Severe pulmonary artery hypertension (PAH) is a rare initial presentation of systemic lupus erythematosus (SLE). SLE associated with PAH carries worse prognosis that isolated SLE. However, there has been improvement in mortality of the patients in the recent years owing to newer treatment options available. Early recognition remains of prime importance. We present here a case of young female who presented with severe pulmonary hypertension with right heart failure leading to cardiogenic shock and was found to have SLE. She was started on appropriate treatment; however, given the severity of her illness, the patient did not survive. This case highlights the importance of early recognition and prompt treatment of SLE-associated PAH, which might improve the survival rate in the patients.
IntroductionThe associations of dietary protein and/or energy intakes with protein or energy wasting in patients on maintenance hemodialysis are controversial. We examined these in the Hemodialysis (HEMO) Study.MethodsIn 1487 participants in the HEMO Study, baseline dietary protein intake (grams per kilogram per day) and dietary energy intake (kilocalories per kilograms per day) were related to the presence of the protein-energy wasting (PEW) syndrome at month 12 (defined as the presence of at least 1 criteria in 2 of the 3 categories of low serum chemistry, low body mass, and low muscle mass) in logistic regression models. In additional separate models, protein intake estimated from equilibrated normalized protein catabolic rate (enPCR) was also related to the PEW syndrome.ResultsCompared with the lowest quartile, the highest quartile of baseline dietary protein intake was paradoxically associated with increased risk of the PEW syndrome at month 12 (odds ratio [OR]: 4.11; 95% confidence interval [CI]: 2.79–6.05). This relationship was completely attenuated (OR: 1.35; 95% CI: 0.88–2.06) with adjustment for baseline body weight, which suggested mathematical coupling. Results were similar for dietary energy intake. Compared with the lowest quartile of baseline enPCR, the highest quartile was not associated with the PEW syndrome at 12 months (OR: 0.78; 95% CI: 0.54–1.12).DiscussionThese data do not support the use of dietary protein intake or dietary energy intake criteria in the definition of the PEW syndrome in patients on maintenance hemodialysis.
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