Some patients with severe alpha-1-antitrypsin deficiency develop renal disease.
The patient may show:
(1) proteinuria (including the nephrotic syndrome)
(2) worsening renal function
(3) nothing out of the ordinary (especially if overshadowed by other manifestations of A1AT deficiency
The most common form of renal involvement is membranoproliferative glomerulonephritis.
Other patients may show:
(1) membranous GN
(2) focal segmental MPGN
(3) diffuse endocapillary proliferative GN
(4) mesangial proliferative GN
(5) focal segmental mesangial proliferative
(6) rapidly progressive GN associated with necrotizing vasculitis
The diagnosis requires exclusion of other causes of glomerulonephritis.
The nephropathy improves following liver tranaplant.
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Specialty: Nephrology, Clinical Laboratory
ICD-10: ,
