Epidemiology
Clinical presentation
Diagnosis
- Extracellular deposit of insoluble polymeric protein fibrils in tissue & organs
- Can be primary (AL type) or secondary (AA) to chronic inflammatory conditions such as:
- Inflammatory arthritis (e.g RA)
- Chronic infections (e.g bronchiectasis, tuberculosis, osteomyelitis)
- Inflammatory bowel disease (e.g Crohn's disease)
- Malignancy (e.g. lymphoma)
- Vasculitis
Clinical presentation
- Asymptomatic proteinuria or nephrotic syndrome
- Restrictive cardiomyopathy
- Hepatomegaly
- Peripheral neuropathy &/or autonomic neuropathy
- Visible organ enlargement (e.g. macroglossia)
- Bleeding diathesis
- Waxy thickening, easy bruising of skin
Diagnosis
- Tissue biopsy (e.g. abdominal fat pad)
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