renal apolipoprotein A4 amyloidosis.

Apolipoprotein A-IV associated amyloidosis (AApoAIV amyloidosis) is a rare cause of amyloidosis with only a single reported case.

Renal AApoAIV amyloidosis typically presents with progressive chronic kidney disease and histologically exhibits extensive medullary involvement with sparing of the cortex.

The diagnosis is best established by mass spectrometry. Hence, a high degree of suspicion and examination of the renal medulla is required to make the diagnosis.

See also

 renal amyloidosis

References

 Clinical, biopsy, and mass spectrometry characteristics of renal apolipoprotein A-IV amyloidosis. Dasari S, Amin MS, Kurtin PJ, Vrana JA, Theis JD, Grogg KL, Alexander MP, Nasr SH, Fervenza FC, Leung N, Sethi S. Kidney Int. 2016 Jun 2. pii: S0085-2538(16)30130-2. doi : 10.1016/j.kint.2016.04.003 PMID: 27262366