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Garelli, Silvia (2016) La sopravvivenza dei pazienti affetti da morbo di Addison in Italia. [Tesi di dottorato]

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Abstract (inglese)

Introduction: Survival of patients affected by Addison’s Disease (AD) is controversial, because only few studies have been published so far. We decided to evaluate survival of an Italian group of patients, affected by AD of various causes.

Materials and Methods: We examinated 1315 patients (58.6% females and 41.4% males) affected by AD, followed-by Endocrine Units all over Italy: the sum of follow-up years was 16983. 70.5% of patients has autoimmune AD (AAD), 113 with APS-1 and 814 with con AAD not APS-1 correlated (AAD not APS-1); 29.5% has non autoimmune AD, 98 of which with post-tuberculosis AD (TBC-AD), 50 with AD due to malignancy (Tu-AD), 97 with congenital AD (Ge-AD) and 143 with AD due to other causes (Ac-AD). We analyzed mean age of diagnosis, years of follow-up and mean age at the end of follow-up, survival was evaluated with Kaplan-Meier analysis and Cox regression. Results were correlated with Italian population data, correlated for sex and age (ISTAT register). We also performed a similar analysis considering only those patients who terminated the follow-up (1006 subjects).

Results: In APS-1 patients, mean age of diagnosis is 16.1±13.8 years, with 15 deaths in 113 subjects (13.3%) at a mean age of 32.5±18.4 years and a mean estimated survival (MES) of 40.4±5.8 years; in MAA non APS-1, mean age of diagnosis is 35.7±14.4 years, with 18 deaths in 814 subjects (2.2%) at 77.9±13.3 years of age and a MES of 56.1±3.8 years; in TBC-AD, mean age of diagnosis is 50.1±15.7 years, with 11 deaths in 98 subjects (11.2%) at a mean age of 81.1±7.4 years and MES of 42.5±4.1 years; in Tu-AD, mean age of diagnosis is 57.9±15.5 years, with 12 deaths in 50 subjects (24%) at 65.6±14.4 years of age and a MES of 11.3±5.8 years; in Ge-AD, mean age of diagnosis is 7.3±10 years, with 4 deaths in 97 subjects (4.1%) at a mean age of 16.7±4.3 years and a MES of 41.1±1.8 years; in Ac-AD, mean age of diagnosis is 43±19.5 years, with 5 deaths in 143 subjects (3.5%) at a mean age of 55.2±40 years and a MES of 44.4±1.8 years. Comparing mean estimated survival of each subgroup with Italian matched population, only APS-1 and Tu-AD appear lower. Comparing subgroups head to head, Tu-AD patients’ survival is lower than all other groups (p<0.001), while survival of APS-1 and TBC-AD subgroups is lower than AAD non-APS-1 subgroups (p<0.001 and p=0.01, respectively); all other comparisons are not statistically different. Cox regression analysis, referring to AAD non-APS-1 subgroup, demonstrate an higher risk of mortality in APS-1 (HR 4.38, CI 2.18-8.8; p<0.001), TBC-AD (HR 2.99, CI 1.39-6.44; p=0.005) and Tu-AD (HR 47.88, CI 21.07-108.81; p<0.001). Survival analysis performed only on those patients who completed the follow-up results similar to the previous analysis.

Conclusions: AD patients survival depends on ethiology of AD: in APS-1 and Tu-AD patients survival is lower than other subgroups and to the matched Italian population. In all other groups (AAD non-APS-1, TBC-AD, congenital AD and Ac-AD), survival is not different from matched Italian population.

Abstract (italiano)

Introduzione: La sopravvivenza dei pazienti affetti da Morbo di Addison (MA) è oggetto di dibattito in Letteratura e pochi studi hanno indagato il problema, fino ad oggi.
Abbiamo quindi deciso di valutare la sopravvivenza dei pazienti italiani con MA di differente eziologia.

Materiali e Metodi: Abbiamo esaminato 1315 pazienti (58.6% femmine e 41.4% maschi) affetti da MA, seguiti ambulatorialmente presso le UO di Endocrinologia di vari Centri italiani, per un totale di 16983 anni di follow-up. Il 70.5% dei pazienti presenta un MA autoimmune (MAA), di cui 113 con SPA-1 (sottogruppo SPA-1) e 814 con MAA non SPA-1 correlato (MAA non SPA-1); il 29.5% è di natura non autoimmune, di cui 98 pazienti con MA post-tubercolare (MA-TBC), 50 con MA secondario a neoplasia (MA-Tu), 97 con MA congenito (MA-Ge) e 143 da altre cause (MA-Ac). Abbiamo analizzato età media alla diagnosi, durata del follow-up ed età media al termine del follow-up; la sopravvivenza è stata valutata applicando il metodo di Kaplan-Meier e la regressione di Cox. I risultati sono stati comparati con i dati della popolazione italiana (fonti ISTAT), correlati per età e sesso. Abbiamo eseguito anche un’analisi simile considerando solo i pazienti che hanno completato il follow-up (1006 soggetti).

