ABSTRACT
Despite being recognised much more frequently than in the past, renal involvement has not previously been regarded as a feature of Behcet's disease (BD). In this study we aimed to assess the frequency of renal involvement in BD by performing urinalyses of 674 consecutive BD patients; we also retrospectively evaluated the charts of 4212 BD patients for the incidence of glomerulonephritis (GN). Urinary abnormalities (proteinuria and/or haematuria) were present in 10.8%; and during a period of 23 years GN was detected by renal biopsy in seven (0.16%) BD patients. Two patients with GN were lost to follow-up; end-stage renal failure developed in only one patient, and she underwent renal transplantation. We were unable to determine any pathognomonic feature that was predictive of renal involvement. Although males tend to have a more serious clinical course of BD the incidences of urinary abnormalities and GN were similar in both sexes in our series. According to our results, we can conclude that urinary abnormalities are more frequent in BD; however, serious renal lesions develop in only very few of these patients.
Subject(s)
Behcet Syndrome/complications , Glomerulonephritis/etiology , Adult , Behcet Syndrome/drug therapy , Behcet Syndrome/urine , Biopsy , Female , Glomerulonephritis/drug therapy , Glomerulonephritis/pathology , Glucocorticoids/therapeutic use , Hematuria/etiology , Humans , Immunosuppressive Agents/therapeutic use , Kidney/pathology , Male , Proteinuria/etiology , Retrospective Studies , UrinalysisABSTRACT
Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia.
Subject(s)
Amyloidosis/etiology , Nephrotic Syndrome/etiology , Scleroderma, Systemic/complications , Amyloidosis/diagnosis , Amyloidosis/therapy , Biopsy, Needle , Disease Progression , Esophageal and Gastric Varices/diagnosis , Esophageal and Gastric Varices/therapy , Fatal Outcome , Gastrointestinal Hemorrhage/diagnosis , Gastrointestinal Hemorrhage/therapy , Humans , Male , Middle Aged , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/therapy , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/therapy , Severity of Illness IndexABSTRACT
Behçet's syndrome (BS), originally described as a triad of oral aphthae, genital ulcerations and uveitis, is a systemic vasculitis that is prevalent in the Middle east, Far East and in the Mediterranean basin. It is characterized by a heightened state of inflammation although the main drive that initiates and sustains this is not yet elucidated. Suppression of this inflammatory state constitutes the major goal of treatment and therapy is tailored according to the specific manifestations observed. We now have considerable more insight on drug management of BS compared to 20 years ago. Particularly, within the recent past we have learned to use more rationally the agents that were already available to us. This is especially true for azathioprine, cyclosporin A, thalidomide and colchicine. Promising data are also being collected with alpha-interferon. With these agents, significant progress has been achieved in the management of uveitis and mucocutaneous symptoms but treatment issues related to thrombotic problems, major vessel involvement and neurological disease have not yet been resolved.
