Clinical features
Signs and Symptoms
SLE can present in a wide variety of ways with non-specific symptoms and can therefore be difficult to diagnose. The final diagnosis should be made by a specialist.
- Skin (cutaneous) manifestations
- Musculo-skeletal symptoms
- Fatigue
- Kidney disorder
- Cardiac disease
- Pulmonary disease
- Disorder of the blood cells
- Neurological involvement
Skin (cutaneous) manifestations (81%)
Four out of five patients have some form of skin manifestation such as:
- Red, butterfly- shaped rash across the cheeks and nose, often appearing after exposure to sunlight, occurring mainly in Caucasians. Occurrence – 20% to 50%.
- Sensitivity to sunlight (“photosensitivity” and photoreactivity) - 40%, mainly Caucasians.
- Skin rash on other parts of the body often in sun-exposed areas of the body such as the “V” of the neck and forearms.
- Mouth ulcers.
Butterfly- shaped rash
This is a classical feature, and is a raised, painful and itchy rash which extends across the nose and cheeks, but not across the nasolabial folds (area running from the outer corners of the nose to the outer corners of the mouth. It has to be differentiated from other common facial rashes, such as rosacea.
Other manifestations are hair loss (alopecia) which may be temporary, or be serious and extensive and permanent with scarring, Raynaud’s phenomenon (which is the constriction of small arteries in the fingers and toes in response to cold or strong emotion), and Livedo Reticularis (a net-like blush of blood vessels especially noticeable on the legs) may occur.
Photosensitivity and Skin Rash
A rash may develop after exposure to sun, in the face, hands and neck but may develop in other parts of the body as well.
The rash may last several days or weeks and can precipitate a flare of other musculo-skeletal or systemic symptoms.
Mouth Ulcers
Non- healing, recurrent crops of mouth ulcers may occur repeatedly.
Musculo-skeletal symptoms (around 90% affected)
Joint pains (arthralgia) are quite common and indeed may be the main symptom. The small joints of the hands and feet may be affected. The arthralgia is episodic and flits about; the joints are not damaged. About 50% get early morning stiffness.
It can mimic rheumatoid arthritis but swelling of the joints is much less noticeable. However, tenosynovitis may occur, and this can lead to damage and deformity, especially in the hands, especially the thumbs (Jaccoud’s Arthropathy). It is a rare complication.
Fatigue (very common)
This symptom varies considerably between individuals, from a mild limitation of normal functioning, to complete overwhelming fatigue which can affect all aspects of self- care.
It can be an indicator of disease activity and may be the first indication that a person’s disease is flaring.
There is a spectrum of fatigue, with proper treatment it may be alleviated, but it never really goes away.
Kidney disorder (around 33%)
The kidneys may be affected with the development of glomerulonephritis. However the onset of kidney involvement can be insidious, therefore it is important to regularly test the urine for blood and protein.
The usual manifestations are blood in the urine (haematuria), protein in the urine (proteinuria) and casts which show up under the microscope. Casts are either collections of abnormal cells (granular casts) which indicate active renal disease, or red blood cells (red cell casts) which are normally indicative of glomerulonephritis.
Treatment aims to halt the progression of disease, and aggressive immunosuppressive treatment is implemented if kidney complications occur. More specialised treatments are also available.
One third of persons with SLE will have kidney (renal) involvement, but only a small percentage (less than 5%) will go on to have life-threatening renal failure.
Cardiac disease (around 40%)
SLE is an inflammatory condition, and there is a 5-6 fold increased risk of developing coronary heart disease. This excess is especially pronounced in younger women, where the risk may be more than 50- fold. (Atherosclerosis is increasingly believed to be a chronic inflammatory condition).
Serositis may take the form of inflammation of the lining of the sac enclosing the heart causing pericarditis.
More rarely, the tissue of the heart (myocarditis, endocarditis) may be affected. Unexplained tachycardia (fast heartbeat) occurs in around 15% of patients with SLE.
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Pulmonary disease (around 50%)
There may be inflammation of the lining of the sacs enclosing the lungs causing pleurisy. This would be manifested by sharp stabbing pains in the chest on breathing in.
Pleurisy is a not uncommon initial presenting feature, and around 45% of patients may have pleuritic pain / pleuritis at some time.
Very rarely, the tissue of the lungs may be affected (lung fibrosis, alveolitis), causing pulmonary difficulties. The latter is known as “shrinking lung”, a rare complication. However, only a very small percentage will suffer this, and the resultant severe disability.
Blood cell disorder (less than 10%)
A very small percentage of people will get haemolytic anaemia – a recognised condition in which lupus auto-antibodies burst the red blood cells.
A low white blood cell count, which leads to the person being more prone to infection, or a low platelet count, which may cause bruising, may also be present, and will show up on a simple blood test.
Neurological involvement (around 50%)
This is an extremely important complication of SLE, but severe neurological complications represent a smaller percentage.
Central nervous system disease is more common than neuromuscular involvement, and carries a poor prognosis, as it is the third commonest cause of death in people with SLE. A wide variety of complications can occur, some of which are:
- Impaired concentration
- Impaired intellectual function
- Headaches (which can vary from persistent, low- grade headaches, tension headaches, cluster headaches, to severe migraine headaches),
- Depression and anxiety
- Epilepsy (including grand mal seizures)
- Strokes
- Cerebellar ataxia
- Movement disorders such as chorea (involuntary, uncontrolled, unco-ordinated movements)
- Cranial neuropathy
- Peripheral neuropathy
- Visual hallucinations
- Psychosis.
- Encephalopathy (degenerative brain disease)
- Myelitis (inflammation of the spinal cord)
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Modern treatment of SLE with immunosuppression has reduced the incidence of neurological complications.
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Amended November 2008
