Focal Segmental Glomerulosclerosis Clinical Presentation

Updated: Dec 07, 2020
  • Author: Sreepada TK Rao, MD, FACP; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Presentation

History

Patients with focal segmental glomerulosclerosis (FSGS) typically present with signs and symptoms of nephrotic syndrome. In children, this usually begins with facial swelling, followed by swelling of the entire body. Adults can present with dependent edema. Typically, edema develops over a few weeks, but the onset may be abrupt, with weight gain of 15-20 lb or more. Frequently, the onset of edema follows a recent upper respiratory tract infection. Foamy urine may be noted. Fatigue and loss of appetite are common.

To identify causes of secondary FSGS, details about the following should be elicited [37] :

  • Birth history (eg, low birth weight/premature birth)
  • Congenital urinary tract malformations (eg,  vesicoureteral reflux)
  • Medical comorbidities
  • Pre-existing kidney disease
  • Exposure to drugs/toxins
  • Recent viral illnesses
  • Family history
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Physical Examination

The most common clinical presenting feature, found in more than 70% of patients, is nephrotic syndrome, which is characterized by generalized or dependent edema, massive proteinuria, hypoalbuminemia, and hyperlipidemia.  In patients with primary (essential) hypertension and analgesic abuse, nephrotic syndrome is not a common manifestation (although hypertension may be observed in patients with nephrotic syndrome from all causes).

Occasionally, routine urinalysis may reveal proteinuria, prompting referral to a nephrologist. Less than a third of patients with FSGS present with nonnephrotic proteinuria along with microscopic hematuria and hypertension. Typically, edema develops over a few weeks, but the onset may be abrupt, with weight gain of 15-20 lb or more. Frequently, the onset of edema follows a recent upper respiratory tract infection.

Pleural effusion and ascites may be present; pericardial effusions are rare. Gross edema may predispose patients to ulcerations and infections in dependent areas (eg, lower extremities). Abdominal pain, a common finding in children, may be a sign of peritonitis. Rarely, xanthomas may be evident in association with severe hyperlipidemia. In many patients, physical examination findings are normal except for generalized or dependent edema.

Severe hypertension (ie, diastolic blood pressure of 120 mm Hg or more) is not uncommon, especially in black patients with renal insufficiency. [2] Rarely, patients experience severe renal failure with signs and symptoms of advanced uremia (eg, nausea, vomiting, bleeding, seizures) or altered mental status.

Patients with FSGS secondary to diseases such as massive obesity, reflux nephropathy, and renal dysplasia/agenesis may present with non-nephritic proteinuria. These patients often experience worsening renal function over a course of months to years.

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