Secondary Causes of Systemic Amyloidosis: Identifying the Exception
Ulcerative colitis is not typically associated with secondary systemic amyloidosis among the listed conditions.
Understanding Secondary Systemic Amyloidosis
Secondary systemic amyloidosis, also known as AA amyloidosis, results from chronic inflammatory or infectious diseases that lead to deposition of amyloid A protein in various organs. This condition differs from other forms of amyloidosis such as AL amyloidosis (associated with plasma cell disorders) or ATTR amyloidosis (associated with transthyretin protein misfolding).
Common Causes of Secondary (AA) Amyloidosis
According to the ACC/AHA guidelines, secondary amyloidosis typically results from chronic inflammatory or infectious conditions 1. The conditions that are well-established causes include:
Chronic infections:
- Tuberculosis
- Leprosy
- Bronchiectasis (with chronic infection)
Chronic inflammatory conditions:
- Rheumatoid arthritis
- Crohn's disease
- Psoriasis
Malignancies:
- Multiple myeloma (primarily causes AL amyloidosis but can be associated with secondary amyloidosis in some cases) 1
Why Ulcerative Colitis Is the Exception
While inflammatory bowel diseases are mentioned in the guidelines as potential causes of secondary amyloidosis, there is a significant difference between Crohn's disease and ulcerative colitis in this regard:
- Crohn's disease is a well-established cause of secondary amyloidosis
- Ulcerative colitis is rarely associated with secondary amyloidosis 1
This distinction is important because both are inflammatory bowel diseases, but they differ in their propensity to cause secondary amyloidosis.
Evidence for Other Conditions
Psoriasis: Multiple case reports confirm the association between psoriasis and secondary amyloidosis, though it is relatively uncommon 2, 3. The chronic inflammatory nature of psoriasis can lead to AA amyloid deposition.
Tuberculosis and Leprosy: These chronic infections are classic causes of secondary amyloidosis due to persistent inflammation 1.
Bronchiectasis: The chronic infection and inflammation associated with bronchiectasis can lead to secondary amyloidosis 1.
Multiple Myeloma: While multiple myeloma is primarily associated with AL amyloidosis (primary), approximately 10-15% of patients with multiple myeloma also have AL amyloidosis 1. It's important to note that this is typically AL amyloidosis rather than AA amyloidosis, but it is still a form of systemic amyloidosis.
Clinical Implications
Understanding the causes of secondary amyloidosis is crucial for:
- Early detection in high-risk patients
- Proper management of the underlying condition to prevent amyloid deposition
- Monitoring for organ involvement, particularly the kidneys and heart
Diagnostic Approach
When secondary amyloidosis is suspected:
- Tissue biopsy with Congo red staining is essential for diagnosis
- Immunohistochemical testing can confirm AA protein type
- Evaluation of organ function, particularly renal and cardiac
Treatment Considerations
The primary treatment approach for secondary amyloidosis focuses on controlling the underlying inflammatory condition, which may halt or slow the progression of amyloid deposition.