Diagnosis and management of chronic fatigue syndrome or myalgic encephalomyelitis (or encephalopathy): summary of NICE guidance

Why read this summary?

Data from other countries indicate that chronic fatigue syndrome (also known as myalgic encephalomyelitis or myalgic encephalopathy) (CFS/ME) is relatively common (affecting at least 0.2-0.4% of the population), although good epidemiological data for the United Kingdom are lacking. Many different potential aetiologies for CFS/ME have been investigated, including neurological, endocrine, immunological, genetic, psychiatric, and infectious, but the aetiology cannot yet be fully explained. CFS/ME can cause prolonged illness and disability and substantially affect patients and their families. Although most patients have mild or moderate symptoms, some have severe CFS/ME and are housebound or even unable to move from their bed. Uncertainties about diagnosis and management may exacerbate the impact of symptoms, and patients often encounter delays in diagnosis and difficulty accessing information, support, and potentially helpful therapies.¹ This article summarises the most recent guidance from the National Institute for Health and Clinical Excellence (NICE) on diagnosing and managing this condition.²

Recommendations

NICE recommendations are based on systematic reviews of best available evidence. When minimal evidence is available (as with CFS/ME), a range of consensus techniques is used to develop recommendations. In this summary, recommendations derived primarily from consensus techniques are indicated with an asterisk (*).

General principles

• • Acknowledge the reality and impact of the condition and symptoms.*

• • Provide information on possible causes of the condition, its course, and management strategies (including benefits and risks of therapies, return to work or education), tailored to individual circumstances.*

• • Offer information about self help and support groups for patients, families, and carers.*

• • Be aware that all patients have the right to refuse or withdraw from any component of their care plan without this affecting other aspects of care or future choices about care.*

• • Aim to establish a supportive, collaborative relationship with patients and their carers and families (especially those of children, young people, and people with severe CFS/ME).*

Presentation

• • Consider the possibility of CFS/ME if a person has fatigue with all of the following features:

  • - New or specific onset (that is, it is not lifelong)

  • - Persistent and/or recurrent

  • - Unexplained by other conditions

  • - Resulted in a substantial reduction in activity level

  • - Characterised by post-exertional malaise and/or fatigue (typically delayed, for example by at least 24 hours, with slow recovery over several days)

• together with one or more of the following symptoms:

  • - Difficulty with sleeping, such as insomnia, hypersomnia, unrefreshing sleep, a disturbed sleep-wake cycle

  • - Muscle and/or joint pain that is multisite and without evidence of inflammation

  • - Headaches

  • - Cognitive dysfunction (such as difficulty thinking; inability to concentrate; impairment of short term memory; and difficulties with word finding, planning or organising thoughts, and processing information)

  • - General malaise or influenza-like symptoms

  • - Sore throat

  • - Painful lymph nodes without pathological enlargement

  • - Dizziness and/or nausea

  • - Palpitations in the absence of identified cardiac pathology

  • - Worsening of symptoms on physical or mental exertion.

• • “Red flag” symptoms that might indicate another serious illness include significant weight loss; clinically significant lymphadenopathy; localising or focal neurological signs; features of inflammatory arthritis, connective tissue disease, or cardiorespiratory disease; and sleep apnoea.*

• • Do not delay advice on symptom management, which should be provided as in usual clinical practice even before establishing a diagnosis of CFS/ME.*

Diagnosis

• • CFS/ME is diagnosed clinically as insufficient evidence exists for routinely using diagnostic tests, such as the head-up tilt test, auditory brainstem responses, and electrodermal conductivity.^{3 4 5}

• • Usual testing to exclude other diagnoses should include:

  • - Urine analysis for protein, blood, and glucose

  • - Full blood count and erythrocyte sedimentation rate or plasma viscosity

  • - C reactive protein

  • - Serum urea, creatinine, electrolytes, and calcium

  • - Random blood glucose

  • - Creatine kinase

  • - Liver function

  • - Thyroid function

  • - Screening blood tests for gluten sensitivity

  • - Serum ferritin (children and young people only).*

• • Do not do serological testing for viral or bacterial infections unless the history indicates such an infection.

• • CFS/ME should be diagnosed in an adult after symptoms have persisted for four months and after exclusion of other likely causes of the symptoms. In a child, the condition should be diagnosed (or the diagnosis confirmed) by a paediatrician after symptoms have persisted for three months and after exclusion of other likely causes.*

General management and referral

• • After diagnosis, manage symptoms as in usual clinical practice, which may include drugs and dietary changes. Other interventions that may improve function and quality of life include sleep management (for example, identifying common changes in sleep patterns seen in CFS/ME), appropriate use of rest periods, relaxation, pacing,* and further dietary changes,* as needed.

