To estimate the frequency of lower urinary tract symptoms (LUTS) and sexual dysfunction in patients with systemic sclerosis (SSc) and associate these symptoms with clinical and functional parameters ...including disability and quality of life.
Patients with SSc seen in Cochin hospital, Paris, between February and April 2010 were assessed for disease type, LUTS by the Urinary Symptom Profile scale, sexual dysfunction by the Feminine Sexual Function Index (FSFI) and International Index for the Erectile Function (IIEF-5), global disability by the Health Assessment Questionnaire and McMaster Toronto Arthritis Patient Preference Disability Questionnaire, anxiety and depression by the Hospital Anxiety and Depression scale, and quality of life by the Medical Outcomes Survey Short Form 36.
The most frequent LUTS among the 73 patients included (13 males) were overactive bladder (n=11, 84.6%) and dysuria (n=8, 61.5%) for males and overactive bladder (n=51, 85%) and incontinence (n=29, 48.3%) for females. Among women, 32 (53.2%) were sexually active: 20 (62.5%) had sexual disorders (mean SD FSFI score 16.3 6.2), the most compromised domains being desire (mean score 2.6 1.3) and arousal (mean score 2.5 1.4). Sexual disorders were associated with short disease duration (P=0.01) and high depression (P=0.04) scores. For men, 7/8 (87.5%) had erectile dysfunction (mean IIEF-5 score 16 5.3).
LUTS seem to be more frequent in SSc patients than in the general population. The most frequent symptom was overactive bladder.
Déterminer la fréquence des troubles urinaires du bas appareil (TUBA) et les troubles sexuels chez les patients ayant une sclérodermie systémique (ScS) et associer ces symptômes aux caractéristiques cliniques et fonctionnelles, incluant le handicap et la qualité de vie.
Des patients ayant une ScS, suivis à l’hôpital Cochin, Paris, entre février et avril 2010, ont été évalués selon le type de sclérodermie, les TUBA avec le Urinary Symptom Profile (USP), les troubles sexuels avec le Feminine Sexual Function Index (FSFI) et le International Index for the Erectile Function (IIEF-5), le handicap global avec le Health Assessment Questionnaire et le McMaster Toronto Arthritis Patient Preference Disability Questionnaire, l’anxiété et la dépression avec le Hospital Anxiety and Depression scale et la qualité de vie avec le Medical Outcomes Survey Short Form 36.
Les TUBA les plus fréquents parmi les 73 patients inclus (13 hommes) étaient l’hyperactivité vésicale (n=11, 84,6 %) et la dysurie (n=8, 61,5 %) chez les hommes et l’hyperactivité vésicale (n=51, 85 %) et l’incontinence (n=29, 48,3 %) chez les femmes. Parmi les femmes, 32 (53,2 %) étaient sexuellement actives, dont 20 (62,5 %) avaient des troubles sexuels (moyenne DS du FSFI de 16,3 6,2), les domaines plus atteints était le désir (score moyen : 2,6 1,3) et l’excitation (score moyen : 2,5 1,4). Les troubles sexuels étaient associés à une durée de la maladie plus courte (P=0,01) et à la dépression (P=0,04). Chez les hommes, 7/8 (87,5 %) avaient une dysfonction érectile (moyenne DS de l’IIEF-5 de 16 5,3).
Les TUBA semblent être plus fréquents chez les patients ayant une ScS que dans la population générale. Le symptôme plus fréquent était l’hyperactivité vésicale.
Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) that may be responsible for severe restrictive lung disease and represents one of the two main causes of ...disease-related death in SSc patients. Since 1993, the beneficial effect of oral or intravenous cyclophosphamide (CYC) in the treatment of SSc-related ILD has been reported in retrospective studies, one study showing improvement of pulmonary function test scores and/or chest computed tomography at 1 year and improvement of survival at 16 months. The results of two controlled trials were recently reported. The Scleroderma Lung Study, a prospective randomized placebo-controlled trial, included 158 patients of whom 145 completed at least 6 months of treatment. The course of forced vital capacity (primary outcome) adjusted at 1 year was significantly better in the group treated with oral CYC (P < 0.03), although the effect of CYC was minor. The Fibrosing Alveolitis in Scleroderma Trial (FAST) included 45 patients with SSc-related ILD who were randomized to receive prednisolone (20 mg per day) and 6 CYC infusions (600 mg/m monthly) or placebo. This trial did not demonstrate significant improvement of the primary or secondary endpoints in the active treatment group versus placebo. Since with the exception of the study of Silver et al. none of the patients included in retrospective or prospective studies were selected on the basis of progression of ILD. Since only a minority of SSc patients develops severe ILD, we propose that further studies evaluating CYC should focus on the subgroup of SSc patients with worsening ILD.
Work disability is a major issue in patients with systemic sclerosis (SSc) and seems to be substantially higher than other connective tissue diseases. Working ability results from complex ...interactions between factors related to socio-demographic characteristics, SSc specificities and working conditions. Working ability is related to global disability, altered hand function, fatigue, pain, type of work and opportunities to adapt workstation. Work disability is responsible for reduced income in patients with SSc.
