Renal Amyloidosis—A Fourteen-year Follow-up

摘要

Forty-eight cases of histologically proven renal amyloidosis are presented, with clinical details and follow-up data of up to 14 years following establishment of the diagnosis. The average length of follow-up was 29 months. The diagnosis was established by fluorescence with Thioflavine T and Congo Red birefringence throughout the series, and the last seven renal biopsies were confirmed by electron microscopy.The over-all incidence of renal amyloidosis in a series of 1,500 renal biopsies was 3 per cent. Many of these cases occurred in patients in whom the diagnosis was unsuspected. Only with the use of the appropriate stains was the diagnosis established. It is suggested that histological staining for amyloid should be performed routinely if cases are not to be missed. Attention is drawn to the epithelial spicular deposits with silver stains, that may be found in some cases.Five cases are presented in which there was evidence of regression of amyloidosis. These were all cases of secondary amyloidosis in whom improvement coincided with adequate treatment of the underlying pathology. It is suggested that energetic treatment of chronic inflammatory disease causing amyloidosis may be effective in preventing progessive renal damage, providing that renal function is not too severely impaired.Twenty-four of the 48 patients had some degree of hypertension (B.P. diastolic more than 90 mmHg) at some stage of the disease. The importance of adequate control of hypertension together with its frequency in amyloid disease is stressed. Other complicating factors of renal amyloid disease, such as fluid depletion and renal vein thrombosis, are discussed.Detailed post-mortem studies on 17 patients are presented. Although there was histological evidence of widespread infiltration throughout the body, clinical evidence of organs other than the kidney being involved was found in few instances.Six cases have been followed for more than five years after the histological diagnosis of renal amyloid was made. It is suggested that if due attention is paid to the control of the underlying pathology, early control of blood-pressure, and the proper management of the usual complications of renal disease, the prognosis of renal amyloidosis need not be as grave as hitherto suspected.