(99m)Tc-DPD scintigraphy is a highly sensitive technique for imaging cardiac ATTR amyloidosis and is an important investigation in the diagnostic pathway of patients with cardiac amyloidosis. It is not specific for ATTR in isolation but must be interpreted in a broad clinical context to avoid dangerous diagnostic errors. Diffuse skeletal muscle uptake identifies muscle as a hitherto unrecognized site that merits investigation as a target organ in ATTR amyloidosis.
High-grade (Perugini grade 2 or 3) cardiac uptake on bone scintigraphy with 99m Technetium labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (99m Tc-DPD) has lately been confirmed to have high diagnostic sensitivity and specificity for cardiac transthyretin (ATTR) amyloidosis. We sought to determine whether patient stratification by Perugini grade on 99m Tc-DPD scintigraphy has prognostic significance in ATTR amyloidosis. Methods and Results Patient survival from time of 99m Tc-DPD scintigraphy was determined in 602 patients with ATTR amyloidosis, including 377 with wild-type ATTR and 225 with mutant ATTR amyloidosis (ATTRm). Patients were stratified according to Perugini grade (0-3) on 99m Tc-DPD scan. The prognostic significance of additional patient and disease-related factors at baseline were determined. In the whole cohort, the finding of a Perugini grade 0 99m Tc-DPD scan (n=28) was invariably associated with absence of cardiac amyloid according to consensus criteria as well as significantly better patient survival compared to a Perugini grade 1 (n=28), 2 (n=436) or 3 (n=110) 99m Tc-DPD scan (p<0.005). There were no differences in survival between patients with a grade 1, grade 2 or grade 3 99m Tc-DPD scan in wild-type ATTR (n=369), V122I-associated ATTRm (n=92) or T60A-associated ATTRm (n=59) amyloidosis. Cardiac amyloid burden, determined by equilibrium contrast cardiac magnetic resonance imaging, was similar between patients with Perugini grade 2 and Perugini grade 3 99m Tc-DPD scans but skeletal muscle/soft tissue to femur ratio was substantially higher in the latter group (p<0.001). Conclusion 3 99m Tc-DPD scintigraphy is exquisitely sensitive for identification of cardiac ATTR amyloid, but stratification by Perugini grade of positivity at diagnosis has no prognostic significance.
We developed and validated a new accurate model for GFR assessment in cirrhosis, the Royal Free Hospital cirrhosis GFR, using readily available variables; this remains to be tested and incorporated in prognostic scores in patients with cirrhosis. (Hepatology 2017;65:582-591).
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