Cross-cultural adaptation of the Brazilian-Portuguese version of the chronic urticaria quality-of-life questionnaire - CU-Q2oL.

BACKGROUND: Chronic urticaria (CU) is a debilitating skin disorder that affects patients' health related quality of life and the only questionnaire prepared specifically to CU is the Chronic Urticaria Quality of Life Questionnaire (CU-Q(2)oL). OBJECTIVE: The purpose of this study was to cross-culturally adapt and validate the CU-Q(2)oL Brazilian-Portuguese version. METHODS: Forward and back translation by three bilingual translators followed by pre-test was used to adapt the questionnaire. The CU-Q(2)oL was self-administered along with the Dermatology Life Quality Index (DLQI) in 112 patients with CU. Disease activity was assessed using the Urticaria Activity Score. Factor analysis was used to identify scales of the Brazilian portuguese CU-Q(2)oL. Internal consistency, convergent validity and known-group validity was determined. Reproducibility was evaluated by interclass correlation coefficient (ICC). Multiple linear regression was used to determine the predicting factors of CU-Q(2)oL results. RESULTS: Factor analysis revealed a three-dimensional structure: sleep/mental status/eating (I), pruritus/impact on life activities (II) and swelling/limits/look (III), which explained 52.49% of the total variance. All scales showed excellent internal consistency. External construct validity was supported by correlations between the CU-Q(2)oL and DLQI. The tool was found to be able to differentiate between patients with high and low levels of urticaria activity. Test-retest reliability was good to excellent (ICC = 0.69-0.86). Disease severity and urticaria type were the only factors predicting results. CONCLUSIONS: The CU-Q(2)oL Brazilian portuguese version was easily filled out, well accepted by the patients, demonstrated an acceptable validity and reliability and might be used to evaluate treatment outcomes and in clinical research.

Initial and accrued damage as predictors of mortality in Brazilian patients with systemic lupus erythematosus: a cohort study.

The aim of this study was to investigate whether initial and accrued organ damage measured by Systemic Lupus International Collaborating Clinics (SLICC) Damage Index (SDI) predicts mortality in cohort of Brazilian patients with systemic lupus erythematosus (SLE). One hundred and five outpatients with SLE were enrolled from July 2000 to March 2001; their demographics, disease manifestations, interventions and quantified disease activity (SLEDAI) were obtained. SDI was measured at baseline and at the end of follow-up. Initial and accrued SDI prognostic values for mortality were investigated by multivariate Cox survival analysis and Kaplan-Meyer survival curves. After a median follow-up of 6.3 years, 19 patients died due to disease activity, end-organ failure, cardiovascular events, cancer and infection. Deceased patients had longer disease duration and greater initial and final SDI than survivors had. After adjustment for age, sex and disease duration, both initial and final SDI >/= 3 points were independent predictors of mortality, with hazard ratios (HRs) of 3.0 (1.1-8.2) and 4.7 (1.6-14.5), respectively. Damage accrual during follow-up was the strongest predictor of death (HR: 5.1, 2.0-13.0). Renal and pulmonary damages were the main predictors of increased mortality risk. In conclusion, baseline and accrued damage increase mortality risk in Brazilian patients with SLE. Measures to prevent damage development and progression are urgent to reduce the mortality of patients with SLE.

QT-interval parameters are increased in systemic lupus erythematosus patients.

Systemic lupus erythematosus (SLE) patients have increased cardiovascular morbidity and mortality. QT-interval parameters are presumed markers of cardiovascular risk and have not been previously evaluated in SLE. Standard 12-lead ECGs were obtained from 140 female SLE outpatients and 37 age and body mass index-matched controls. QT interval was measured in each lead and heart rate-corrected maximum QT-interval duration (QTcmax) and QT-interval dispersion (QTd) were calculated. Risk factors for cardiovascular disease and lupus clinical features, disease treatment, disease activity and damage index were recorded. SLE patients have increased QT-interval parameters when compared to controls (QTcmax: 427.91 +/- 31.53 ms(1/2) versus 410.05 +/- 15.45 ms(1/2), P < 0.001; QTd: 52.38 +/- 22.21 ms versus 37.12 +/- 12.88 ms, P < 0.001). These differences persisted after excluding those patients with arterial hypertension, diabetes and with ECG abnormalities (QTcmax: 419.90 +/- 28.78 ms(1/2) versus 409.15 +/- 15.85 ms(1/2), P = 0.041; QTd: 54.74 +/- 26.00 ms versus 37.96 +/- 13.05 ms, P = 0.001). Multivariate linear regression for factors associated with QTcmax selected the presence of electrocardiographic left ventricular hypertrophy (ECG-LVH) (P = 0.003), nonspecific ST-T-wave abnormalities (P = 0.022) and left atrial enlargement (P = 0.044). Multivariate associates with QTd were age (P = 0.018), ECG-LVH (P = 0.022) and ST-T abnormalities (P = 0.031). In conclusion, SLE patients have increased QT interval parameters when compared to controls. This prolongation may lead to an increased cardiovascular risk. This finding might be due to subclinical atherosclerotic cardiovascular disease.

