RESUMO
Anti-MDA5 antibody dermatomyositis (DM) is a special type of myositis, which can potentially cause rapidly progressive interstitial lung disease (RP-ILD). Mixed connective tissue disease (MCTD) is a complex disease with different characteristics of autoimmune connective tissue disease, associated with ILD. Both are rare diseases, and few patients with both diseases have been reported. A 71-year-old woman complained of palpitations, with a 2 months history of rash around her hands, extensor surface of right elbow, and the nape of her neck. Subsequently, the patient had acute exacerbation of dyspnea and tachypnea. Anti-Ro52, U1 RNP and MDA5 antibodies were positive; the presenting evidence was suggestive of anti-MDA5+ DM-RP-ILD complicated with MCTD. Our patient deteriorated rapidly and had a fatal outcome, despite "triple therapy" for RP-ILD. This case illustrates that patients with coexisting anti-MDA5+ DM and MCTD have the former's typical clinical manifestations, and may develop ILD quickly rather than slowly as in MCTD, especially with the coexistence of anti-Ro52 antibodies.
Assuntos
Doenças Autoimunes , Dermatomiosite , Doenças Pulmonares Intersticiais , Doença Mista do Tecido Conjuntivo , Humanos , Feminino , Idoso , Doença Mista do Tecido Conjuntivo/complicações , Autoanticorpos , Doenças Autoimunes/complicações , Estudos RetrospectivosRESUMO
INTRODUCTION: The Chinese herb da huang (DH) (Rhubarb) is commonly used for GIF intensive care unit (ICU)/pediatric intensive care unit (PICU) gastrointestinal failure (GIF) patients in China. However, the potential preventive and therapeutic effect of DH in these patients has not yet been studied systematically. OBJECTIVES: The aim of this study was to evaluate the preventive and therapeutic effects of DH in treating ICU/PICU GIF patients with the most recent evidence. METHODS: We systematically searched 7 databases from inception to March 30, 2018. RevMan 5.3 software was used to perform a meta-analysis. GRADE methodology was applied to evaluate the quality of evidence for each outcome. The review protocol was registered on PROSPERO (CRD42018092710) in advance. RESULTS: Seven studies comprising 788 pediatric or adult participants were included in this analysis. Three indicators, including GIF occurrence rates (gastrointestinal mucosal hemorrhage, enteroplegia), multiple organ dysfunction syndrome (MODS)-related items (occurrence rates of MODS, mortality rates of MODS) and duration in the ICU was analyzed. The GIF occurrence rate meta-analysis result was (RR 0.47, CI 95% 0.37-0.60; Pâ=â.95); MODS related items indicator result was (RR 0.44, CI 95% 0.33-0.59; Pâ=â.41); ICU duration ICU result was (RR -2.87, CI 95% -3.53--2.21; Pâ=â.40). The safety of Chinese herb DH (Rhubarb) remains unclear. CONCLUSION: Current evidence suggests that the Chinese herb rhubarb (DH) powder combined with Western medicine was inferior to Western medicine alone in terms of preventive and therapeutic effects in ICU/PICU patients in terms of decreasing GIF occurrence rates (gastrointestinal mucosal hemorrhage and enteroplegia), occurrence rates of MODS, mortality from MODS, and shortened duration time in the ICU/PICU. However, larger sample sizes and rigorously-designed studies are necessary to conclusively determine the association between DH powder and outcomes in ICU/PICU GIF patients.