Focal segmental glomerulosclerosis with occult onset of proteinuria

FSGS

Focal segmental glomerulosclerosis (FSGS) nephrotic syndrome in adults the third most common cause (20% to 30%), especially in the black. FSGS is idiopathic, but also related to intravenous drug use, can be found in 20% of non-HIV-positive homosexual persons.

Evidence suggests that a non-immunoglobulin proteins circulating factor-mediated glomerular permeability. Glomerular permeability changes in protein can cause hardening. FSGS renal medulla begin in the near (not easy in biopsy samples). Showed segmental hyalinization, nodular or coarse granular IgM and C3 deposition and diffuse podocyte foot processes disappear. Global sclerosis can occur, leading to glomerular atrophy.

Symptoms, signs and diagnosis

Although sometimes the only signs of asymptomatic non-nephrotic range proteinuria, FSGS patients with nephrotic syndrome usually presents with hematuria, hypertension and renal insufficiency. Typical non-selective proteinuria. IgG levels are often lower. Diagnosis was confirmed by biopsy.

Prognosis and Treatment

Because the treatment is not very effective, and spontaneous remission is rare, so the prognosis is poor. More than 50% of patients with renal failure occurred in 10 years, 20% of end-stage renal failure in two years. The more children, the adult prevalence progress faster. Capillaries disappear (collapsing variation) poor prognosis. Pregnancy can aggravate FSGS.

In 20% to 30% of kidney transplant patients in FSGS recurrence, sometimes within a few hours after transplantation proteinuria appears. Recurrence of FSGS patients, 30% to 50% graft loss; young children, 3 years after the onset of development to mesangial proliferative renal failure and patients with the highest risk.

FSGS induced nephrotic syndrome due to heroin addiction, as early in the disease to stop using heroin can complete remission of nephrotic syndrome.

FSGS sometimes spontaneous regression or corticosteroids effective (such as prednisone 40 ~ 80mg / d8 ~ 12 weeks, followed the next day prednisone 30 ~ 40mg8 ~ 12 weeks). If only mild improvement or recurrence, the plus cyclophosphamide (day 2 ~ 3mg / kg12 week) or cyclosporine (adult daily 5mg / kg or children's daily 6mg / kg16 week) can cause remission. For advanced disease, long-term use of ACE inhibitors tended to reduce proteinuria, and may slow down disease progression. Another option is to merge plasmapheresis tacrolimus immunosuppression. Active treatment can reduce progression to renal failure but increases the risk of serious complications. Anticoagulant and antithrombotic usefulness has not been determined.