THE DIAGNOSIS OF LUPUS



LUPUS is an autoimmune disease, a type of self-allergy, whereby the patient's immune system creates autoantibodies which instead of protecting the body from bacteria, viruses and other foreign matter attack the person's own body tissues. This causes symptoms of extreme fatigue, joint pain, muscle aches, anaemia, general malaise and can result in the destruction of vital organs. It is a disease with many manifestations, and each person's profile or list of symptoms may be different. LUPUS can mimic other diseases, such as multiple sclerosis and rheumatoid arthritis, making it difficult to diagnose by GPs as they see few cases of lupus and thus are not alert to its possibility.

Physicians are often cautious with their diagnosis as they do not want to label anyone until they are certain of LUPUS. Moreover, a careful review of the patient's entire medical history is necessary, coupled with analysis of results obtained from tests relating to their immune status to provide accurate diagnosis. Currently there is no single test that can definitely say whether a person has LUPUS or not. Only by comprehensive examination and consideration of symptoms and their history can a diagnosis be achieved.

LUPUS is a complex disease in which almost every system of the body can be affected, and DIAGNOSIS is bases on a combination of symptoms, signs and test results. Diagnosis of LUPUS or any other chronic illness may be established using the 5 Step Programme.

1. Review patient's symptoms.

2. Detailed physical examination

3. Battery of tests.

4. Rule out other diseases.

5. Time is sometimes necessary to observe the course of the disease.


Once a diagnosis of LUPUS is made, the patient's symptoms should be treated as necessary. The goal of the treatment is to bring the symptoms and the disease under control so that the patient can lead as normal a life as possible.

Dr Graham RV Hughes
Consultant Rheumatologist
St Thomas' Hospital,
London SE1 7EH.

THE ST THOMAS' 14 CRITERIA FOR THE DIAGNOSIS OF LUPUS

The American Rheumatism Association criteria has provided the benchmark for the classification of lupus for the last 20 years. Many thousands of lupus patients passing through St Thomas' Hospital, London, have led Dr Graham Hughes to offer the following 14 criteria aimed more toward diagnostic help and not to aid classification.

1. Teenage "growing pains"

Growing pains in the UK, is a label widely used for joint pains in teenagers and seems to cover a spectrum of rheumatology from arthritis through to lupus.

2. Teenage migraine

headache, cluster headache and migraine can be encountered and a strong history of teenage migraine may be of lupus significance, either at the time or subsequently.

3. Teenage "glandular fever"

Prolonged teenage glandular fever is a label which crops up time and time again in lupus patients and prolonged periods off school in many SLE patients is a recurrent theme.

4. Severe reaction to insect bites

This is a feature of so many lupus patients. Not only are they susceptible to insect bites but often reactions are severe and prolonged - the skin is a major organ affected by lupus.

5. Recurrent miscarriages

Lupus itself seems not to be a cause of recurrent miscarriage but where the antiphospholipid syndrome (APS) is present, recurrent spontaneous fetal loss can be significant.

6. Premenstrual tension

Although difficult to quantify, it is believed that significant pre-menstrual disease flare is sufficiently prominent in lupus to be included in this list. All rheumatic diseases are clinically influenced by the menstrual cycle.

7. Septrin (and sulphonamide allergy)

Adverse reactions to these drugs is quite common in lupus and the clinical onset of the disease may have coincided with the use of eg Septrin.

8. Agoraphobia

Agoraphobia/claustrophobia are often present at a time when lupus disease is active. A history of these conditions (including panic attacks), can be protracted, lasting for months or even years. In many cases the history is not volunteered or the episodes are in the interim considered unrelated to lupus.

9. Finger Flexor Tendonitis

Arthralgia and tenosynovitis are common features in lupus and although not specific, the finding of mild to moderate ten-finger flexor synovitis is a useful pointer in the presence of other lupus features. It si subtly yet significantly different in pattern from other arthritic diseases.

10. Family history of autoimmune disease

As the genetics and statistics of the various autoimmune diseases become better defined, the strength of a particular family history will become more precise. the family history is important, as lupus is genetically determined (although not 100% concordant as with, for example, genetic diseases which are always passed on eg haemophilia).

11. Dry Shirmer's Test

A "bone dry" Shirmer's Test (levels of eye moisture) points towards one of the autoimmune diseases and in the patient with vague or nonspecific symptoms is worth its weight in gold.

12. Borderline C4

Genetic complement deficiencies have been known to be associated with lupus for over three decades and in the diagnostically difficult patient, especially where a family history is present, borderline C4 levels can be significant indicators.

13. Normal CRP with raised ESR

An important diagnostic aid. A very low CRP in an otherwise inflammatory situation is strongly supportive of lupus or primary Sjögren's Syndrome.

14. Lymphopenia

In the patient with non-specific complaints and unremarkable blood tests, a borderline or low lymph count can be overlooked. It can be common in lupus and is certainly worth inclusion among minor criteria.

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