreased rheumatoid factor, gamma-globulin and beta2-microglobulin levels, and the level of B cell activating factor of the tumour necrosis factor family (BAFF) increased concomitantly with B cell depletion. Five patients were re-treated, with good efficacy and tolerance, except for one with probable serum sickness-like reaction.
This study shows good efficacy and fair tolerance of RTX for systemic features. In addition, RTX allows for a marked reduction in corticosteroid use. Except for BAFF, the level of which increases, serum B cell biomarker levels decrease after taking RTX. Controlled trials should be performed to confirm the efficacy of RTX in pSS.
Annals of the Rheumatic Diseases 04/2007; 66(3):351-7. · 8.73 Impact Factor
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ABSTRACT: Wegener granulomatosis (WG) is an antineutrophil cytoplasmic antibody (ANCA)-associated granulomatous vasculitis of small and medium-sized vessels. This vasculitis involves mainly the upper and lower respiratory tracts and kidneys, although WG may affect any organ. Central nervous system (CNS) involvement is an uncommon manifestation of WG, reported in 7%-11% of patients. Three major mechanisms have been incriminated as causing CNS disease in WG: contiguous invasion of granuloma from extracranial sites, remote intracranial granuloma, and CNS vasculitis. Herein we describe 6 patients with WG-related CNS involvement, 2 of whom had chronic hypertrophic pachymeningitis, 3 with pituitary involvement, and 1 with cerebral vasculitis. CNS involvement was present at disease onset in 2 patients and occurred 5-18 years after WG diagnosis in the remaining 4. Based on these observations and a review of the literature, we discuss the pathogenic mechanisms, clinical features, imaging findings, treatment, and outcome of meningeal, pituitary, and vascular involvement, with an emphasis on differential diagnoses, prognosis, and therapeutic management of WG-related CNS involvement.
Medicine 02/2006; 85(1):54-65. · 4.35 Impact Factor
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ABSTRACT: BACKGROUND: The Health Assessment Questionnaire Disability Index (HAQ-DI) is the most widely used measure of function in rheumatoid arthritis (RA). OBJECTIVE: To evaluate individualised forms of the HAQ-DI and thus enhance the incorporation of patients' views in outcome assessment. PATIENTS AND METHODS: HAQ-DI data were prospectively obtained from 370 outpatients with RA treated with leflunomide over a 6-month period. At baseline and final visits, patients had to rate the importance they attached to each activity addressed by the 20 HAQ-DI items, and to select the five activities they considered the most important. Different individualised scales were evaluated: scales preserving all domains, in which the score for each item is multiplied by or added to its importance; and scales involving, for each patient, only the five most important items. The psychometric properties of these scales were compared with those of the HAQ-DI. RESULTS: For each HAQ-DI item, severity and importance scores were weakly correlated. Scores for all individualised scales were highly correlated with the HAQ-DI score (r(s)>0.75). All scales had a good internal consistency (Cronbach's alpha 0.87-0.88). Compared with the HAQ-DI, individualised scales did not have better sensitivity to change (standardised response mean 0.64-0.69 vs 0.74). CONCLUSION: Individualised scales have similar properties to the HAQ-DI. However, individualised questionnaires measuring importance gave complementary information to the measure of disability. Individualisation is probably not needed for group assessment in all randomised controlled trials but, the use of individualised questionnaires may be clinically relevant for individual patients with RA.
Ann Rheum Dis.
