A Rare Case of Renal AA Amyloidosis Secondary to Sjogren’s Syndrome
  • Mylene Costa
    Internal Medicine, Hospital Pedro Hispano, Porto, Portugal
  • Helena Greenfield
    Internal Medicine, Hospital Pedro Hispano, Porto, Portugal
  • Ricardo Pereira
    Internal Medicine, Hospital Pedro Hispano, Porto, Portugal
  • Teresa Chuva
    Nephrology, Instituto Português de Oncologia do Porto, Portugal
  • Rui Henrique
    Pathological Anatomy, Instituto Português de Oncologia do Porto, Portugal
  • Cátia Cunha
    Nephrology, Hospital Pedro Hispano, Porto, Portugal - Faculdade de Medicina de Lisboa, Centro Hospitalar Lisbona Norte, Lisbon, Portugal

Keywords

Sjogren's syndrome, AA amyloidosis, renal amyloidosis, renal biopsy

Abstract

AA amyloidosis is a rare complication of chronic inflammatory disorders and has been associated with rheumatoid arthritis and ankylosing spondylitis.
We present a case of AA amyloidosis secondary to Sjogren’s syndrome (SS). A 79-year-old woman presented with rapidly progressive renal failure and complaints of asthenia, anorexia and generalized oedema. She had severe renal failure (creatinine 6.0 mg/dl), with microscopic haematuria, nephrotic proteinuria and low serum albumin levels, and an increased erythrocyte sedimentation rate. Serum protein electrophoresis revealed a peak in the gamma globulin zone. The patient was started on haemodialysis and corticosteroids. Clinical results showed the patient met the diagnostic criteria for primary SS, and neoplastic haematological disease was excluded. Renal biopsy revealed a diagnosis of AA amyloidosis.
Renal AA amyloidosis is a rare condition in patients with primary SS. However, in patients with proteinuria and/or renal failure, it should be included in the differential diagnosis and a renal biopsy should be performed.

VIEW THE ENTIRE ARTICLE

References

  • Ooms V, Decupere M, Lerut E, Vanrenterghem Y, Kuypers DR. Secondary renal amyloidosis due to long-standing tubulointerstitial nephritis in a patient with Sjögren syndrome. Am J Kidney Dis 2005;46(5):e75–80.

  • Nikolova M, Ivanov G, Makova G. Sjogren’s syndrome and the kidney. Arch Immunol Allergy 2018;1(2):20–24.

  • Evans R, Zdebik A, Ciurtin C, Walsh S. Renal involvement in primary Sjogren’s syndrome. Rheumatology 2015;54:1541–1548.

  • François H, Mariette X. Renal involvement in primary Sjorgren’s syndrome. Nat Rev Nephrol 2016;12(2):82–93.

  • Erdogmus S, Kendi Celebi Z, Akturk S, Kumru G, Duman N, Ates K, et al. Profile of renal AA amyloidosis in older and younger individuals: a single-centre experience. Amyloid 2018;25(2):115–119.

  • Yilmaz M, Unsal A, Sokmen M, Kaptanogullari OH, Alkim C, Kabukcuoglu F, et al. Renal involvement in AA amyloidosis: clinical outcomes and survival. Kidney Blood Press Res 2013;37(1):33–42.

  • Lachmann HJ, Goodman HJ, Gilbertson JA, Gallimore JR, Sabin CA, Gillmore JD, et al. Natural history and outcome in systemic AA amyloidosis. N Engl J Med 2007;356(23):2361–2371.

  • Kweon SM, Koh JH, Lee HN, Kim E, So MW, Shin HJ, et al. Primary Sjogren syndrome diagnosed simultaneously with localized amyloidosis of the lacrimal gland: a case report. Medicine (Baltimore) 2018;97(23):e11014.

  • Dember LM. Amyloidosis-associated kidney disease. J Am Soc Nephrol 2006;17(12):3458–3471.

  • Brito-Zerón P, Retamozo S, Gandía M, Akasbi M, Pérez-De-Lis M, Diaz-Lagares C, et al. Monoclonal gammopathy related to Sjögren syndrome: a key marker of disease prognosis and outcomes. J Autoimmun 2012;39(1–2):43–48.

    • Views: 1150
    HTML downloads: 144
    PDF downloads: 559

    Published: 2019-09-16
    Issue: Vol 6 No 9 (view)

    How to cite:
    1.
    Costa M, Greenfield H, Pereira R, Chuva T, Henrique R, Cunha C. A Rare Case of Renal AA Amyloidosis Secondary to Sjogren’s Syndrome. EJCRIM 2019;6 doi:10.12890/2019_001226.