Risultati: L’età media di comparsa del MA per i pazienti SPA-1 è di 16.1±13.8 anni, con 15 decessi su 113 pazienti (13.3%) ad un’età media di 32.5±18.4 anni e una sopravvivenza stimata media (SSM) di 40.4±5.8 anni dopo la diagnosi; per i soggetti con MAA non-SPA-1 l’età di esordio è di 35.7±14.4 anni, con 18 decessi su 814 pazienti (2.2%) ad 77.9±13.3 anni e una SSM di 56.1±3.8 anni; per il gruppo MA-TBC l’età di comparsa del MA è di 50.1±15.7 anni, con 11 decessi su 98 casi (11.2%) a 81.1±7.4 anni, con una SSM di 42.5±4.1 anni; per il gruppo MA-Tu l’età del MA è di 57.9±15.5 anni, con 12 decessi su 50 casi (24%) a 65.6±14.4 anni e SSM di 11.3±5.8 anni; per il gruppo MA-Ge l’età di esordio del MA è di 7.3±10, i decessi 4 su 97 soggetti (4.1%) a 16.7±4.3 anni con SSM di 41.1±1.8 anni; per il gruppo MA-Ac l’età di comparsa del MA è di 43±19.5, i decessi 5 su 143 soggetti (3.5%) a 55.2±40 anni con SSM di 44.4±1.8 anni. Comparando la curva della sopravvivenza di ciascun sottogruppo di pazienti con MA con la popolazione italiana di riferimento, solo le curve dei pazienti con SPA-1 e MA-Tu sono risultate inferiori. Confrontando fra loro i singoli sottogruppi di pazienti, la sopravvivenza dei pazienti con MA-Tu risulta inferiore rispetto a quella di tutti gli altri pazienti (p<0.001), mentre quella dei soggetti con SPA-1 e MA-TBC appare inferiore al gruppo MAA non SPA-1 (rispettivamente p<0.001 e p=0.01); tutte le altre comparazioni risultano non significative. L’analisi di regressione di Cox, tenendo come sottogruppo di riferimento il MAA non-SPA-1, dimostra che vi è un aumentato rischio di mortalità nei sottogruppi SPA-1 (HR 4.38, IC 2.18-8.8; p<0.001), MA-TBC (HR 2.99, IC 1.39-6.44; p=0.005) e MA-Tu (HR 47.88, IC 21.07-108.81; p<0.001). L’analisi di sopravvivenza eseguita solo sui pazienti che hanno terminato il follow-up è risultata sostanzialmente analoga a quella della totalità dei pazienti.

Conclusioni: La sopravvivenza dei pazienti con MA dipende strettamente dall’eziologia della malattia stessa: nei pazienti con SPA-1 e con MA neoplastico è infatti inferiore, rispetto sia agli altri gruppi che alla popolazione generale di riferimento. In tutti gli altri gruppi (autoimmuni non-SPA-1, MA post-tubercolare, MA congenito e MA da altre cause) la sopravvivenza è risultata invece comparabile a quella della popolazione italiana di pari età e sesso.

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Tipo di EPrint:Tesi di dottorato
Relatore:Betterle, Corrado
Dottorato (corsi e scuole):Ciclo 28 > Scuole 28 > SCIENZE MEDICHE, CLINICHE E SPERIMENTALI > METODOLOGIA CLINICA, SCIENZE ENDOCRINOLOGICHE, DIABETOLOGICHE E NEFROLOGICHE
Data di deposito della tesi:21 Aprile 2016
Anno di Pubblicazione:21 Aprile 2016
Parole chiave (italiano / inglese):Morbo di Addison; Insufficienza surrenalica primitiva; Sindromi poliendocrine autoimmuni; Addison's Disease; Primary adrenal insufficiency; Autoimmune Polyendocrine Syndromes
Settori scientifico-disciplinari MIUR:Area 06 - Scienze mediche > MED/13 Endocrinologia
Struttura di riferimento:Dipartimenti > Dipartimento di Scienze Cardiologiche, Toraciche e Vascolari
Codice ID:9624
Depositato il:07 Ott 2016 09:52
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