Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.

Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P < 0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors.

Differential expression pattern of microRNAs in CD4+ and CD19+ cells from asymptomatic patients with systemic lupus erythematosus.

OBJECTIVE: The aim of this study was to investigate the pattern of microRNA (miRNA) expression in CD19+ and CD4+ cells from asymptomatic patients with systemic lupus erythematosus (SLE). METHODS: A screening of the expression of 377 miRNAs was performed in human CD4+ and CD19+ cells isolated from the peripheral blood by using a TaqMan Human MicroRNA Array. Validation of differential expression pattern of those was performed using TaqMan assays in these cell populations obtained from a larger cohort of patients and controls. RESULTS: According to the screening assays, three miRNAs were differentially expressed (p value <0.1) in cell populations from both patients and controls: hsa-miR-143, hsa-miR-224 and hsa-miR-576-5p for CD4+ cells, and hsa-miR-10a, hsa-miR-31 and hsa-miR-345 for CD19+ cells. After validation, significant differences (p value <0.05) were confirmed only for hsa-miR-143 and hsa-miR-224 in CD4+ cells and for hsa-miR-10a and hsa-miR-345 in CD19+ cells. In all cases, the miRNAs were over expressed in SLE patients compared with healthy donors. CONCLUSIONS: Our results support a different pattern of miRNA expression in SLE patients.

Screening for PAH in patients with systemic sclerosis: focus on Doppler echocardiography.

It is well established that patients with CTDs such as SSc carry a considerable risk of developing pulmonary arterial hypertension (PAH). Such SSc-PAH patients have an even worse prognosis than patients with only one of these two conditions. In view of the high incidence and prevalence of PAH in SSc, and the available treatment options that improve quality of life, exercise capacity and possibly survival, systematic screening has been recommended. The present article reviews current recommendations from PAH guidelines, focusing on studies that used Doppler echocardiography for screening, and describes limitations associated with the procedure. Furthermore, characteristics and parameters used to identify patients at high risk of developing PAH are summarized.

[Diagnosis and treatment of pulmonary hypertension]. / Diagnóstico y tratamiento de la hipertensión pulmonar.

Pulmonary arterial hypertension is an idiopathic process or can be associated with another circumstances (connective tissue diseases, congenital heart disease, portal hypertension, exposure to appetite suppressants or another drugs or infectious agents such as HIV). Most patients are diagnosed as the result of an evaluation of symptoms, whereas others are diagnosed incidentally or during screening of asymptomatic populations at risk. We reviews systematic screening for the approach to diagnosing pulmonary arterial hypertension. A diagnostic algorithm can guide the evaluation but it can be modified according to specific clinical circumstances. The number of therapeutic options has increased.in the last years. We reviews the use of calcium-channel blockers, prostacyclin (and analogues), endothelin-receptor antagonists, and phosphodiesterase-5 inhibitors, and the use of combination therapy, and provides specific recommendations about the actual treatment.

[Vogt-Koyanagi-Harada disease. Characteristics of a series of Andalusian patients]. / Enfermedad de Vogt-Koyanagi-Harada. Características de un grupo de pacientes andaluces.

BACKGROUND: To describe the characteristics of a series of patients diagnosed of Vogt-Koyanagi-Harada disease (VKHD) and controlled by an Uveitis Unit (composed of ophthalmologists and internists) in our population. PATIENTS AND METHOD: Retrospective descriptive study of 11 patients with VKHD (5 males and 6 women; median age at diagnosis 32.6 years old) followed-up between 1980 and 2003. RESULTS: All patients suffered panuveitis and/or exudative retinal detachment. Extraocular signs were present in all cases: neurological in 63.7% (aseptic meningitis and/or focal symptoms), cutaneous in 81.8% (vitiligo, whiteness, poliosis, alopecia), neurosensorial hypoacusis (50% of patients with audiometry), and general symptoms in 25%. They all received systemic corticosteroids. Cyclosporine was added in 5 patients (45.4%) with posterior uveitis, and azathioprine in 2 of them for iridocyclitis. The final visual acuity was 0.5 or better in 81.8% of cases, but 2 patients had an unfavourable evolution (one, who had cataract and band keratopathy in the left eye and severe visual worsening, needed right vitrectomy, and the other suffered severe ocular hypotension). Complications developed in 2 other patients: cataract and glaucoma in one, and synechiae without glaucoma in the other. CONCLUSIONS: We found less frequency of exudative retinal detachment and greater frequency of cutaneous signs than the communicated. A significant percentage of cases needed immunosuppressive agents. Final visual acuity was good in the majority of patients.

