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THU0399 Systemic Amyloidosis as A Multi-Organ, Life-Threatening Disease: Predictive Baseline Factors Associated to Mortality in 570 Patients (Ramyd-Geas-Semi Registry)
  1. P. Brito Zeron1,
  2. L.E. Cajamarca2,
  3. R. Perez-Alvarez3,
  4. D. Real de Asua4,
  5. S. Benito Conejero5,
  6. F. Beamud5,
  7. F. Martinez-Valle6,
  8. X. Bosch7,
  9. I. Villaverde8,
  10. E. Fonseca9,
  11. L. Acevedo10,
  12. L. Gonzalez Vazquez11,
  13. G. de la Red12,
  14. C. Santiago13,
  15. E. Gonzalez Vazquez14,
  16. L. Inglada15,
  17. A. Robles Marhuenda16,
  18. A. Castro Salomo17,
  19. R. Jordana2,
  20. J. Fernandez Martin3,
  21. M. Perez de Lis3,
  22. S. Retamozo1,
  23. H. Gheitasi18,
  24. A. Lopez Soto7,
  25. M. Ramos-Casals1
  26. on behalf of RAMYD-GEAS-SEMI
  1. 1Dept. Autoimmune Diseases, Hospital Clinic, Barcelona
  2. 2Dept. Internal Medicine, Hospital Parc Tauli, Sabadell
  3. 3Dept. Internal Medicine, Hospital do Meixoeiro, Vigo
  4. 4Dept. Internal Medicine, Hospital La Princesa, Madrid
  5. 5Dept. Internal Medicine, Hospital Juan Ramon Jimenez, Huelva
  6. 6Dept. Internal Medicine, Hospital Vall d'Hebron
  7. 7Dept. Internal Medicine, Hospital Clinic, Barcelona
  8. 8Dept. Internal Medicine, Hospital Xeral, Vigo
  9. 9Dept. Internal Medicine, Hospital de Cabuenes, Gijon
  10. 10Dept. Internal Medicine, Hospital Gregorio Maranon, Madrid
  11. 11Dept. Internal Medicine, Hospital Povisa, Vigo
  12. 12Dept. Internal Medicine, Hospital Espiritu Santo, Barcelona
  13. 13Dept. Internal Medicine, Hospital Virgen de las Nieves, Granada
  14. 14Dept. Internal Medicine, Hospital de Ourense, Ourense
  15. 15Dept. Internal Medicine, Hospital Rio Hortega, Valladolid
  16. 16Dept. Internal Medicine, Hospital La Paz, Madrid
  17. 17Dept. Internal Medicine, Hospital Sant Joan, Reus
  18. 18Dept. Systemic Autoimmune Diseases, Hospital Clinic, Barcelona, Spain

Abstract

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

DOI 10.1136/annrheumdis-2014-eular.5896

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