Clinical and therapeutic aspects of AA amyloidosis

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Summary

Approach to the management of AA amyloidosis complicating RA.

  • (A)

    In case of proteinuria or loss of renal function a rectal biopsy or a subcutaneous fat biopsy is a suitable screening method for the detection of amyloidosis. If in any doubt, try to ascertain the diagnosis by renal biopsy. Adequate staining with alkaline Congo red and preferably immunohistochemical staining with anti-AA antibodies should be performed. Beware of renal pathology other than amyloidosis even in the presence of a positive rectal biopsy.

  • (B)

    A vigorous attempt to control disease activity of the RA should be made in order to eliminate the production of SAA, an acute phase protein. The response to treatment should be monitored by serial measurements of CRP and preferably SAA.

  • (C)

    The function of some vital organs should be evaluated:

    • (a)

      endogenous creatinine clearance and the extent of proteinura;

    • (b)

      electrocardiogram and optional echocardiography;

    • (c)

      thyroid function and adrenocortical function;

    • (d)

      intestinal absorption tests;

    • (e)

      optional—SAP scintigraphy and turnover studies.

    • (D)

      Attention should be given to adequate supportive treatment:

      • (a)

        blood pressure control;

      • (b)

        treatment of intercurrent infections;

      • (c)

        corticosteroids during major surgical procedures;

      • (d)

        pay attention to the possible effect of NSAID on proteinuria and renal function.

      • (E)

        In case of total renal failure or uncontrollable proteinuria:

        • (a)

          consider the possibility of primary renal transplantation;

        • (b)

          otherwise regular haemodialysis is indicated.

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