Long-term thalidomide use in refractory cutaneous lesions of lupus erythematosus: a 65 series of Brazilian patients.

Thalidomide has been reported as efficacious in refractory cutaneous lupus erythematosus (LE). The most fearful side-effects are teratogenicity and neuropathy. We reported clinical efficacy of long-term low-dose use of thalidomide in 65 patients with LE, emphasizing the prevalence of adverse effects, especially of neuropathy and its related factors. Data obtained from medical records included age, sex, disease duration, and the presence of diagnostic criteria for systemic lupus erythematosus (SLE), the extent and activity of cutaneous lesions and previous treatments. Sixty-three patients (98.9%) presented complete or partial improvement with thalidomide therapy. Drowsiness occurred in 50 patients (77%). Twenty-eight patients (43.2%) presented neuropathy symptoms. Nerve conduction studies were done in 21 (75%) of them and were abnormal in 12 (57%). With the interruption of thalidomide, 24 (82.5%) had complete or partial improvement of neuropathy symptoms and 23 (82%) of them had cutaneous relapse. There were no significant differences between those who developed or not neuropathy in treatment duration, age, total dose and systemic versus cutaneous LE. In conclusion, thalidomide can be used in refractory cutaneous LE with great efficacy and relative security. Controlled studies with schemes with lower doses or intermittent usage or alternative drugs are wanted to reduce the burden of cutaneous morbidity of lupus erythematosus.

Rate, pattern and factors related to damage in Brazilian systemic lupus erythematosus patients.

The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index (SDI) is an accepted instrument to ascertain damage. It has been shown to vary among different SLE populations. The aim of this study was to assess SDI score, pattern and factors related to damage in Brazilian SLE outpatients. The SDI was obtained in 105 patients with a median age of 41 (5-95%, 19-61.7) years and a median SLE duration of 127 (17.6-345.9) months. Patients had a median SDI of 2 (0-8) and 81.9% had some damage (SDI > 0). Damage was associated with a higher number of ACR criteria for SLE in multivariate analysis (OR = 2.32, 95%CI = 1.23-4.37, P = 0.009). Antiphospholipid syndrome (APS) (OR = 9.82, 95%CI = 2.74-35.23, P < 0.001), methylprednisolone pulses (OR = 3.91, 95%CI = 1.19-12.81, P = 0.024), age (OR = 1.70, 95%CI = 1.02-1.13, P = 0.011) and prednisone use duration (OR = 1.01, 95%CI = 1.002-1.02, P = 0.020) were related to severe damage (SDI > or = 4). Hypertension was associated with renal, cardiac and atherosclerotic damage, as cyclophosphamide pulses were with premature menopause. In conclusion, damage was very frequent in Brazilian SLE patients, mainly due to skin involvement, compared to other SLE populations. The presence of APS was the major independent contributor to the development of severe damage. Arterial hypertension was identified as a common risk factor for renal, cardiac and atherosclerotic damage.

Humoral intestinal immunity in systemic lupus erythematosus.

Forty-five patients with systemic lupus erythematosus (SLE) underwent a cross-sectional study to evaluate intestinal secretory immunity. Peroral jejunal biopsy with histologic and immunohistochemical assessment of the mucosa were carried out in the patients and in 12 healthy volunteers. It was observed that an altered pattern of immunoglobulin-bearing plasma cells distributed in the lamina propria and complementary components were invariably present, mainly in the patients with active disease. The basement membrane of the intestinal crypt epithelium exhibited immunoglobulin and complementary deposits, similar to the lupus band test. None of the immunologic findings correlated with the medical treatment and with the peripheral blood analysis. The local changes in humoral immunity in patients with SLE did not correlate with gastrointestinal symptoms and may reflect the systemic effects of the disease.