[Oculomotor response to the vertical cephalic autorotatory test in patients with benign paroxistic positional vertigo of the posterior canal]. / Respuesta oculomotora al test de autorrotación cefálica vertical en pacientes con vértigo posicional paroxístico benigno del conducto posterior.

OBJECTIVE: To evaluate the eye movement response to the head auto-rotation test (HART) in the vertical plane in patients with benign paroxysmal positional vertigo. DESIGN: A transversal, descriptive study. SETTING UP: Outpatient clinic in a general Hospital. INDIVIDUALS: 34 posterior canal BPPV cases with a video-oculographic diagnosis, older than 18 years old, 7 of them were not able to perform the HART. INTERVENTION: HART was performed by a an electrooculographic system with simultaneous recording of head movement by an acelerometer in the vertical plane (Vorteq, Micromedical Instruments). The HART with eyes fixation was performed 3 times to determine its reliability. MAIN OUTCOME MEASURES: Gain, asymmetry and phase for the vertical VOR respectively. A statistical analysis was carried out to determine the test reliability and the number of individuals with an abnormal result. RESULTS: Gain is the only variable that showed a reproducible result in the HART for the active head movement at 1-2 Hz (test-retest reliability 0.83-0.89). The values of gain showed a moderate correlation at the frequencies 1-3 Hz (correlation 0.60-0.87). Asymmetry and phase were not reproducible variables (correlation < 0.55). Thirteen of 27 (48%) patients presented a decrease of the vertical gain, another 13 showed normal values and one case showed raised values. CONCLUSION: Gain is the only useful variable in the vertical HART. Forty-eight percent of patients with posterior canal BPPV have a reduced vertical gain, suggesting an impairment of inferior vestibular nerve function.

[Reference values for the vestibulo-ocular reflex response to the head shaking and the bithermal caloric tests]. / Valores poblacionales de referencia para video- oculografía en el test de agitación cefálica y la prueba calórica bitérmica.

OBJECTIVE: To obtain reference values for the vestibulo-ocular reflex response to the head-shaking nystagmus and the bithermal caloric test in the spanish population. DESIGN: A descriptive study. SET UP: General hospital. INDIVIDUALS: One hundred and seven healthy sex and age stratified voluntiers were included. The final sample included 97 individuals over 18 years of age. INTERVENTION: Spontaneous nystagmus (SN), head-shaking nystagmus (HSN) and caloric induced nystagmus were explored. The eyes movement was recorded by a video-oculographic system. The SN or HSN was considered significant when at least 6 consecutive beats with a slow phase velocity of 2 degrees/sec were detected. The caloric test was performed with water flow at 250mL/20 s at 30 degrees C and 44 degrees C with an interval of 10 minutes between irrigations. The percentage of canal paresis (CP) and directional preponderance (DP) was calculated using maximum slow phase velocity and the Jongkees index formula. MAIN OUTCOME MEASUREMENTS: Presence of SN, horizontal and vertical SHN, percentage of CP and DP. RESULTS: SN was found in 10,3% individuals (6 women and 4 men). Horizontal HSN was also observed in 9 women and 10 men (19,5%), and vertical HSN in 8 women and 10 men (18,6%). The mean and 95 percentil for CP were 13,10 and 28,60 in women and 11,02 and 27,30 in men. For DP, the mean and 95 percentil were 11,76 and 35,80 in women and 11,58 and 28,65 in men. The 5 percentils for slow phase velocity of nystagmus after each irrigation were 6,30; 5,14; 5,96; 4,02 degrees/s (left ear 44 degrees, right ear 44 degrees, left ear 30 degrees, right ear 30 degrees, respectively) in the group of women; and 5,82; 6,99; 5,67; 4,55 degrees/s in men (with the same irrigation sequence). CONCLUSION: Results presented should be considered as a population-based norms and may be useful as references for water bithermal test for VOG studies. The SN or HSN could be observed in subjects without vestibular pathology.