• • During a setback (or relapse) with increased symptoms, advise patients to maintain physical activity if possible. If not possible, aim for a gradual return to previous exercise and functional routines.*

• • Pharmacological treatments (such as antidepressants, steroids, thyroxine in euthyroid patients) are not recommended as evidence for their overall benefit is equivocal.*

• • Advise patients to maintain a well balanced diet. Dietary supplements (including vitamins and minerals) and complementary therapies are not recommended as there is insufficient evidence of benefit. However, patients may wish to try these therapies for symptom control as part of a self management strategy.*

• • Advise on fitness for work and education and recommend flexible adjustments or adaptations to work or studies for return to these when the patient is ready and fit enough.* With the patient's consent, liaise with employers, education providers, and support services such as occupational health services, disability services through Jobcentre Plus (www.jobcentreplus.gov.uk/), schools, home education services, and local education authorities, and/or disability advisers in universities and colleges.*

• • Consider referral to a specialist on the basis of the person's needs and symptoms: offer referral within six months of presentation to those with mild symptoms, within three to four months to those with moderate symptoms, and immediately to those with severe symptoms.*

Specialist care

• • Collaborate with the patient on an individualised programme, aiming to sustain or gradually extend the patient's physical, emotional, and cognitive capacity, and to manage the physical and emotional impact of symptoms on the individual and his or her carers.

• • Offer cognitive behaviour therapy and/or graded exercise therapy to people with mild or moderate CFS/ME and provide these therapies to those who choose them, as these interventions show clearest evidence of benefit. These interventions should only be delivered by appropriately trained professionals with experience in CFS/ME, and with appropriate clinical supervision.

• • Cognitive behaviour therapy should follow the usual principles and include asking the patient to self monitor activity, rest, thoughts, feelings, and behaviours; discussing with the patient his or her adjustment to the diagnosis; and encouraging acceptance of current functional limitations.

• • Graded exercise therapy should include the establishment of a baseline followed by planned increases in duration of low intensity physical activity, followed by gradual increases in intensity leading to aerobic exercise (which increases the pulse rate). It should be based on current level of activities and daily routines and on the patient's own goals. Both patient and healthcare professional should recognise that it may take weeks to years to achieve these goals.

• • Diagnosis, investigation, management, and monitoring for people with severe CFS/ME should be supervised or supported by a specialist in the condition. This may include providing domiciliary services or using methods such as telephone or email as appropriate.

Overcoming barriers

• • A lack of services (and resources)¹ and misperceptions about the nature of CFS/ME¹ may hinder implementation of these recommendations. Recognising the condition and the impact it can have on the lives of patients and their families is therefore a necessary preliminary to improving care.

• • People with severe CFS/ME who are housebound or need prolonged bed rest require specialised care, but evidence is lacking about which treatments are most effective to reduce their symptoms.

• • Little research exists on CFS/ME generally, and specifically on its causes and diagnosis.

• • The guideline development group recognised the need for wider research on aetiology and pathogenesis, as a basis for developing new treatments.

• • For all people with CFS/ME the guidelines recommend an integrated, multidisciplinary approach, incorporating health and social care, with support for education and work when and if the patient is ready. This comprehensive approach can be difficult to achieve unless one named professional has responsibility for coordinating the patient's care.

Further information on the guidance

Methods

In developing the guideline, the guideline development group aimed to draft recommendations to promote access to appropriate care and support and to encourage a collaborative relationship between patients, carers, and professionals.

Formal consensus methods were used to develop this guideline. The guideline development group used ratings of appropriateness to agree on evidence statements and associated recommendations.⁶ This was supplemented by use of the same methods with a sample of people (219/399; response rate of 55%) outside the guideline development group, including health professionals (doctors, nurses, therapists) involved in the care of people with CFS/ME, patients with CFS/ME, and carers. The ratings of the wider group were used to inform the guideline group of the range of opinions among health professionals, patients, and carers and to highlight draft statements that were ambiguous or unclear.

NICE has produced four different versions of the guideline: a full version; a quick reference guide; a version known as the “NICE guideline” that summarises the recommendations; and a version for patients and the public. All these versions are available from the NICE website (www.nice.org.uk/CG053). Future updates of the guidelines will be produced as part of the NICE guideline development programme.⁷

The National Collaborating Centre for Primary Care thanks Professor Rosalind Raine and her team for advice on the use of the consensus method, and the Centre for Reviews and Dissemination at York for their review of the evidence.