Small-bowel involvement in systemic lupus erythematosus: a morphometric and immunohistochemical study.

BACKGROUND: We have investigated the intestinal mononuclear cell subpopulations in patients with systemic lupus erythematosus (SLE) and correlated these with the disease activity. METHODS: Eighteen female outpatients were studied; in 10 of them lupus activity was measured with the Lupus Activity Criteria Count and the SLE Disease Activity Index. Eight patients were in lupus remission. The control group consisted of 10 healthy volunteers. Peroral jejunal biopsy was performed in all individuals, at the angle of Treitz, using a Watson capsule, under X-ray control. Histologic studies analysed the villous to crypt ratio, lamina propria cells, and intraepithelial lymphocyte count. Immunohistochemical evaluation was carried out with the indirect immunoperoxidase technique, using monoclonal antibodies against CD3, CD4, CD8, D1, D7, D9, and M1. RESULTS: Lamina propria CD3+, CD8+, D7+, and M1+ cells from patients with SLE did not differ significantly from those of controls. CD4+ cells were decreased in all patients with SLE, especially in the clinically inactive patients. D1+ and D9+ cells were also decreased in all patients. CONCLUSION: The finding of quantitative abnormalities in the cell-mediated immunity of the intestinal mucosa may reflect systemic defects of the immune system in SLE.

[AIDS and myopathy: report of a case and review of the literature]. / AIDS e miopatia. Relato de um caso e revisão da literatura.

Report of an unusual case of myopathy in an HIV infected patient, responsive only to the immunosuppressor drug methotrexate. The patient was a 39 year old homosexual male with no past history of HIV-related manifestations. One month prior to admission he noticed that his left thigh was swollen and painful. Two weeks later both arms became enlarged and tender. A few days before admission he noticed intermittent fever and progressive dyspnea. Upon admission, oral thrush, dyspnea and global enlargement of both arms was noted. There was no articular involvement. Fiberoptic bronchoscopy revealed Pneumocystis carinii pneumonia (PCP). Serology for HIV was positive. Tests for antinuclear antibodies were negative. Serum CPK level was 1019 IU. Capillaroscopy was compatible with vasculitis. Muscle biopsy revealed multifocal myonecrosis. PCP was successfully treated with standard doses of TMP/SMZ. Although indomethacin, prednisone and dexamethasone were administered in succession, there was relentless progression of myopathy and persistence of fever. Six days after administration of methotrexate, the patient defervesced, volume of arms and legs diminished. CPK levels returned to normal after a second course of methotrexate. Upon reduction of the dose thigh enlargement recrudesced. The patient remained asymptomatic on weekly doses of methotrexate. He died five months later of acute respiratory failure.

PIP: Myopathy may be associated with the syndrome of seroconversion in individuals infected by the human immunodeficiency virus (HIV) or may represent the initial symptom of AIDS. In 1990, 39-year old white, single homosexual who was admitted 1 month prior had experienced an episode of edema and pain in the left thigh that faded with the use of nonhormonal antiinflammatory drugs. 15 days later both forearms became enlarged accompanied by pain and erythema. Erythromycin and cefalexine were used without success. Intermittent fever started to appear before admission accompanied by dyspnea when straining. Examination showed tachypnea, oral candidiasis, and enlargement of both upper arms with pain and local erythema without articular involvement. Neurological examination revealed hypotonia and generalized hyperreflexia with intact muscle strength. Serology was positive for HIV, rheumatic activity tests were negative, and muscle biopsy indicated multifocal myonecrosis. Creatinine phosphokinase was 1019 IU (decrease to 44 IU after treatment), aldolase was 19 IU (decrease to 5.6 IU), and glutamic-pyruvic transminase was 50 IU (decrease to 22 IU). Radiography of the thorax indicated interstitial infiltration. Fiberoptic bronchoscopy indicated Pneumocystis carinii pneumonia. Sulfamethoxazole and trimetropim treatment cured the dyspnea and hypoxemia, but the enlargement of both arms progressed. Capillaroscopy indicated vasculitis that was treated without success with indomethacin (150 mg/day), for 7 days; prednisone (40-80 mg/day) for 10 days; and dexamethasone (280 mg/day) for 2 days. 6 days after methotrexate (50 mg/dose/week) treatment the fever disappeared and the enlargement in the extremities receded, but a lower dose of 7.5 mg caused the return of fever and edema in the right thigh. The myopathy remained asymptomatic for 5 months with a weekly dose of 15 mg of methotrexate.