Lupus – a basic understanding

Health Focus
By DR DAVID GOTLIEB, a leading Cape Town rheumatologist

The body has an immune system designed to protect the individual from infections and external threats but the immune system may become autonomous and cause disease by attacking the person’s body itself.

This phenomenon is called autoimmunity, and there are multiple diseases which arise through the inability of the body to distinguish itself from an outside environmental agent or infection. Collectively, the diseases are known as Autoimmune disease. 

These diseases are based on a genetic component, and exposure to an infection or unknown agent in the environment, frequently years before onset of clinical disease.

These include, amongst many diseases:

•  Rheumatoid arthritis, which primarily affects the joints.
•  Crohn’s disease which affects the bowel.
•  Ulcerative colitis, which affects the colon.
•  Psoriasis can affect skin, nails, tendons and joints.
•  Systemic lupus erythematosus (lupus) which can affect multiple organ systems.

In the case of lupus, what follows is activation of the blood white cells, especially lymphocytes, and the production of antibodies which attach to and damage or destroy various organ tissues. 

The disease occurs in 1-10 per 100 000 people worldwide, but some populations are more susceptible to it, e.g. people of mixed race in the Western Cape, African American women, Hispanics, and Asians. There is also a significant incidence among Jewish people, especially those of  Sephardic background. In fact, a study in Israel showed between 49 and 155 cases per 100 000. The incidence among Ashkenazi Jews is lower, and the disease is generally less severe among Ashkenazim. 

Lupus is more common among women in the age group 15-45. However, approximately 10% of cases start in childhood.

The severity of the condition may also be affected by genetics and one’s hormone status. 

It presents in a number of different ways, and the clinical manifestations vary from minor symptoms to life-threatening disease.

These are illustrated in the table below.

Diagnosis

The diagnosis is made on clinical suspicion with the clinical findings of the patient, with a full history and examination, since the illness is so multisystem in nature. At the same time an indication of severity is made, by assessing for major organ involvement, especially kidney involvement. Thereafter blood can be sent to the pathologists, who conduct various tests on the blood, and do special tests for lupus antibodies.

Once a diagnosis is suspected, the specialist of choice is the rheumatologist who will liaise with other specialists, especially renal physicians. A kidney biopsy might be required.

With relatively few trained rheumatologists in South Africa, it may be necessary to consult a physician who will liaise with a rheumatologist as best as possible.

The disease is characterised by flares of activity, with constitutional, skin and joint disease seen very frequently. The organ complications are less common, but the specialist should see the patient more frequently, depending on symptoms, or for regular monitoring every six months. The patient should have their urine checked at each visit.

Treatment of lupus

Treatment of the disease has improved significantly over the past 30 years and, today, with proper treatment, patients can have normal lives, and have normal pregnancies. In fact, survival has improved from 50%, in the past, to over 90% in the current era.

Various medications are used to assist patients: from antimalarial treatment with hydroxychloroquine; to antiinflammatory medications; to cortisone, in varying doses for immune suppression; to immune suppressant drugs; and the newer class of biologic drugs.

World Lupus Day is held on 10 May to draw attention to this medical condition. The disease gets its name from the Latin word for ‘wolf’ (Lupus): many people with lupus show a distinctive rash, which looks like the bite of a wolf. 


Editor’s comment:
I came across this interesting World Economic Forum video which is about why autoimmune diseases affect more women than men. Take a look here. (Source: World Economic Forum via LinkedIn)



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