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INTRODUCTION: Adult-onset Still's disease is a rare systemic inflammatory disorder of unknown aetiology characterized by the classic triad of persistent high spiking fevers, joint pain and a distinctive salmon-colored bumpy rash however, the multiorgan involvement can be present. CASE DESCRIPTION: A 40-year-old woman previously healthy was referred to our hospital with 7 days of high fever and generalized arthralgia, The physical exam revealed angioneurotic edema detected on soles, palms and tongue and widespread red, urticated plaques in a symmetrical distribution affecting the arms, dorsal hands, upper and lower chest and back. Followed 5 days later by fever, the patient presented dyspnea, cough and hypoxemia, the imaging studies showed unilateral consolidation and pleural effusion. The bronchoscopy with bronchoalveolar lavage and skin biopsy were consistent with neutrophilic urticarial. The hematological disorders, infections and other autoimmune diseases were excluded. DISCUSSION AND EVALUATION: The diagnosis of adult-onset Still's disease can be very difficult. There are no specific tests and reliance is usually placed on a symptom complex and the well described typical rash seen in most patients. In recent years, however, other cutaneous manifestations of Adult-onset Still's disease have been reported but these are not so well known. CONCLUSIONS: The evidence of rare manifestations is growing and the early clinical presentation of Adult-onset Still's is extremely variable, making diagnosis difficult. For this reason, data on early clinical presentation of the disease are of interest. We reported the first case of acute Adult-onset Still's disease with the association of pulmonary hemorrhage, urticaria and angioedema including a rare systemic manifestation as leukemoid reaction.
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BACKGROUND: Original Fried's frailty criteria have not demonstrated their prognostic validity of mortality, disability and mobility loss in European cohorts. OBJECTIVES: To analyze whether frailty implies increased risk of death, incident disability in basic (BADL) or instrumental (IADL) activities of daily living, or mobility impairment. DESIGN: Concurrent cohort study. SETTING: Albacete City, Spain. PARTICIPANTS: 993 participants over age 70 from the FRADEA Study. MEASUREMENTS: Mortality, BADL and mobility using the Barthel Index, and IADL using the Lawton IADL Index, were recorded. BADL disability was defined as loss of the ability to perform bathing, grooming, dressing, toilet use, or feeding, while deterioration of mobility was defined as loss of ability to perform transfers, walk, or use stairs, and IADL disability as losing any of the activities included in the Lawton Index. The risk of presenting adverse events was determined by Cox and Kaplan-Meier proportional hazard analysis and logistic regression adjusted for age, sex, function, and comorbidity. RESULTS: Mean follow-up was 534 days (SD 153), during which 105 participants (10.6%) died. Mean time to death was 363 days (SD 218), while 192 (25.4%) lost at least one BADL, 492 (60%) at least one IADL, and 222 (28.9%) lost mobility. Frail subjects had a greater adjusted risk of death (HR 5.5, CI 95% 1.5-20.2), of losing BADL (HR 2.5, CI 95% 1.3-4.8), of losing mobility (HR 2.7, CI 95% 1.5-5.0), and of losing IADL (HR 1.9, CI 95% 1.1-3.3) than non-frail patients. CONCLUSION: Fried's frailty criteria are associated with death, incident disability, and mobility impairment in a Spanish cohort of older adults.