Post-partum Fever of Unknown Origin: An Inaugural Flare of Severe Lupus With Multisystemic Involvement and Hemophagocytic Syndrome.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect almost every organ. Lupus protein-losing enteropathy (PLE) is one of the rarest manifestations of gastrointestinal involvement. Lupus flare as initial presentation is rare and the disease can act as a trigger to other pathologic immune syndromes like Hemophagocytic Lymphohistiocytosis (HLH), although this association is rare. We report the case of a previously healthy African 39-year-old female patient, with a recent history of cesarean section. Admitted to the Emergency Department (ED) with diffuse abdominal pain and fever, having completed a cycle of antibiotic therapy for initially suspected endometritis. The clinical picture progressed with sustained high fever, new-onset lymphadenopathies, systemic rash, acute pulmonary edema and seizures. Laboratory findings included hyperferritinemia, hypertriglyceridemia, proteinuria and hypoalbuminemia. The auto-immune panel was positive for antinuclear antibodies (ANA), anti-dsDNA, anti-SSA and anti-SSB, anti-PL7, anti-RNP, anti-U1-SnRNP, and anti-Pm-Scl75. She also presented hypocomplementemia. An inaugural flare of SLE with multisystemic involvement and concomitant secondary Hemophagocytic Syndrome was considered and therapy with methylprednisolone pulses, Anakinra and Cyclophosphamide was started. By the end of the first cycle of cyclophosphamide, the patient presented clinical worsening with abdominal pain recrudescence and profuse diarrhea. After the exclusion of an infectious process, a Lupus PLE was assumed and Cyclophosphamide protocol was resumed, with sustained clinical improvement after the induction protocol. Despite initially suspected gynecological infection, the clinical progression with multisystemic involvement together with the auto-immune panel made the diagnosis of SLE possible, with other laboratory findings raising the suspicion of HLH. This case represents a rare report of severe SLE with multiple organ involvement accompanied by HLH. Gastrointestinal involvement with PLE added rarity and morbidity to the clinical picture. The case reinforces the idea that when organ dysfunction is due to a severe autoimmune response, supportive treatment can be lifesaving until immunosuppressive drugs reach their full effect.

Cancer Screening in Patients with Unprovoked Thromboembolism: How to do it and Who Benefits?

INTRODUCTION: Unprovoked venous thromboembolism (uVTE) may be the first manifestation of cancer. The main objectives of this study were to compare limited screening (LS) and extended screening (ES) and to make a protocol to approach these patients. METHODS: This is a retrospective, unicentric observational study that included 245 patients with venous thromboembolism (VTE) admitted to an Internal Medicine Service for five years. The incidence of cancer and mortality during hospitalization, and at one and three years after admission were calculated in both LS and ES groups and compared. RESULTS: Of the 245 patients with VTE, 59 (24.1%) had uVTE: 35 (59.3%) were submitted to LS and 24 (40.7%) to ES, with 10 (4.1%) diagnosis of cancer. In the following three years, 10 more patients were diagnosed. There were no statistically significant differences in inpatient diagnosis rates (8.6% vs. 4.2%; p=0.51) or in-hospital mortality (2.9% vs. 4.2%; p=0.79) or mortality at one year (8.6% vs. 8.3%; p=0.97) and three years (20.0% vs. 20.8%; p = 0.94) between LS and ES groups respectively. The Computerized Registry of Patients with Venous Thromboembolism (RIETE) score was equal or superior to 3 in 69.5% (N=41) of the population with uVTE. DISCUSSION: The results of our study are consistent with the literature; there are no differences between screenings, as the difference in the number of diagnoses does not reflect on mortality. CONCLUSION: There were no statistically significant differences between the two types of screening in this population. We suggest a protocol that includes the RIETE score to better select the patients who might benefit the most from an ES.

Risk stratification in normotensive acute pulmonary embolism patients: focus on the intermediate-high risk subgroup.

BACKGROUND: Patients with acute pulmonary embolism are at intermediate-high risk in the presence of imaging signs of right ventricular dysfunction plus one or more elevated cardiac biomarker. We hypothesised that intermediate-high risk patients with two elevated cardiac biomarkers and imaging signs of right ventricular dysfunction have a worse prognosis than those with one cardiac biomarker and imaging signs of right ventricular dysfunction. METHODS: We analysed the cumulative presence of cardiac biomarkers and imaging signs of right ventricular dysfunction in 525 patients with intermediate risk pulmonary embolism (intermediate-high risk = 237) presenting at the emergency department in two centres. Studied endpoints were composites of all-cause mortality and/or rescue thrombolysis at 30 days (primary endpoint; n=58) and pulmonary embolism-related mortality and/or rescue thrombolysis at 30 days (secondary endpoint; n=40). RESULTS: Patients who experienced the primary endpoint showed a higher proportion of elevated troponin (47% vs. 76%, P<0.001), elevated N-terminal pro-brain natriuretic peptide (67% vs. 93%, P<0.001) and imaging signs of right ventricular dysfunction (47% vs. 80%, P<0.001). Multivariate analysis revealed N-terminal pro-brain natriuretic peptide (hazard ratio (HR) 3.6, 95% confidence interval (CI) 1.3-10.3; P=0.015) and imaging signs of right ventricular dysfunction (HR 2.8, 95% CI 1.5-5.2; P=0.001) as independent predictors of events. In the intermediate-high risk group, patients with two cardiac biomarkers performed worse than those with one cardiac biomarker (HR 3.3, 95% CI 1.8-6.2; P=0.003). CONCLUSIONS: Risk stratification in normotensive pulmonary embolism should consider the cumulative presence of cardiac biomarkers and imaging signs of right ventricular dysfunction, especially in the intermediate-high risk subgroup.

Derivation and external validation of the SHIeLD score for predicting outcome in normotensive pulmonary embolism.

BACKGROUND: Identifying patients with normotensive pulmonary embolism (PE) who may benefit from thrombolysis remains challenging. We sought to develop and validate a score to predict 30-days PE-related mortality and/or rescue thrombolysis. METHODS: We retrospectively assessed 554 patients with normotensive PE. Independent predictors of the studied endpoint were identified from variables available at admission in the emergency department and were used to create a score. The model was validated in 308 patients from a separate hospital. RESULTS: A total of 64 patients died or needed rescue thrombolysis (44 in the derivation cohort). Four independent prognostic factors were identified: Shock index ≥ 1.0 (OR 3.33; 95% CI 1.40-7.93; P = 0.006), HypoxaemIa by the PaO2/FiO2 ratio (OR 0.92 per 10 units; 95% CI 0.88-0.97; P < 0.001), Lactate (OR 1.38 per mmol/L; 95% CI 1.09-1.75; P = 0.008) and cardiovascular Dysfunction (OR 5.67; 95% CI 2.60-12.33; P < 0.001) - SHIeLD score. In the development cohort, event rates for each risk tercile were 0.0%, 2.2%, and 21.6%. In the validation cohort, corresponding rates were 0.0%, 1.9%, and 14.3%. The C-statistic was 0.90 (95% CI 0.86-0.94, P < 0.001) in the derivation cohort and 0.82 (95% CI 0.75-0.89, P < 0.001) in the validation cohort. Decision curve analysis showed that the SHIeLD score is able to accurately identify more true positive cases than the European Society of Cardiology decision criteria. CONCLUSIONS: A risk score to predict 30-days PE-related mortality and/or rescue thrombolysis in patients with normotensive PE was developed and validated. This score may assist physicians in selecting patients for closer monitoring or aggressive treatment strategy.