Nefro - 22-2.indd
نویسندگان
چکیده
Pauci -immune necrotising glomerulonephritis with extracapillary proliferation is a common renal histological manifestation of systemic vasculitis. Although its aetiopathogenesis is not well known, it is possible that environmental factors may have a bearing on genetically predisposed subjects, since there are many familial cases of systemic vasculitis. The frequent association between systemic vasculitis and ANCAs suggests autoimmune mechanisms play a part in its pathogenesis. We report two cases of necrotising extracapillary proliferative glomerulonephritis in two siblings sharing the same environment. We do not know if the first case had ANCAs, meaning the role of the autoimmune mechanism is not clear (diagnosis was made in 1988 when it was not possible to measure ANCAs in this hospital). Since the second case had the same kind of illness associated to positive ANCAs and autoimmune hypothyroidism, we presume that both siblings presented the same pathogenesis. These two cases of familial vasculitis appear to support the hypothesis that environmental, genetic and autoimmune factors influence the pathogenesis of systemic vasculitis. Key -Words: ANCA; familial systemic vasculitis; necrotising glomerulonephritis. INTRODUCTION Pauci -immune necrotising glomerulonephritis (PING) with extracapillary proliferation is the renal anatomopathological expression of systemic vasculitis. This disease typically affects small to medium -size blood vessels and is commonly associated with the presence of antineutrophil cytoplasmic antibodies (ANCAs)1. The pathogenesis of systemic vasculitis is unknown, although it involves genetic and environmental factors2,3. The literature describes different associations of familial vasculitis4,5. We report the presence of necrotising glomerulonephritis in two siblings.
منابع مشابه
Nefro - 22-4.indd
The uraemic syndrome is a complex mixture of organ dysfunctions attributed to the retention of a myriad of compounds that under normal conditions are excreted by healthy kidneys. While recent years have seen major steps in the identification and characterisation of uraemic retention solutes and in the knowledge of their patho-physiological importance, this knowledge remains far from complete. T...
متن کاملNefro - 22-2.indd
Introduction: Patients with chronic kidney disease have greater mortality than the general population, mainly due to cardiovascular disease. Left ventricular hypertrophy, common in these patients, is also associated with increased mortality due to cardiovascular events. The prevalence of left ventricular hypertrophy and cardiovascular disease is influenced by traditional and emerging risk facto...
متن کاملNefro - 22-2.indd
Anti -neutrophil cytoplasmic antibodies (ANCA)associated small -vessel vasculitides include Wegener’s granulomatosis, microscopic polyangiitis, Churg -Strauss syndrome and renal -limited vasculitis. While conventional immunosuppressive therapy with oral cyclophosphamide and corticosteroids has dramatically improved the prognosis of patients with these potentially life -threatening disorders, th...
متن کاملNefro-urological mortality causes in Jerez de los Caballeros (Badajoz) in the nineteenth century.
The causes of mortality from nefro-urologic diseases in Jerez de los Caballeros (Badajoz) during the nineteenth century will be our object of study. We have analyzed the death registry books of the parishes in Jerez. The percentage of deaths from nefro-urologic diseases compared to other pathologies is 0.8%, being most affected males in age ranges from 25-34 and 65-75 years of age. The months w...
متن کامل