Al amyloidosis renal changes E85.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Al-Amyloidosis renal involvement; Al Amyloidosis with kidney involvement; AL amyloidosis with renal involvement; Amyloid kidney

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AL-amyloidosis of the kidney (see also systemic amyloidosis) is defined by fibrillary deposits of amyloid in the renal parenchyma, consisting of monoclonal light chains.

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(Nephrogenic) AL amyloidosis occurs not only in multiple myeloma but also in monoclonal gammopathy of indeterminate significance (MGUS). In about 30% of patients with AL amyloidosis, multiple myeloma can be diagnosed as the underlying disease. 15% of patients with multiple myeloma develop AL amyloidosis with renal involvement.

Clinical features
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In systemic AL amyloidosis, 1 or more organ involvement occurs:

  • renal involvement (in 50%): nephrotic syndrome
  • restrictive cardiomyopathy (at 30%)
  • AL skin and mucous membrane changes (30-50%): Purpura (face, especially eyelids, scalp, palmae and plantae), less frequently nodular skin changes.
  • Liver involvement (16%)
  • Amyloid arthropathy (especially in beta2-micoglobulin-associated amyloidosis)
  • 50% show a reduced glomerular filtration rate at diagnosis
  • Hypertension (25%)
  • peripheral neuropathymacroglossia
  • Carpal tunnel syndrome

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Oral combination therapy with melphalan and prednisolone improves survival rate for a few months

High-dose chemotherapy with melphalan and autologous stem cell transplantation

Bortezomib with dexamethasone (Huang X et al. 2016)

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The AL - amyloidosis has a bad prognosis if left untreated:

  • the average survival time is 12 months
  • cardiac involvement leads to death in 40% of patients

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  1. Favà A et al (2018) Treatment of multiple myeloma with renal involvement: the nephrologist's view. Clin Kidney J 11:777-785.
  2. Huang X et al (2016) Bortezomib with dexamethasone as first-line treatment for AL amyloidosis withrenal involvement. Amyloid 23:51-57.
  3. Hutchison CA et al. (2019) High cutoff versus high-flux haemodialysis for myeloma cast nephropathy inpatients receiving bortezomib-based chemotherapy (EuLITE): a phase 2 randomised controlled trial.Lancet Haematol 6:e217-e228.
  4. Kanzaki G et al (2019) Monoclonal Immunoglobulin Deposition Disease and Related Diseases. J Nippon Med Sch 86:2-9.
  5. Leung N et al (2018) Dysproteinemias and Glomerular Disease. Clin J Am Soc Nephrol 13:128-139.
  6. Sallée M et al (2019) Myeloma cast nephropathy: the dusk of high cutoff haemodialysis. Lancet Haematol 6:e174-e176.


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Last updated on: 29.10.2020