Objectives To analyze the main epidemiological, clinical and immunological characteristics and baseline predictors associated with survival in a large cohort of patients with systemic amyloidosis.
Methods In May 2013, the Study Group on Autoimmune Diseases (GEAS) of the Spanish Society of Internal Medicine created the national registry of patients with amyloidosis (RAMYD). The classification of amyloidosis was based on the chemical characterization of the precursor protein. The 4 main types of systemic amyloidosis are AL, AA, ATTR, and Aβ2M type/others. Patients with localized deposition of amyloid were excluded.
Results A total of 570 patients with amyloidosis were included, 311 men, 259 women (mean age at dx: 64 years, range 19-93). Associated diseases were: hematological diseases 19%, inflammatory rheumatic diseases 10%, systemic autoimmune diseases 8%, non-hematologic neoplasms 6% and chronic infections 6%. We were able to classify amyloidosis in 71% patients: 36% AA amyloidosis, 135% AL, 20% ATTR and 9% others. Vital status was obteined in 478 patients, of which 298 (62%) died. Patients who died had an older mean age at diagnosis (67.29 vs 57.58 years in survivals, p<0.001). A higher mortality rate was observed in patients with associated hematological diseases (23% vs 16%, p=0.043), those with chronic infections (3% vs 8%, p=0.038), and in patients presenting with renal (38% vs 20%, p<0.001), cardiac (29% vs 15%, p=0.002) and pulmonary (16% vs 10%, p=0.048) involvements, while those presenting with peripheral neuropathy (13% vs 29%, p<0.001) and skin involvement (1% vs 7%, p=0.001) showed a low rate of mortality. Global mortality rate was 78% in AL amyloidosis, 66% in AA amyloidosis, 62% in patients who failed to amyloidosis classification, 47% in ATTR amyloidosis and 39% in patients with other types of amyloidosis.
Conclusions The rate of mortality of patients diagnosed with systemic amyloidosis exceeds 60% of cases, with AL amyloidosis having the highest mortality rate. The main baseline prognostic factors associated with death were older age, hematological diseases, and involvement of kidneys, lungs, heart. Our results, which demonstrated a global mortality rate of two-thirds of cases, serve to emphasize that the optimal management for systemic amyloidosis remains to be defined.
Disclosure of Interest : None declared