Thrombotic microangiopathy is characterised by endothelial cell injury, intravascular platelet-fibrin thrombi, and vascular damage, leading to acute kidney injury, thrombocytopenia, and microangiopathic haemolytic anaemia. Among the autoimmune diseases related to thrombotic microangiopathy, anti-neutrophil cytoplasmic antibody-associated vasculitis-related thrombotic microangiopathy cases have been rarely reported; therefore, the optimal treatment for associated vasculitis-related thrombotic microangiopathy remains unknown. An 84-year-old woman without significant medical history presented with a 1-month history of general fatigue, fever, and deteriorating bilateral leg numbness and was admitted to our hospital. She had elevated myeloperoxidase anti-neutrophil cytoplasmic antibody levels, polyneuropathy, and rapid progressive glomerulonephritis because of pauci-immune crescentic glomerulonephritis, as revealed by a kidney biopsy. Accordingly, we diagnosed her with microscopic polyangiitis. After administering methylprednisolone pulse therapy, rituximab, and intravenous immunoglobulin, the patient’s mental state deteriorated, presenting signs of thrombotic microangiopathy with posterior reversible encephalopathy syndrome. Intermittent haemodialysis and plasma exchange were initiated; however, her condition was not improved, and eculizumab administration was initiated thereafter. The patient’s symptoms showed a remarkable response to eculizumab; thrombotic microangiopathy findings, kidney function, and neurological symptoms improved after only two doses of eculizumab, and she achieved sustained remission. The extremely effective course of eculizumab treatment indicated that overt complement activation affected the development of thrombotic microangiopathy. Anti-neutrophil cytoplasmic antibody-associated vasculitis-related thrombotic microangiopathy may be mediated by complement activation, and prompt induction of eculizumab therapy may be a superior strategy to prevent organ damage. Further studies should elucidate the role of complement activation in associated vasculitis-related thrombotic microangiopathy and the efficacy of eculizumab treatment.
Thrombotic microangiopathy (TMA) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). Macrophage activation syndrome (MAS) is also a rare, life-threatening hyperinflammatory condition that is comorbid with SLE. However, the association between TMA and MAS in patients with SLE has rarely been assessed, and the difficulty of diagnosing these conditions remains prevalent. The efficacy of eculizumab has been reported for SLE patients whose conditions are complicated with TMA. However, no study has investigated the therapeutic efficacy of eculizumab for TMA concomitant with SLE-associated MAS. Herein, we report the first case of TMA concomitant with SLE-associated MAS that was initially refractory to conventional immunosuppressive therapy but showed remarkable recovery after eculizumab treatment. Furthermore, we evaluated serum syndecan-1 and hyaluronan levels, which are biomarkers of endothelial damage. We found that these levels decreased after the administration of eculizumab, suggesting that TMA was the main pathology of the patient. This case illustrates that it is important to appropriately assess the possibility of TMA during the course of SLE-associated MAS and consider the use of eculizumab as necessary.
Background The difference in the clinical impact of alcohol consumption on kidney function based on sex remains to be elucidated. This study aimed to assess the association between the dose of alcohol consumption and the incidence of proteinuria and chronic kidney disease stratified by sex. Methods This retrospective cohort study included 26,788 workers (19,702 men and 7086 women) with normal renal function (estimated glomerular filtration rate ≥60 mL/min/1.73 m2) at annual health examinations between January 2010 and March 2015 in Japan. The main exposure was alcohol consumption. The primary outcomes were the incidence of proteinuria (dipstick urinary protein ≥1) and incidence of chronic kidney disease (an estimated glomerular filtration rate <60 mL/min per 1.73 m2 and a 25% decrease from the baseline estimated glomerular filtration rate). Results During a median observational period of 4 years (interquartile range: 2–6), 1993 (10.1%) men and 462 (6.5%) women developed proteinuria, whereas 667 (3.4%) men and 255 (3.6%) women developed chronic kidney disease. After adjustment for clinically relevant factors using a Cox proportional hazards model, alcohol consumption of ≥40 g/day in females was significantly associated with the incidence of proteinuria (hazard ratio, 1.65; 95% confidence interval, 1.09–2.51) and chronic kidney disease (hazard ratio, 1.77; 95% confidence interval, 1.09–2.85). However, no significant association between alcohol consumption and primary outcomes was observed in men. Conclusions In conclusion, daily higher alcohol consumption was significantly associated with a higher incidence of proteinuria and chronic kidney disease among women. Women might be prone to high alcohol consumption with kidney dysfunction.
Background: Although previous studies have evaluated risk factors for the incidence of severe infection in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV), the relationship between body mass index (BMI) and severe infection in AAV has not been elucidated. We hypothesized that elderly AAV patients with a low BMI would be at a higher risk of infection. To evaluate this hypothesis, we investigated the association between underweight at AAV diagnosis and subsequent severe infection in elderly AAV patients.Methods: This single-center retrospective cohort study included 98 consecutive elderly AAV patients treated at the Aichi Medical University Hospital in Japan between 2004 and 2018. The relationships between BMI at diagnosis and subsequent first severe infection were assessed using multivariate Cox proportional hazards models.Results: During the entire follow-up period (median, 22 months; interquartile range, 7‒52 months), 32 (32.7%) patients developed at least one severe infection. Low BMI (< 18.5 kg/m2 compared with normal BMI [18.5‒23.0 kg/m2], adjusted hazard ratio [HR] = 2.70, 95% confidence interval [CI]: 1.18–6.17; P = 0.018) and use of methylprednisolone pulse therapy (adjusted HR = 3.09, 95% CI: 1.37–6.99; P = 0.007) were the significant predictors of severe infection. Furthermore, an interaction effect between unintentional body weight loss (> 10%) within 6 months before diagnosis and low BMI was observed (P < 0.001).Conclusions: Low BMI was associated with a higher risk of severe infection in elderly AAV patients, suggesting that careful management may be required to prevent the development of infection in patients with a low BMI. Further studies are needed to elucidate the optimal treatment strategy for these patients.
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