Lupus nephritis (LN) is a serious and common complication of systemic lupus erythematosus (SLE) that predisposes to significant morbidity and mortality. Studies show that prompt diagnosis and treatment improves patient survival. We present a case of a 49-year-old female with an atypical presentation of LN who initially presented with new-onset hypertension, edema, arthritis, serositis and recently diagnosed leukocytoclastic vasculitis who later developed acute kidney injury, hematuria and nephrotic syndrome. Laboratory testing showed mixed cryoglobulinemia and elevated perinuclear anti-neutrophil cytoplasmic (p-ANCA) and myeloperoxidase (MPO) antibodies. SLE-related serologies were negative. Kidney biopsy showed diffuse proliferative global glomerulonephritis with a full-house nephropathy pattern on immunofluorescence suggestive of LN. Due to high clinical suspicion and renal biopsy findings, she was treated for LN with prompt renal response to immunosuppression. Cryoglobulins, p-ANCA and MPO titers normalized and the negative SLE serologies remained negative. Literature review on antinuclear antibody (ANA)-negative and seronegative LN revealed the following patient presentations: (1) renal-limited or renal and extra-renal manifestations of SLE with negative serologies and (2) renal and extra-renal manifestations of SLE with negative serologies at presentation who develop positive serologies later in follow-up. Both groups represent a unique and challenging cohort of patients who may require longer follow-up and further testing to rule out other glomerular diseases that may mimic LN on renal biopsy. The absence of SLE-related serologies should be weighed against a high pre-test probability of ANA-negative or seronegative LN. If highly suspected, the patient should be treated promptly with close monitoring.

Background ADAMTS13 test distinguishes thrombotic thrombocytopenic purpura (TTP) from other thrombotic microangiopathies (TMAs). PLASMIC score helps determine the pretest probability of ADAMTS13 deficiency. Due to inherent limitations of both tests, and potential adverse effects and cost of unnecessary treatments, we performed a cost-effectiveness analysis (CEA) investigating the benefits of incorporating an in-hospital ADAMTS13 test and/or PLASMIC score into our clinical practice. Methods A CEA model was created to compare 4 scenarios for a patient with TMAs, utilizing either an in-house vs. send-out ADAMTS13 assay with or without prior risk stratification using PLASMIC scoring. Model parameters, including probabilities and costs, were gathered from the medical literature, except for the ADAMTS13 send-out and in-house tests, which were obtained from our institutional data. Results If only the cost is considered, in-house ADAMTS13 test for patients with intermediate-high risk PLASMIC score is the least expensive option ($4,732/patient). If effectiveness is assessed as measured by the number of averted deaths, send-out ADAMTS13 test is the most effective. Considering the cost/effectiveness ratio, the in-house ADAMTS13 test in patients with intermediate-high risk PLASMIC score is the best option, followed by the in-house ADAMTS13 test without the PLASMIC score. Conclusions In patients with clinical presentations of TMAs, having an in-hospital ADAMTS13 test to promptly establish the diagnosis of TTP appears to be cost-effective. Utilizing the PLASMIC score increases the cost-effectiveness of the in-house ADAMTS13 test. Our findings indicate the benefit of having a rapid and reliable in-house ADAMTS13 test, especially in the tertiary medical center.

Background Acquired haemophilia A (AHA) is an autoimmune bleeding disorder with significant morbidity and mortality. Bleeding AHA patients with high titre inhibitors can be treated with either activated prothrombin complex concentrate (aPCC) or recombinant activated factor VII (rFVIIa). Given that both replacement therapies have inherent benefits and limitations, a cost‐effectiveness analysis (CEA) was performed in this population to compare rFVIIa with aPCC. Methods In high‐titered AHA patients with bleeding treated with either aPCC or rFVIIa, during a 5‐day study period, a Markov model was developed such that these patients were transitioned into four different health states: (1) continuous bleeding, (2) thrombosis, (3) stop bleeding and (4) death, with states (2), (3) and (4) modelled as absorbing states. Model parameters, including probabilities, health utility index and costs, were gathered from the medical literature, except for the costs of aPCC and rFVIIa, which were obtained from our institutional data. Results During the 5‐day period, the total treatment cost of rFVIIa was substantially more than the cost of aPCC ($13 635 vs. $1741). The average quality‐adjusted life days (QALDs) gained for rFVIIa were slightly lower compared to aPCC (4·08 vs. 4·09). Overall, aPCC prevailed over rFVIIa. Sensitivity analysis confirmed the robustness of the model across tested ranges of all input variables. Conclusion In high‐titered AHA patients with bleeding, aPCC is a cost‐effective treatment option when compared to rFVIIa. Thus, aPCC may be considered in these patients, if available, and provided there is no clinical contraindication.

No abstract

A 30-year-old woman at 33.4 weeks of gestation who had a history of gestational diabetes was admitted for acute epigastric pain and respiratory distress. On admission, her triglyceride (TG) level was 7716 mg/dL, total cholesterol was 1638 mg/dL, lipase was 349 U/L, and amylase was 208 U/L; thus, she was diagnosed with acute pancreatitis. Because pharmacologic treatment options for hypertriglyceridemia are limited in pregnancy, therapeutic plasma exchange (TPE) was performed emergently to acutely decrease the level of TGs in the Labor and Delivery Unit, with full support from the Obstetrical Service in case emergency delivery was necessary ( Fig. A shows her lipemic plasma compared with nonlipemic plasma from a control patient in Fig. B). Briefly, the plasma component containing TG is separated extracorporeally by centrifugation and is replaced by 5% albumin as the replacement fluid, as in this case. Other inflammatory cytokines may also be removed during the process. 1 Furthermore, several changes during pregnancy should be considered. For example, plasma volume (PV) can increase by 10 to 15% by the second half of the first trimester, further expanding through 30 to 34 weeks; therefore, accounting for this fact, we calculated the blood volume based on Nadler's formula and exchanged 1.5 PVs in this patient. [2][3][4] Low resistance in the uteroplacental circulation and other vessels contributes to a total decline in vascular resistance, so replacement fluids should be readily available for procedure-induced hypotension, if needed. 5 Finally, although heparin may release lipoprotein lipase that enhances TG clearance, and although pregnancy is a hypercoagulable state, we elected to use citrate as the anticoagulation during TPE, because the procedure also removes coagulation factors and thus may predispose the patient to an increased bleeding risk. 6 After two procedures, her TGs decreased to 750 mg/dL. She was then started on a low-fat diet and omega-3-acid ethyl esters for lipid control for the remainder of her pregnancy, with a goal TG <1000 mg/dL. Another TPE was performed 16 days later, because her TGs had increased to 974 mg/dL (Fig. C shows the relationship between TG level and TPEs). She From the