FSGS treatment and prognosis

Focal segmental glomerulosclerosis (focal segmental glomerular scerosis, FSGS) is the primary glomerular diseases in a unique set of clinical and pathological syndrome, lesions characteristic is the part of some of the glomeruli and glomerular capillary climbthe segmental sclerotic changes and hyaline change, and the deep renal cortex next to the marrow glomerular First of all involved; clinical manifestations of proteinuria, nephrotic syndrome, poor response to a variety of treatment, the disease was chronic progressive process, and ultimately, chronic renal failure.
Clinical manifestations
FSGS seen in any age, usually occurs in children and adolescents, with an average age of 21. Men than women. No characteristic clinical manifestations, the most common first symptom of nephrotic syndrome, and about 2/3 of patients with massive proteinuria and severe edema. Microscopic hematuria common, accounting for 90%, gross hematuria.Small subset of patients is found in a routine urine examination, urine protein, and this type of asymptomatic proteinuria sustainable for a long time, the prognosis is good.Proteinuria and the vast majority of non-selective, but early high or moderate selectivity.Light, blood pressure and severe rise. Renal impairment seen in newly diagnosed, but more in the course of the gradual emergence. Serum C3 levels were normal. IgG levels decline. Often the performance of proximal tubular dysfunction, such as grapes, diabetes, and amino acid in urine. The above symptoms of upper respiratory tract infections or allergies can make worse. Most patients with glomerular filtration rate decreased.
Treatment
The treatment of this disease more difficult to advocate more comprehensive treatment.
(1) General treatment: in patients with renal insufficiency should be low-protein diet plus keto acid preparations for treatment can delay progress. Edema, hypertension, sodium restriction, diuretic, antihypertensive drugs such as converting enzyme or receptor inhibitors, calcium channel blockers.
(2) Hormones: observed in recent years to extend the hormone treatment can make the effective rate of 40%. Minimal change to the disease, history of hormone-sensitive response to treatment are mostly good. Prednisone 0.5 to 1mg / (kg × d) '6 ~ 8 weeks.Then gradually reducing the transition to every other day therapy, the total course of more than one year. However, patients with a higher hardening rate, the use of hormones should be careful. (
3) immunosuppressive agents: this is still controversial. Alkylating agents: cyclophosphamide, tumor Ning, azathioprine, hormone-sensitive, immunosuppressive agents can significantly reduce the recurrence rate and prolong the remission period, reducing the hormone dosage. Hormone antagonist, it was suggested that cyclophosphamide allows the original hormone antagonistic to sensitive. In recent years, was also noted with cyclosporine A or mycophenolate, etc. have some efficacy in the treatment of this disease.
(4) Other treatments: converting enzyme inhibitors and receptor blockers can reduce the amount of urinary protein, reducing pressure and glomerular basement membrane permeability of the ball and protect renal function. This disease there are systemic and renal hypercoagulable state, anticoagulation therapy have a certain effect. Often used dipyridamole, heparin, urokinase, defibrase warfarin. Vitamin E is an endogenous antioxidant, protection of renal function.
Prognosis
The prognosis of a variety of factors:
(1) the degree of proteinuria: the nephrotic syndrome and nephrotic syndrome five years survival rates were 76% and 92%. (2) response to treatment: where very little development of hormone-sensitive to renal failure. Complete remission of end-stage renal failure rate was 15%, not in complete remission compared to 85%.
(3) the time of biopsy, serum creatinine, high blood pressure and poor prognosis.
(4) BIOPSY: the extent of damage, especially the degree of interstitial fibrosis is a reliable indicator to predict end-stage renal failure incidence.
(5) serum C3 levels prognosis is good.

After Kidney Transplant, FSGS Comes Back again