To study the frequency and characteristics of patients with Wegener's granulomatosis (WG) strictly and persistently localized to one organ.
Retrospective analysis of the French Vasculitis Study Group ...(FVSG) WG cohort.
Sixteen patients (3.2% of the cohort) were identified who had isolated lung nodules, ear-nose-throat, or ocular involvement that did not progress to systemic disease (median followup, 58 mo) over the period of observation. Ten received first-line therapy with cyclophosphamide, which was effective in 4. Cotrimoxazole alone achieved remission in one, combined with corticosteroids in 3. Eight required subsequent treatments because of first-line failure or relapse.
Strictly and persistently localized WG is uncommon. Optimal treatment remains to be determined.
Work disability is a major issue in patients with systemic sclerosis (SSc) and seems to be substantially higher than other connective tissue diseases.
Working ability results from complex ...interactions between factors related to socio-demographic characteristics, SSc specificities and working conditions.
Working ability is related to global disability, altered hand function, fatigue, pain, type of work and opportunities to adapt workstation.
Work disability is responsible for reduced income in patients with SSc.
La perte ou la diminution de la capacité de travail est une préoccupation majeure au cours de la sclérodermie systémique (ScS) et semble plus importante que dans les autres connectivites.
La capacité de travail résulte d’interactions complexes entre les facteurs liés aux caractéristiques sociodémographiques du patient, aux particularités de la ScS et aux conditions de travail.
La capacité de travail est influencée par le niveau de handicap, la perte de fonction de la main, la fatigue, la douleur, le type de travail et la possibilité d’adapter son poste de travail.
La diminution de la capacité de travail au cours de la ScS entraîne une diminution, voire une perte des revenus.
Single-agent chemotherapy is usually effective in HIV-associated multicentric Castleman's disease (MCD). However, in most patients, chemotherapy cannot be discontinued.
To evaluate the efficacy of ...four weekly rituximab infusions (375 mg/m(2)) after discontinuation of chemotherapy in HIV-associated MCD, 24 patients were enrolled onto a prospective open-label trial.
At study entry, the median time from MCD diagnosis was 21 months. All patients had stable disease on chemotherapy and were dependent on chemotherapy for a median time of 13 months. The median CD4 cell count was 270 x 10(6)/L, and the plasma HIV RNA was less than 50 copies/mL in 18 patients. One patient died with progressive disease at day 15, and 23 patients completed the four cycles of rituximab. Sustained remission (SR) off treatment at day 60 (primary end point) was achieved in 22 patients (92%). From day 60 to day 365, one patient died with acute respiratory failure of undetermined origin, and four patients experienced relapse. Seventeen patients (71%) were alive in SR at day 365 without specific treatment, and the overall survival rate was 92% (95% CI, 71% to 98%). Rituximab was well tolerated, and the majority of adverse events were mild to moderate infections. Mild exacerbation of Kaposi's sarcoma (KS) lesions was observed in eight of 12 patients with previous KS.
Rituximab was both effective and safe in HIV-infected patients with chemotherapy-dependent MCD.
Objective. To assess employment status and socio-economic burden in SSc patients. Methods. Eighty-seven SSc patients (72 females), fulfilling the ACR or the Leroy and Medsger criteria, or both, were ...evaluated for employment status, socio-economic burden and handicap. Statistical analysis involved Mann–Whitney U-test and Fisher’s exact test and backward stepwise regression analysis. Results. In total, 60.9% of the SSc patients were on full-time sick leave and 35.6% were receiving a disability pension. On univariate analysis, myalgia was the only clinical manifestation more frequently encountered in sick-leave patients than others (73.6 vs 47.1%; P = 0.012). Karnofsky performance status (KPS) was lower in SSc patients who were on sick leave or were receiving a disability pension than others 78.5 (10.6) vs 85.8 (9.0); P = 0.004 and 78.1 (8.7) vs 83.1 (11.2); P = 0.016, respectively. In addition, greater global, hand and mouth handicaps and depression were observed in patients on sick leave HAQ 0.9 (0.7) vs 0.6 (0.5); P = 0.021; Cochin Hand Function Scale 21.7 (18.9) vs 10.7 (12.1); P = 0.003; mouth handicap scale 20.2 (10.8) vs 14.6 (10.0); P = 0.014; and depression dimension of the hospital anxiety and depression scale 7.1 (3.9) vs 4.8 (3.4); P = 0.003. On multivariate analysis, factors associated with sick leave were KPS odds ratio (OR) 0.92; 95%CI 0.88, 0.98 and myalgias (OR 3.19; 95% CI 1.19, 8.58), and the factor associated with receiving a disability pension was decreased income (OR 8.19; 95% CI 2.67, 25.12). Conclusions. SSc patients commonly have to take full-time sick leave from work. Despite such patients receiving disability pensions, the socio-economic burden is considerable.
