RESUMEN
PURPOSE OF REVIEW: Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare, multisystem, autoimmune disease characterised by microvascular inflammation. Over the past 20 years, advances in immunological management have improved short-term patient outcomes. Longer-term patient outcomes remain poor with cardiovascular disease now the leading cause of death in AAV. Here, we examine the potential pathways that contribute to the increased risk of cardiovascular disease in AAV and the current evidence to manage this risk. RECENT FINDINGS: The incidence of cardiovascular disease in AAV exceeds that expected by traditional risk factors alone, suggesting a contribution from disease-specific factors. Similarly, it is unclear how different immunosuppressive therapies contribute to and modify cardiovascular risk, and there is a paucity of data examining the efficacy of traditional cardioprotective medications in AAV. There is a lack of evidence-based cardiovascular risk assessment tools and cardioprotective therapies in patients with AAV which should be addressed to improve long-term outcomes.
Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Enfermedades Cardiovasculares , Humanos , Enfermedades Cardiovasculares/etiología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Inflamación/complicaciones , Factores de Riesgo , Anticuerpos Anticitoplasma de Neutrófilos , Citoplasma/metabolismoRESUMEN
Membranous nephropathy with microspherular deposits is a rare renal condition associated with sub-nephrotic or nephrotic-range proteinuria. We report a case presenting with severe nephrotic syndrome and pathological features of collapsing glomerulopathy. This is the first case we are aware of that progressed to requiring dialysis. The patient received rituximab and corticosteroids. She has now been off dialysis for over a year with both serum creatinine and urine protein-creatinine ratio returning to baseline.