Co-presentation with both ANCA and anti-GBM antibodies is thought to be relatively rare. Current studies of such 'double-positive' cases report small numbers and variable outcomes. To study this further we retrospectively analyzed clinical features and long-term outcomes of a large cohort of 568 contemporary patients with ANCA-associated vasculitis, 41 patients with anti-GBM disease, and 37 double-positive patients with ANCA and anti-GBM disease from four European centers. Double-positive patients shared characteristics of ANCA-associated vasculitis (AAV), such as older age distribution and longer symptom duration before diagnosis, and features of anti-GBM disease, such as severe renal disease and high frequency of lung hemorrhage at presentation. Despite having more evidence of chronic injury on renal biopsy compared to patients with anti-GBM disease, double-positive patients had a greater tendency to recover from being dialysis-dependent after treatment and had intermediate long-term renal survival compared to the single-positive patients. However, overall patient survival was similar in all three groups. Predictors of poor patient survival included advanced age, severe renal failure, and lung hemorrhage at presentation. No single-positive anti-GBM patients experienced disease relapse, whereas approximately half of surviving patients with AAV and double-positive patients had recurrent disease during a median follow-up of 4.8 years. Thus, double-positive patients have a truly hybrid disease phenotype, requiring aggressive early treatment for anti-GBM disease, and careful long-term follow-up and consideration for maintenance immunosuppression for AAV. Since double-positivity appears common, further work is required to define the underlying mechanisms of this association and define optimum treatment strategies.
- MeSH
- ANCA-asociované vaskulitidy krev imunologie mortalita terapie MeSH
- antirenální glomerulonefritida krev imunologie mortalita terapie MeSH
- autoprotilátky krev MeSH
- chronické selhání ledvin krev imunologie mortalita terapie MeSH
- dialýza ledvin MeSH
- dítě MeSH
- dospělí MeSH
- imunosupresiva terapeutické užití MeSH
- Kaplanův-Meierův odhad MeSH
- krvácení imunologie MeSH
- ledviny imunologie patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- následné studie MeSH
- plicní nemoci imunologie MeSH
- přežití po terapii bez příznaků nemoci MeSH
- protilátky proti cytoplazmě neutrofilů krev MeSH
- recidiva MeSH
- retrospektivní studie MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- věkové faktory MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- srovnávací studie MeSH
2nd edition 144 stran : ilustrace
Early detection of renal problems coupled with the appropriate therapeutic strategy can radically reduce the progressive nature of, and complications associated with, chronic kidney disease, and in many instances will result in the successful treatment of acute kidney injury. As many patients will not be seen by nephrologists, it is essential that all healthcare professionals, in hospitals and in the community, have an awareness of renal disease – the presenting signs, differential diagnoses, treatment strategies and approach to the management of complications. 'Fast Facts: Renal Disorders' is an easy-to-read, evidence-based guide to renal diseases and disorders for all doctors, nurses and medical students. It includes:• A clear explanation of proteinuria, hematuria, electrolyte imbalances and acid–base disorders• A concise summary of kidney function tests, imaging techniques and biopsy• Important questions for prompt diagnosis of acute kidney injury• Management options for chronic kidney disease and its complications• Practical guidance on the most common renal problems, including glomerulonephritis, systemic disease, UTIs and kidney stonesWritten by three specialists of international repute, 'Fast Facts: Renal Disorders' provides the key information required for the optimal care of renal patients. This fully updated second edition will help healthcare professionals assess, identify, treat and refer patients with renal problems appropriately. Directly applicable to the clinical setting, it is essential reading for all primary care providers, junior hospital doctors, specialist trainees, renal nurses and medical students.Contents:• Proteinuria, hematuria and renal investigations• Electrolyte disturbances and acid–base disorders• Acute kidney injury• Chronic kidney disease• Hypertension and diabetic nephropathy• Glomerulonephritis• Systemic disease• Inherited kidney disease• Urinary tract infection• Kidney stones• Urinary tract obstruction and tumors• Renal replacement therapy and transplantation
- MeSH
- nefrologie MeSH
- nemoci ledvin MeSH
- Publikační typ
- příručky MeSH
- NLK Obory
- nefrologie
Oxford Medical Publications
3rd ed. 671 s. : il.
- Konspekt
- Patologie. Klinická medicína
- NLK Obory
- nefrologie
xvii, 595 s. : il.
- Konspekt
- Patologie. Klinická medicína
- NLK Obory
- nefrologie