What is Active Acromegaly and Which Parameters do we Have?

S. J. C. M. M. Neggers; N. R. Biermasz; A. J. van der Lely


Clin Endocrinol. 2012;76(5):609-614. 

In This Article

Disease-specific Symptom Score and Quality of Life Questionnaires

Acromegaly Quality of Life Questionnaire (AcroQoL)

AcroQoL is a disease-specific quality of life questionnaire, comprising 22 questions. Each question has five possible answers scored 1–5, with a total maximum score of 110 and quoted as a percentage. The score of 110 reflects the best possible QoL. The 22 questions are divided into two main categories: physical and psychological function. The psychological dimension is subdivided into appearance and personal relationships.[35,36] The AcroQoL has a good internal consistency (Cronbach's > 0·8),[44] although there is, to some extent, a lack of normative data. The QoL scores of the AcroQoL from different countries are interchangeable, with cure around 80, and active disease 50. This suggests that there is a high homology between countries and patients even though they have a different background.

Patient-assessed Acromegaly Symptom Questionnaire (PASQ)

The PASQ is a disease-specific symptom questionnaire, which consists of six questions scoring 0–8 and the seventh question addressing overall health status, based on the other six questions, scoring 0–10.[25,37] The first six questions evaluate symptoms of headache, excessive sweating, joint pain, fatigue, soft tissue swelling, and numbness or tingling of the extremities. The maximum score of these six questions is 48 and indicates severe symptoms, with lower scores reflecting milder symptoms.

Quality of Life as Marker of Disease Activity. Despite biochemical control, patients may experience ongoing signs of GH excess, such as mild hypertension, relative glucose intolerance and arthralgia, soft tissue swelling and perspiration.[29,31] These symptoms can be present because of (ongoing) pathological GH secretion, while IGF1 and GH are within the normal range or irreversible changes. Thus, biochemical assessments fail to discriminate perfectly in cases of mild excess.[31] A very important issue is whether quality of life and patients' perceived health, as assessed by questionnaire, perform better than biochemical assessments and may be of additive value in the detection of disease activity.

In general, QoL in patients is influenced by several factors, which may limit the discriminatory value of QoL. These include, firstly, somatic factors: the disease itself, signs and symptoms related to the disease and unrelated co-morbidity. At present, there are no cut-off points for quality of life scores. In previous studies, QoL scores were significantly related to the presence of joint pain, anxiety/depression, but not to obstructive sleep apnoea and cardiomyopathy. Secondly, several psychological factors are known to influence quality of life. For example, anxiety and depression negatively influence quality of life, including physical scales. Also, coping behaviour, personality and understanding of the disease as can be measured by illness perceptions are relevant factors.[45,46]

In acromegaly, quality of life has been reported to be significantly reduced in active untreated disease and in controlled treated disease, even after long-term follow-up. Although QoL improves in biochemically cured patients, limitations remain, possibly due to irreversible damage or elevated GH action. QoL has been measured with disease-specific questionnaires, the AcroQoL, and several general health questionnaires, most frequently by assessing functional status with the SF-36, but also using HADS and MFI-20 and EQ-5D.[5,38,47–49] Comparative studies within the field of pituitary disease have suggested that acromegaly and Cushing's are the diseases with most impaired QoL, with acromegaly most frequently causing impairments in physical function and pain.[50]

To date, the number of longitudinal studies is limited. However, improvements of QoL are generally observed in patients with new treatments, although biochemical changes, GH and IGF1 concentrations, and QoL changes frequently dissociate.[38,42,48,51,52] QoL does relate to patients' perceived symptom scores.[49] Based on quality of life outcomes, there is not a clear preference for any one treatment modality. In Dutch[53] and Danish[27] cohorts of patients with acromegaly, the best QoL was reported in patients with surgical cure – without pituitary failure – and a progressive decline in QoL score occurred in irradiated patients.[47] Improved QoL was reported after adding pegvisomant to controlled patients during LA-SRIF analogue treatment.[30] Treatment of GHD in patients with acromegaly was also associated with improved QoL in one study, but not in another.[54,55]

Pain, especially joint pain, was an important negative factor for QoL. Patients with (clinical) acromegalic arthropathy had decreased QoL in comparison with patients without arthropathy.[56–58] By analogy, patients with hypertension, restless legs and a high symptom score also had impaired QoL.[40,49,57–60]

Patients with acromegaly are at risk for depression and other neuropsychiatric symptoms[61,62] characterized by reduced impulsivity and novelty seeking. Depressive and anxiety symptoms assessed by HADS were associated with more impaired QoL.[53] Currently, little is known about coping strategies and illness perceptions in relation to quality of life in acromegaly. These have been associated with QoL in other chronic diseases. In an exploratory study, coping strategies in acromegaly appeared to be suboptimal, and coping strategies were related to QoL.[46]

Quality of life is an important patient-based outcome measure of treatment[63] and is a component of the quality-adjusted life year (QALY) unit, which is used in cost-benefit assessments.[64] Several studies have reported that QoL changes with major changes in disease status. However, in stable disease and during minor changes in disease state within the (near) normal range, biochemical markers and QoL frequently dissociate. This is because of imperfections in the ability of biochemical markers to adequately detect disease status. When all available data are taken into consideration, QoL could add additional value to the current available biochemical markers.


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