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The clinical picture



The onset of lupus can be either dramatic (coming on over a period of days) or, more commonly, gradual (sometimes missed or wrongly diagnosed in its early stages).


Key points

Clinical features include:

  • Depression/headache

  • Cold circulation (Raynaud's)

  • Flu-like symptoms

Lupus can settle down over time


Classical lupus

The most well known 'classical' picture of lupus is demonstrated by the following example.


A 22 year old woman with a 1-2 year history of vague general illness and fatigue develops fever, severe aches and pains, rashes, including a noticeable reddish rash on the cheeks and nose, and hair loss. Further testing reveals anaemia, a low white blood cell count and protein in the urine, suggesting kidney inflammation.


Prior to this increase in symptoms, she may have gone through a particularly stressful period in her life (eg, at home or in college).


There is often, as in this clinical example, a suggestion that mild or 'grumbling' lupus had been present for months or even years before the diagnosis.


In this patient it is possible that stress contributed to the flare While it is difficult to quantify stress, there is no doubt that this is a common story and one which does need more study.


However, while some triggering factors (eg, ultraviolet light, certain drugs such as Septrin®) are well recognised in lupus, in the majority of patients there is no clear antecedent history.


'Cold circulation' (Raynaud's)

Many lupus patients complain of cold fingers and toes. In some cases a more extreme form called Raynaud's phenomenon occurs, where the fingers (sometimes only one or two fingers) turn first white, then blue, and then red. In some patients (especially in children and teenagers), this 'cold circulation' can lead to small sores or chilblains on the tips of the fingers and toes. In many patients the history of cold circulation precedes the main diagnosis by many years.


It should be said that while Raynaud's phenomenon is troublesome to patients and in some cases severe, it rarely leads to more severe circulation disorders in lupus patients.


Fatigue

In any large survey of lupus, fatigue is the most common complaint. Often it is the sole and overwhelming symptom.


Aches and pains

High on the list of symptoms and often flu-like, the pains associated with lupus are not simply confined to the joints, but affect muscles and tendons, giving rise to the 'pain all over' description so often reported in active lupus. The joints can be swollen, but rarely to the degree seen in rheumatoid arthritis. Importantly, the joints seldom become permanently damaged, again unlike rheumatoid arthritis.


Tendon problems can be prominent; for example, the fingers can feel tight and bendy (difficulty saying one's prayers) and, in more severe cases, the fingers and thumb can become pulled out of shape. The hitch-hiking' thumb is an unusual but very characteristic feature in some lupus patients.


Rashes

Any rash can occur, though sun-exposed areas such as the face, the V of the neck, ear lobes and hands are especially frequently involved. The rashes are commonly worse after sun exposure.


Involvement of the scalp leads to hair loss, often mild, but sometimes patchy and even severe; this is an important clue in making a diagnosis.


Depression

This can be an important feature of lupus. It is often dismissed as merely secondary to the aches and pains of lupus. Wrong! Depression usually is an integral part of the disease in lupus, often improving strikingly when the lupus is treated. Indeed, some lupus patients who fare after pregnancy are labelled as having puerperal depression.


Headache

Another major feature of lupus is headache; any type of headache can occur, ranging from mild to very severe, including 'cluster headache' and migraine. Headache and migraine are strongly associated with the antiphospholipid (Hughes) syndrome, often improving or even disappearing with anticoagulant treatment.


Lupus over time

In many patients, as the decades go by, lupus settles down. Indeed more and more patients these days are eventually weaned off all drugs. Despite this optimistic outlook, many patients still suffer many flares of the disease. The unpredictability of lupus is what makes it so difficult to gauge treatment precisely for many patients.


Late complications

Even when the disease has gone quiet (ie, into remission), there are reasons for vigilance. Prolonged steroid treatment can lead to longer term complications, which can be identified and treated early in many cases. These include osteoporosis, bone softening and cataracts. Some lupus patients develop artery complications including heart attacks, and much effort is now focused on prevention, for example by maintaining a healthy diet, controlling cholesterol, avoiding smoking and, in those patients with sticky blood, using anti-clotting medicines such as low-dose aspirin.


My children

As has been stated, there is a definite, albeit weak, genetic tendency in lupus. If any patient is worried about their offspring having lupus, then a simple blood test (ANA) is usually adequate to screen for lupus. The most appropriate time to test (especially in girls who are more likely to be affected) is in the teens after puberty.

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