[Evaluation of the treatment of benign paroxysmal positional vertigo with the DHI-S questionnaire]. / Evaluación del tratamiento del vértigo posicional paroxístico benigno mediante el cuestionario DHI-S.

Benign paroxysmal positional vertigo (BPPV) is a recurrent chronic disease and its handicap is usually underestimated. The aim of this study is to determine the impact of the treatment by Epley maneuver on short-term BPPV-related quality of life. Forty-two individuals with BPPV were included: 39 with posterior canal affected, 2 with the lateral canal and one with the anterior canal. Diagnosis was established if a consistent clinical history was found and Dix-Hallpike test (DHT) in cases with canal posterior involvement. Subjects with positive DHT were treated by a single Epley maneuver and were recommended to avoid supine for the next 48 hours. The BPPV relapses were investigated at 7th and 30th day post-treatment. BPPV-associated quality of life was evaluated by the Dizzness Handicap Inventory Short-form (DHI-S) at days 1st and 30th post-treatment. Total and partial scores for emotional, physical and functional subscales were compared by Wilcoxon test for paired samples. Dix-Hallpike test was found positive in el 59% individuals (23/39), and 41% cases did not required any treatment. Among 23 patients treated with Epley maneuver, DHT was found negative in 90% at 30th day follow-up. Mean and standard deviation of the total scores obtained in the DHI-S at the first day were 19.22 +/- 9.66 in the DHT positive-patients and 19.79 +/- 10.14 in the whole group (DHT positive or negative). These scores significantly decreased to 10.84 +/- 10.99 at 30 days post-treatment (p = 0.002 and p = 0.001, respectively). In conclusion, the DHI-S is a specific health questionnaire able to assess BPPV-related health and the effectiveness of treatment.

[Comparative study of misoprostol and nifedipine in the treatment of Raynaud's phenomenon secondary to systemic diseases. Hemodynamic assessment with Doppler duplex]. / Estudio comparativo de misoprostol y nifedipino en el tratamiento del fenómeno de Raynaud secundario a enfermedades sistémicas. Valoración hemodinámica mediante Doppler-dúplex.

OBJECTIVE: To evaluate the mid-term efficiency and therapeutic safety at a mid term of the orally administered misoprostol, a synthetic PGE1, analogue, compared with nifedipine for the treatment of RP secondary to autoimmune systemic diseases. METHODS: A double blind, crossover study was designed. Patients were randomly distributed to receive either retard nifedipine (20 mg/12 hourly) and misoprostol (200 micrograms/12 hourly) in 10-day periods (washing period with placebo for 10 days). At the end of each period a clinical assessment was obtained on the frequency and severity of symptoms as well as on secondary drug reactions. Simultaneously, blood flow changes in radial artery were Doppler-duplex investigated (pulsatility index, resistance index). RESULTS: Twenty patients were studied (15 women and 5 men). The mean basal daily frequency of attacks was 4.8 +/- 2.0 compared with 2.4 +/- 1.4 with nifedipine (p < 0.001) and 2.6 +/- 1.2 with misoprostol (p < 0.001). The mean basal severity of attacks, according to a pre-established scale decreased from 3.7 +/- 0.6 to 1.9 +/- 0.9 with nifedipine (p < 0.001) and to 2.0 +/- 1.0 with misoprostol (p < 0.001). The mean basal value of blood flow in radial artery was 24.9 +/- 14.4 ml/min; with nifedipine it increased to 43.0 +/- 19.2 ml/min (p < 0.001) and with misoprostol to 46.9 +/- 19.2 ml/min (p < 0.001). Five patients (25%) had secondary effects with nifedipine and three (15%) with misoprostol; in no case had therapy to be discontinued. CONCLUSIONS: Misoprostol was similar to nifedipine for the treatment of Raynaud phenomenon secondary to systemic diseases and can be a therapeutic alternative for these patients.