TREATMENT OF AA AMYLOIDOSIS IN A SINGLE CENTER IN ARGENTINA

POSADAS MARTINEZ, MARIA LOURDES; ET AL

Congreso; XVII INTERNATIONAL SYMPOSIUM ON AMYLOIDOSIS (ISA); 2020

Posadas-Martinez, ML. Research Unit of Internal Medicina, Italian Hospital of Buenos Airesxxxxx}Nucifora, E. Hematology, Italian Hospital of Buenos AiresKey word: AA amyloidosis, treatment, prognosisBackground: Therapeutics of AA are directed against the underlying cause and in the idiopathic ones to the blockade of the inflammation. Organic involvement, evolution time, comorbidities, treatment availability and underlying disease are likely to have an impact on treatment options. Aim: Describe treatments in AA amyloidosis. Estimate global survival Methods: Retrospective cohort of patients with amyloidosis AA in the Institutional Registry of Amyloidosis (NCT01347047) at Hospital Italiano de Buenos Aires from 2010-2019. All patients were followed to death for all causes. Survival rates are expressed as the percentage surviving calculated using Kaplan Meier method.Results: During the period, 160 patients with amyloidosis were included, 14% (23) had diagnosis of AA. 48% were female (11), with a median age of 52 years (RII 49-64). The diseases that caused the deposition of SAA were, in order of frequency, idiopathic, autoimmune and infections. The kidney was the most frequently affected organ (86%), followed by the heart (43%) and the digestive tract (32%). During follow-up, 4 patients died, the overall mortality rate was 17% (n = 4, CI 6-40%). The cause of death in three of the 4 patients was related to amyloidosis, one patient died of heart failure, one patient died of end-stage renal failure, a patient of refractory septic shock, and one patient died of accidental traumatic cause, not related to underlying disease. Thirteen patients (56%) received treatment, and one died of sepsis. At the beginning of treatment 60% had a PS of 2 and a Charlson score of 4. Biologic agents were administered to 54% of patients with AA (7). Tocilizumab (TCZ) was the biologic agent most frequently indicated (7) as first or second line of therapy, two patients received etanercept and canakinumab as first and second line previously to TCZ, and two patients received cyclophosphamide and mycophenolate. All patients showed good responses to TCZ. Antimicrobial agents were prescribed in 6 patients. All treated patients but one, stabilized their condition, improved inflammation biomarkers and achieved a normal nutritional status. Conclusión: Half of the patients received treatment, directed to the underlying disease or in idiopathic cases directed to inflammation. Treatment is critic in stabilizing the disease progression Overall prognosis and survival are similar to other series.