Acromegaly: clinical features at diagnosis.
BACKGROUND
Acromegaly is a rare and underdiagnosed disorder caused, in more than 95% of cases, by a growth hormone (GH)-secreting pituitary adenoma. The GH hypersecretion leads to overproduction of insulin-like growth factor 1 (IGF-1) which results in a multisystem disease characterized by somatic overgrowth, multiple comorbidities, physical disfigurement, and increased mortality.


OBJECTIVE
This article aims to review the clinical features of acromegaly at diagnosis.


CONCLUSIONS
Acromegaly affects both males and females equally and the average age at diagnosis ranges from 40 to 50 years (up to 5% of cases < the age 20). Due to insidious onset and slow progression, acromegaly is often diagnosed five to more than ten years after its onset. The typical coarsening of facial features include furrowing of fronthead, pronounced brow protrusion, enlargement of the nose and the ears, thickening of the lips, skin wrinkles and nasolabial folds, as well as mandibular prognathism that leads to dental malocclusion and increased interdental spacing. Excessive growth of hands and feet (predominantly due to soft tissue swelling) is present in the vast majority of acromegalic patients. Gigantism accounts for up to 5% of cases and occurs when the excess of GH becomes manifest in the young, before the epiphyseal fusion. The disease also has rheumatologic, cardiovascular, respiratory, neoplastic, neurological, and metabolic manifestations which negatively impact its prognosis and patients quality of life. Less than 15% of acromegalic patients actively seek medical attention for change in appearance or enlargement of the extremities. The presentation of acromegaly is more often related to its systemic comorbidities or to local tumor effects.
Division of Endocrinology, Hospital das Clínicas, Federal University of Pernambuco, Rua Heitor Maia Filho, 100/502, Madalena, Recife, CEP 50.720-525, Brazil. lvilarf@gmail.com.
Lucio
http://orcid.org/0000-0003-4815-6963
L
Vilar
Endocrine Research Center of Pernambuco, Recife, Brazil.
Clarice Freitas
CF
Vilar
Division of Endocrinology, Hospital das Clínicas, Federal University of Pernambuco, Rua Heitor Maia Filho, 100/502, Madalena, Recife, CEP 50.720-525, Brazil.
Ruy
R
Lyra
Endocrine Research Center of Pernambuco, Recife, Brazil.
Raissa
R
Lyra
Division of Endocrinology, Brasilia University Hospital, Brasilia, Brazil.
Luciana A
LA
Naves
D000172:Acromegaly / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D005877:Gigantism / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D008297:Male
Acromegaly; Clinical features; Gigantism; Somatotropinoma