Background: Clinical features of acromegaly develop insidiously and the diagnosis is often delayed. However, diagnostic delay (DD) in acromegaly has not been systematically investigated. Our primary aim was to investigate DD in a nationwide cohort of patients with acromegaly. Secondary aim was to study the impact of DD on morbidity and mortality.
Methods: Adult patients diagnosed with acromegaly between 2001 and 2013 were identified in the Swedish National Patient Registry. Forty-four diagnostic codes for pre-defined comorbidities associated with acromegaly, registered from 1987 to 2013, were recorded. The DD was calculated as the time between the first registered comorbidity and the diagnosis of acromegaly.
Results: A total of 603 patients with acromegaly (mean age at diagnosis: 51.8±15.3 years) were included (280 men, 323 women). The mean DD was 5.5±6.2 years (median 3.3; range 0.09.4), with a tendency towards a longer DD in women than in men [median 4.1 (0.110.6) vs 2.7 (0.08.0); P=0.055]. DD was 1<5 years in 23% patients; 5<10 years in 17%; and ≥10 years in 24%. Patients without comorbidities and those with comorbidities identified within a year before the acromegaly diagnosis, or later, were analysed as a single group, named patients without DD (36%). Of 603 patients, 579 (96%) had comorbidities at any time during the study period. The median number of comorbidities was 4.0 (2.05.0), and was significantly higher in patients with longer DD (P<0.0001). The most frequent comorbidities were neoplasms, excluding pituitary tumours (61%), cardiovascular (57%), and musculoskeletal (51%) diseases, with a higher frequency in patients with longer DD (P<0.0001). The longest DD was found for neurological-psychiatric symptoms (4.6±6.3 years) and the shortest for local effects; e.g. symptoms due to tumor compression of surrounding structures (1.7±3.4 years). DD for cardiovascular, musculoskeletal disorders, and neoplasms was 2.6±4.5, 3.4±4.9, and 3.7±5.4 years, respectively. The observed number of deaths was 61, and expected number 42.2, resulting in a standardized mortality ratio of 1.45, 95% CI 1.111.86. Excess mortality was only found in patients with DD ≥10 years (1.76, 95% CI 1.122.65) whereas mortality was similar to the general population in the groups with shorter DD [1<5 years, 1.32 (95% CI 0.682.31); 5<10 years 1.54 (95% CI 0.742.83)] as well as in patients without DD (1.18; 95% CI 0.681.92).
Conclusions: Our findings suggest that diagnosis of acromegaly is delayed in a substantial number of patients. The delay of the acromegaly diagnosis is associated with excess morbidity and mortality.
18 - 21 May 2019
European Society of Endocrinology