Organ | Diagnostic criteria* |
Kidney | 24-hour urine protein >0.5 g/day, predominantly albumin |
Heart | Mean LV wall thickness >12 mm in diastole on echocardiography (no other cardiac cause) Elevated NT-proBNP (>332 ng/L) in the absence of renal failure or atrial fibrillation |
Liver | Hepatomegaly with total liver span >15 cm in the absence of heart failure or alkaline phosphatase >1.5 times institutional upper limit of normal |
Nerve | Peripheral: symmetric lower extremity sensorimotor peripheral neuropathy Autonomic: gastric emptying disorder, pseudo-obstruction, postural hypotension, erectile dysfunction (males), voiding dysfunction unrelated to direct organ infiltration |
Gastrointestinal tract | Direct biopsy verification with symptoms |
Lung | Direct biopsy verification with symptoms or Radiographic pattern of interstitial infiltration |
Soft tissue | Macroglossia Arthropathy Claudication, presumed vascular amyloid Skin lesions Myopathy by biopsy or pseudohypertrophy of muscle Lymphadenopathy (may be localised) Carpal tunnel syndrome |
*Non-invasive diagnostic criteria in patients for whom a diagnosis of systemic amyloidosis has been made by tissue biopsy; once the diagnosis of systemic amyloidosis has been established, biopsy of organs to determine extent of involvement is not recommended.
LV, left ventricular; NT-proBNP, N-terminal fragment of pro-brain natriuretic peptide.