Progressive Lipodystrophy Workup
- Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD more...
Urinalysis may be helpful in progressive lipodystrophy. Approximately one third of patients with this condition have some degree of glomerulonephritis, and, if hypocomplementemia is present, the risk is higher. Manifestations of glomerulonephritis range from asymptomatic proteinuria to a nephrotic syndrome and renal insufficiency.
Baseline creatinine and blood urea nitrogen studies, as well as urine studies, should be performed to detect proteinuria and hematuria.
A CBC count should be obtained. Normochromic normocytic anemia is often present.
Immune studies can be ordered. Immune studies may be needed to identify patients with associated immune disorders, particularly if the diagnosis of partial lipodystrophy is made at a younger age. The presence of serum antinuclear antibodies and anti–double-stranded DNA antibodies has been reported in some patients.
Complement levels may be determined. The most common laboratory abnormality in patients with progressive lipodystrophy is a low complement level. Hypocomplementemia is characterized by a low C3 complement level, a normal C4 level, and the presence of C3 nephritic factor.
Lipid studies may be performed. Hypertriglyceridemia secondary to nephrotic syndrome may be present in some patients.
Head MRIs show evidence of fat loss in progressive lipodystrophy patients.
Immunofluorescence studies in progressive lipodystrophy patients show deposits of C3 in a granular pattern in the basement membranes.
Skin biopsy findings confirm the progressive lipodystrophy diagnosis. Kidney biopsy is required if a glomerulonephritis is associated with the progressive lipodystrophy.
Cutaneous biopsy in progressive lipodystrophy reveals a reduction or absence of subcutaneous fat in affected areas. Subcutaneous fat cells are decreased in number.
Traces of adipose tissue may be found around hair follicles and sebaceous glands. The dermis and epidermis are normal.
Renal biopsy findings are characterized by a membranoproliferative glomerulonephritis with a proliferation of mesangial cells and matrix, as well as thickening of the basement membranes by amorphous electron-dense deposits.
Immunofluorescence studies show deposits of C3 in a granular pattern in the basement membranes.
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