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Acromegaly: clinical features at diagnosis

Abstract

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.

Discussion/Conclusion

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.

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Correspondence to Lucio Vilar.

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For this review article, the authors have no conflicts of interest and they do not receive any kind of funding. This review article was mostly based on previous articles published in indexed journals of the english literature on acromegaly and pituitary tumors, including some where the authors were coworkers or the first authors.

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Vilar, L., Vilar, C.F., Lyra, R. et al. Acromegaly: clinical features at diagnosis. Pituitary 20, 22–32 (2017). https://doi.org/10.1007/s11102-016-0772-8

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Keywords

  • Acromegaly
  • Gigantism
  • Somatotropinoma
  • Clinical features