A Rare Case of Renal AA Amyloidosis Secondary to Sjogren’s Syndrome

Authors

• 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

mylenecosta@gmail.com

Keywords

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.