Use of Pegvisomant in acromegaly. An Italian Society of Endocrinology guideline

Giustina, Andrea; Ambrosio, Maria Rosaria; Peccoz, Paolo Beck; Bogazzi, Fausto; Cannavò, Salvatore; Marinis, Laura De; Menis, Ernesto De; Grottoli, Silvia; Pivonello, Rosario

doi:10.1007/s40618-014-0146-x

Cited by 49 publications

(45 citation statements)

References 115 publications

(168 reference statements)

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“…An adjustment of this time schedule could be discretional in case of combination treatments with SRLs. Discontinuation of treatment is mandatory for cases of severe liver toxicity (acute hepatitis, one case in Italian experience, and transaminase increase [5 times upper limit of normal, three cases in this same report) [20,21]. Less clear is the recommended behavior in the more frequent cases of mild transaminases increase without liver failure: in the case pegvisomant is continued intensive (weekly) monitoring of liver function is mandatory [20].…”

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confidence: 87%

“…SRLs elevation of liver enzymes, particularly of severe degree, was a quite infrequent occurrence. Based on this observation and available literature, only the presence of liver dysfunction contraindicates pegvisomant use [20]. However, not only a monthly monitoring of serum transaminases is necessary in the first 6 months of treatment, but also afterward a careful evaluation of liver function (quarterly for next 6 months, and then semi-annually) is required [34].…”

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confidence: 98%

“…Therefore, the advent of pegvisomant, which acts at the tissue level blocking the GH receptor and GH action, in the clinical scenario more than a decade ago has been surrounded by many hopes and expectations for a potential global control of acromegaly [19]. However, despite accumulating clinical experience and ad hoc designed surveillance studies specific guidelines for pegvisomant in acromegaly are limited [20], and its use appears still not always to be optimal. Why?…”

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confidence: 99%

“…SRL treatment [21]. Based on this experience and on available data pegvisomant could be considered as ''primary'' medical therapy only when surgery and radiation can not be performed and SRL may not be effective as in patients with acromegaly and McCune Albright syndrome [31] or in case of infrequent but possible clear-cut intolerance to SRLs [20]. Otherwise, optimal use of pegvisomant concerning tumor safety should take into account the history of the adenoma (aggressiveness/invasiveness), previous tumor response to treatments including SRLs [32,33] and tumor volume at start of treatment [34].…”

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confidence: 99%

“…Otherwise, optimal use of pegvisomant concerning tumor safety should take into account the history of the adenoma (aggressiveness/invasiveness), previous tumor response to treatments including SRLs [32,33] and tumor volume at start of treatment [34]. Moreover, all patients treated with pegvisomant should undergo regular sellar MRI to screen for potential tumor growth and likely a more intensive MRI follow-up protocol should be followed in non-irradiated patients [20]. Another safety issue that so far limited the use of pegvisomant is liver toxicity.…”

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confidence: 99%

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Optimal use of pegvisomant in acromegaly: are we getting there?

Giustina

2014

Endocrine

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confidence: 87%

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confidence: 98%

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confidence: 99%

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confidence: 99%

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confidence: 99%

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Optimal use of pegvisomant in acromegaly: are we getting there?

Giustina

2014

Endocrine

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Complications of acromegaly: cardiovascular, respiratory and metabolic comorbidities

et al. 2017

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Acromegaly is associated with an enhanced mortality, with cardiovascular and respiratory complications representing not only the most frequent comorbidities but also two of the main causes of deaths, whereas a minor role is played by metabolic complications, and particularly diabetes mellitus. The most prevalent cardiovascular complications of acromegaly include a cardiomyopathy, characterized by cardiac hypertrophy and diastolic and systolic dysfunction together with arterial hypertension, cardiac rhythm disorders and valve diseases, as well as vascular endothelial dysfunction. Biochemical control of acromegaly significantly improves cardiovascular disease, albeit completely recovering to normal mainly in young patients with short disease duration. Respiratory complications, represented mainly by sleep-breathing disorders, particularly sleep apnea, and respiratory insufficiency, frequently occur at the early stage of the disease and, although their severity decreases with disease control, this improvement does not often change the indication for a specific therapy directed to improve respiratory function. Metabolic complications, including glucose and lipid disorders, are variably reported in acromegaly. Treatments of acromegaly may influence glucose metabolism, and the presence of diabetes mellitus in acromegaly may affect the choice of treatments, so that glucose homeostasis is worth being monitored during the entire course of the disease. Early diagnosis and prompt treatment of acromegaly, aimed at obtaining a strict control of hormone excess, are the best strategy to limit the development or reverse the complications and prevent the premature mortality.

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