Essential guide to...
Raynaud's phenomenon and fatigue
Concluding our series
on musculoskeletal conditions, consultant rheumatologist Dr Colin Tench provides a fresh perspective on these key features of connective tissue disease
Connective tissue diseases (CTDs) are a group of multisystem disorders characterised by abnormalities in blood vessels and connective tissues. Many of the diseases within this group – including systemic lupus erythematous (SLE), iodiopathic inflammatory myopathies and rheumatoid arthritis – have overlapping clinical features, such as joint pain, Raynaud's phenomenon and
fatigue, and share immunological abnormalities.
CTDs can have multiple effects on different organs and hence the clinical manifestations are diverse.
These diseases are rare, for example the prevalence of SLE in women in Britain has been estimated at one in 2,000. The rarity of these conditions and the fact that they often present with non-specific systemic features, including malaise, fatigue, fever and weight loss, can lead to diagnostic difficulties.
However, musculoskeletal involvement is often an important feature of the illness, and the diagnosis should be considered in any young female patient presenting with widespread joint pain, especially if associated with profound fatigue.
Raynaud's phenomenon is a common symptom in CTD and patients will often describe symptoms of cool peripheries on cold exposure, with associated colour change of the fingers and toes.
The classical triphasic response comprises initially white digits due to vasospasm of the digital arteries, followed by blue digits due to tissue ischemia and cyanosis. During the recovery phase the digits become red due to vasodilatation.
Approximately 5 per cent of the population suffers from primary Raynaud's phenomenon and hence most patients seen in primary care do not have a secondary underlying cause, such as SLE, systemic sclerosis or rheumatoid arthritis.
Suspicious features which may suggest a secondary cause include:
• first presentation over the age of 30
• asymmetrical involvement of the digits
• painful attacks
• skin ulceration.
Any of these features should prompt a more detailed history, searching for symptoms such as recurrent mouth ulcers, hair fall, photosensitive skin rashes, tightness or puffiness of the skin over the digits, and symptoms of dry eyes and dry mouth. If these suspicious features, or additional clinical symptoms, are present then further investigations are mandatory (see box below).
Serological abnormalities are very helpful in confirming a diagnosis of CTD and in the absence of any positive serology, it is difficult to make a diagnosis with any confidence.
More than 95 per cent of patients with SLE have a positive ANA or positive Ro antibodies and hence this is a useful screening test. Less than 2 per cent of patients with Raynaud's phenomenon and a negative ANA go on to develop a CTD and this group can be reassured that they will not develop SLE or systemic sclerosis.
Since 3-4 per cent of the healthy population have a positive ANA, a positive result in a patient with Raynaud's phenomenon alone has a low predictive value for the development of a CTD and these patients need careful follow-up for a few years.
All patients with Raynaud's phenomenon should be given general advice about avoiding cold exposure, wearing gloves, thick socks and hats and smoking cessation.
Pharmacological treatments include calcium-channel blockers, such as nifedipine, or angiotensin-receptor blockers, such as losartan. Hydroxychloroquine is very helpful for some of the milder symptoms of SLE and Sjogren's, such as joint pain, rashes and oral ulcers; but many patients with CTDs require treatment with systemic corticosteroids and additional immunosuppressive drugs.
What really matters to patients?
Most patients with CTDs report significant fatigue. This is often the most disabling symptom experienced and can be difficult to assess and to treat successfully. In patients with SLE, fatigue is associated with a number of factors, including:
• poor sleep quality
• reduced quality of life
• disease activity.
SLE patients are physiologically deconditioned compared to healthy individuals and in a randomised controlled trial of exercise, nearly half the patients felt better or
much better after three months of aerobic exercise.
To treat lupus fatigue successfully I recommend optimising treatment of active disease, excluding contributing factors such as hypothyroidism and anaemia, and encouraging regular aerobic exercise. Any exercise is better than none, but to have significant benefits, patients need to exercise for a minimum of 30 minutes, three times a week.
For most patients fast walking or jogging is the most convenient way of achieving this, and should be performed at an intensity that makes them a little breathless and sweaty.
For those that have never exercised before, a graded exercise programme is helpful, starting off at five minutes a day and
increasing by a minute a week. Too much too soon inevitably leads to deteriorating fatigue, with worsening joint and muscle pain and rapid cessation of the exercise programme.
Sleep hygiene is also important in this group of patients and simple advice such as keeping the bedroom cooler than the rest of the house, using the bedroom for sleep only and avoiding eating a large meal late in the evening can all be effective.
Keep CTDs in mind
CTDs are often difficult to diagnose and should be considered in any patient
presenting with symptoms of widespread joint pain, Raynaud's phenomenon and/or fatigue.
The ANA is a useful screening test to
confirm the diagnosis and, in addition, helps to identify patients who require
further investigation and treatment.
Colin Tench is a consultant rheumatologist at
St Mary's Hospital, London
Competing interests None declared
• Full blood count – to look for anaemia and leucopenia/lymphopenia
• U and E
• Inflammatory markers – ESR often raised with normal CRP in patients with SLE
• Creatinine kinase
• Antinuclear antibody