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Secondary amyloidosis in progressive systemic sclerosis.

G E Pamuk1, O N Pamuk, M R Altiparmak

  • 1Cerrahpaşa Medical School, University of Istanbul, Turkey. onpamuk80@hotmail.com

Clinical Rheumatology
|September 1, 2001
PubMed
Summary

Progressive systemic sclerosis (PSS) rarely causes secondary amyloidosis. This case highlights a PSS patient who developed nephrotic syndrome from amyloidosis, ultimately succumbing to gastrointestinal bleeding.

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Area of Science:

  • Rheumatology
  • Nephrology
  • Pathology

Background:

  • Progressive systemic sclerosis (PSS) is a multisystem autoimmune connective tissue disease.
  • Kidney involvement in PSS can lead to significant morbidity.
  • Secondary amyloidosis is an uncommon complication of PSS.

Observation:

  • A patient with a 9-year history of PSS presented with nephrotic syndrome.
  • Renal biopsy confirmed the presence of secondary amyloidosis.
  • The patient also had oesophageal telangiectasia.

Findings:

  • The patient's nephrotic syndrome was attributed to secondary amyloidosis.
  • The underlying cause of the amyloidosis was progressive systemic sclerosis.
  • Massive upper gastrointestinal bleeding, secondary to oesophageal telangiectasia, was the cause of death.

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Implications:

  • This case underscores the importance of considering secondary amyloidosis in PSS patients with nephrotic syndrome.
  • Early diagnosis and management of renal complications in PSS are crucial.
  • Oesophageal telangiectasia represents a potential source of life-threatening bleeding in PSS.