Scleroderma renal crisis Mouthon, Luc; Bussone, Guillaume; Berezné, Alice ...
Journal of rheumatology,
06/2014, Volume:
41, Issue:
6
Journal Article
Peer reviewed
Open access
Scleroderma renal crisis (SRC) is characterized by malignant hypertension and oligo-anuric acute renal failure. It occurs in 5% of patients with systemic sclerosis (SSc), particularly in patients ...with diffuse disease during the first years. SRC is more common in patients receiving corticosteroids, the risk increasing with increasing dose. The disease is sometimes triggered by use of nephrotoxic drugs and/or intravascular volume depletion. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features. Thrombotic microangiopathy is detected in 43% of cases, and anti-RNA-polymerase III antibodies are present in one-third of patients. Renal biopsy is not necessary if SRC presents classical features. However, biopsy may help to define the prognosis and guide treatment in atypical forms. The prognosis of SRC has greatly improved with the introduction of angiotensin-converting enzyme (ACE) inhibitors. However, the 5-year survival for SSc patients with full SRC remains low (65%). The treatment of SRC relies on aggressive blood pressure control with an ACE inhibitor, combined with other antihypertensive drugs if needed. Dialysis is frequently indicated but can be stopped in about half of patients, mainly those with good blood pressure control. Patients who need dialysis for more than 2 years qualify for renal transplantation.
Objective
Data on adult IgA vasculitis (Henoch‐Schönlein) (IgAV) are scarce. This survey was designed to better define the clinical spectrum of IgAV and efficacy of treatments in a French patient ...population.
Methods
Data on clinical characteristics, histologic features, and treatment response from 260 patients with IgAV included in a French multicenter retrospective survey were analyzed. Efficacy data were compared using different statistical models.
Results
The mean ± SD age of the patients with IgAV at diagnosis was 50.1 ± 18 years, and 63% of patients were male. Baseline manifestations included purpura (100%), arthralgias/arthritis/myalgia (61%), glomerulonephritis (70%), and/or gastrointestinal involvement (53%). Thirty percent of patients showed renal failure at baseline. In univariate analysis, the response to therapy was 80% (64 of 80) in patients treated with corticosteroids (CS) alone, compared to 77% (23 of 30) in patients treated with CS plus cyclophosphamide (CYC) and 59% (10 of 17) in patients treated with colchicine (P = 0.17). Multivariable analysis showed that treatment with CS or CS plus CYC was more effective than colchicine in achieving a response. Efficacy differences were demonstrated using different statistical models: in the multivariable logistic regression model, odds ratio (OR) 3.68, 95% confidence interval (95% CI) 1.10–12.33 (P = 0.03); in the inverse probability weighting on propensity score model, OR 3.75, 95% CI 1.28–10.99 (P = 0.02). The efficacy of CS plus CYC as compared to CS alone was discordant according to the analytic method used. Analysis with the multivariable logistic regression model did not demonstrate a difference between CS plus CYC and CS alone (OR 0.88, 95% CI 0.29–2.67; P = 0.82). In contrast, inverse probability weighting on propensity score showed that CS plus CYC was more effective than CS alone (OR 1.79, 95% CI 1.00–3.20; P = 0.049).
Conclusion
This series constitutes the largest series of adults with IgAV reported in the literature so far. It provides data on clinical and histologic presentation and therapeutic efficacy, suggesting that CS alone appears to be a reasonable first‐line therapy in patients with IgAV, while the benefit of adding CYC to CS remains uncertain.
Objective. To evaluate the influenza vaccination rate and factors influencing it in patients with SSc. Methods. A total of 177 SSc patients fulfilling the ACR and/or LeRoy and Medsger criteria were ...evaluated during annual meetings of the French patient association in 2006 (n = 71) and 2007 (n = 70) or during hospitalization in the Internal Medicine Department of Cochin Hospital in 2007 (n = 36). Information on influenza vaccination was collected by a standardized form. Results. Mean (s.d.) age and disease duration were 58.7 (12.6) and 10.5 (9.5) years, respectively. Overall, 69 (39%) patients received an influenza vaccination during the previous year. Among the 108 patients who were not vaccinated, 78 (72.2%) presented at least one indication for vaccination. The most frequent reasons for non-vaccination were absence of physician recommendation and fear of side effects. Patients who were and were not vaccinated did not differ in anxiety, depression, global disability or quality of life. Vaccination rate was significantly higher (59%) for patients who remembered receiving a letter from the French National Health Insurance Agency encouraging vaccination than among those who did not (26%, P = 0.0001). Multivariate analysis revealed the year of the last vaccination and age as two independent parameters associated with vaccination. Conclusions. Influenza vaccination coverage is low in SSc patients. Lack of information and fear of adverse effects are the most common reasons for non-vaccination. Efforts are needed to increase the influenza vaccination coverage in this population.
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