Secondary Amyloidosis in Progressive Systemic Sclerosis

Abstract

Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia.

Primary systemic amyloidosis presenting as idiopathic inflammatory colitis

“…With the widespread use of antibiotics and anti-inflammatory medications, the predominant type of amyloid in the western world has shifted from secondary (AA) amyloidosis to primary (AL) amyloidosis. Other recognised types of amyloid include: patients undergoing long-term haemodialysis with deposits of β2-microglobulin11 12; hereditary amyloidosis (an autosomal dominant inheritance of amyloidogenic proteins) and Senile amyloidosis is found in 10–36% of patients over 80 years old and occasionally in younger individuals 9 10…”