Aa-type amyloidosis E85.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

AA-type amyloidosis; systemic amyloidosis

Definition
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Systemic amyloidosis with deposition of amyloid A protein (AA), a fragment of serum amyloid A protein (SAA). One speaks of idiopathic AA amyloidosis in the absence of a recognizable underlying disease.

Occurrence/Epidemiology
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Most common systemic amyloidosis worldwide. Incidence (Central Europe): 1/100.000 inhabitants/year.

Etiopathogenesis
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Clinical features
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  • Integument: Skin symptoms are generally rather rare. The occurrence of bleeding and alopecia was described by amyloid deposits in the dermis and subcutis.
  • The skin symptoms are accompanied by unspecific general symptoms caused by the primary underlying disease.
  • Extracutaneous manifestation: Cardinal symptom: Progressive renal insufficiency due to amyloid deposits in the basal membrane of the glomerula. Deposits also in the liver, gastrointestinal tract, adrenal glands and CNS.

Histology
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Therapy
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Treatment according to the underlying disease and by internists. Use of colchicine in amyloidosis in hereditary Mediterranean fever.

Literature
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  1. Akpolat T et al (2000) Behcet's disease and AA-type amyloidosis. At J Nephrol 20: 68-70
  2. Boorjian S (2002) A rare case of painless gross hematuria: primary localized AA-type amyloidosis of the urinary bladder. Urology 59: 137
  3. Cohen AS, Jones LA (1991) Amyloidosis. Curr Opin Rheumatol 3: 125-138
  4. Cohen AS, Jones LA (1993) Advances in Amyloidosis. Curr Opin Rheumatol 5: 62-76
  5. Csikos M et al (2003) Dystrophic epidermolysis bullosa complicated by cutaneous squamous cell carcinoma and pulmonary and renal amyloidosis. Clin Exp Dermatol 28: 163-166
  6. Escriba A et al (2000) Secondary (AA-type) amyloidosis in patients with polymyalgia rheumatica. At J Kidney Dis 35: 137-140
  7. Hazenberg BP et al (2007) Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis. Amyloid 14: 133-140
  8. Komatsuda A et al (2003) Amyloid A-type renal amyloidosis in a patient with sarcoidosis: report of a case and review of the literature. Clin Nephrol 60: 284-288
  9. Lubarsch O (1899) Hyaline and amyloid degeneration. Erg allg Path 4: 449-460
  10. Picken MM (2007) New insights into systemic amyloidosis: the importance of diagnosis of specific type. Curr Opin Nephrol Hypertens 16: 196-203
  11. Ruzicka T et al (1990) Cutaneous amyloidosis. Dermatologist 41: 245-255
  12. Virchow R (1854) On the course of amyloid degeneration. Virch Arch 8: 364-368

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Last updated on: 29.10.2020