Table 2

Consensus criteria for organ involvement

Organ Diagnostic criteria*
Kidney24-hour urine protein >0.5 g/day, predominantly albumin
HeartMean 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
LiverHepatomegaly with total liver span >15 cm in the absence of heart failure or
alkaline phosphatase >1.5 times institutional upper limit of normal
NervePeripheral: 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 tractDirect biopsy verification with symptoms
LungDirect biopsy verification with symptoms or
Radiographic pattern of interstitial infiltration
Soft tissueMacroglossia
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.