RESUMO
Adult onset Still's disease (AOSD) is a systemic autoinflammatory disorder that presents with recurrent fever, extreme fatigue, and joint pain. Pulmonary involvement is not uncommon and, although rare, severe pneumonitis can progress to respiratory failure. Still's disease-associated pneumonitis is generally treated with immunosuppressive agents, but improvement in our understanding of systemic inflammatory processes led us to explore alternative agents. Anakinra is an interleukin-1 receptor antagonist used to treat autoinflammatory disorders resistant to immunosuppressive therapy. Several case reports have demonstrated efficacy of anakinra in treating AOSD, but its relevance in cases complicated with severe pneumonitis has not been examined. Our patient's disease activity was not controlled with systemic steroids and cyclophosphamide. Treatment with anakinra led to a dramatic clinical response. This is the first reported case of AOSD with severe pneumonitis refractory to conventional therapy successfully treated with anakinra.
Assuntos
Artralgia , Ciclofosfamida , Fadiga , Febre , Imunossupressores , Proteína Antagonista do Receptor de Interleucina 1 , Interleucina-1 , Pneumonia , Insuficiência Respiratória , Esteroides , Doença de Still de Início TardioRESUMO
BACKGROUND: The urine dipstick is widely used as an initial screening tool for the evaluation of proteinuria; however, its diagnostic accuracy has not yet been sufficiently evaluated. Therefore, we evaluated its diagnostic accuracy using spot urine albumin/creatinine ratio (ACR) and total protein/creatinine ratio (PCR) in proteinuria. METHODS: Using PCR > or = 0.2g/g or > or = 0.5g/g and ACR > or = 300mg/g or > or = 30mg/g as the reference standard, we calculated the diagnostic accuracy profile: sensitivity, specificity, positive and negative predictive value, and the area under the curve (AUC) of the receiver operating characteristic curve. RESULTS: PCR and ACR were available for 10,348 and 3,873 instances of dipstick testing. The proportions with PCR > or = 0.2g/g, > or = 0.5g/g and ACR > or = 300mg/g, > or = 30mg/g were 38.2%, 24.6% and 8.9%, 31.7%, respectively. The AUCs for PCR > or = 0.2g/g, > or = 0.5g/g, and ACR > or = 300mg/g were 0.935 (trace: closest to ideal point), 0.968 (1+), and 0.983 (1+), respectively. Both sensitivity and specificity were > 80% except for PCR > or = 0.5g/g with trace cutoff. For the reference standard of ACR > or = 30mg/g, the AUC was 0.797 (trace) and the sensitivity was 63.5%. CONCLUSION: Urine dipstick test can be used for screening in older outpatients with ACR > or = 300mg/g or PCR as the reference standard for proteinuria. However, we cannot recommend the test as a screening tool with ACR > or = 30mg/g as the reference owing to its low sensitivity.