SLE (systemic lupus erythematosus) also simply called as lupus is an autoimmune disorder in which auto antibodies & immune complexes may attack various tissues and organs in the body and cause their malfunctioning.

The role of immune system is to protect the body however there are certain instances where instead of helping it actually troubles the body and may produce myriad number of disease conditions and this situation is called as autoimmune disorder.

Frequency & distribution

A whopping 90% of SLE sufferers are women and belonging to the reproductive age however no race, age, sex is exempt from this disease.

What causes SLE?

SLE as mentioned above is an autoimmune disorder and appears to result from a complex interaction between;

* Genetic &
* Environmental factors.

The environmental factors thought to contribute for the development of SLE include;

* Gender (Female sex – hormonal factors)
* Ultraviolet light
* Infections
* Other factors.

The staggering high incidence of SLE in women is imputed to the female hormonal factors. Estrogen hormone in certain ways appears to activate or sustain the activity of the abnormal immune pathway and effectuate the cell damage. Women who are on estrogen containing birth control pills or HRT (hormonal replacement therapy) are found to have increased susceptibility to SLE further supports the hormonal role.

When SLE patients are exposed to UV light about 70% of patients develop flare up of this condition and it indicates that this environmental factor has some role too.

EBV (Epstein-Barr virus) is one of the viral environmental factors that are ascribed to trigger the SLE in susceptible patients. Studies have shown that SLE patients have a higher incidence of this viral infection as compared to non SLE people. The virus survives in side the B lymphocytes for decades and also certain amino acid sequences of this virus resemble the sequences contained in human body cells so the antibodies generated against EBV may act as auto antibodies and damage the patient’s tissues and organs.

It appears that it all begins initially in the form of an introduction of certain antigens to the susceptible individual. Patient’s body tries to tackle these antigens through its immune system by activating its various immune components as T cells, B cells, complement actors, macrophages etc. Due to certain handicap in the immune system the antigen are not contained efficiently and this results in the formation of abnormal immune complexes, auto antibodies etc.

The activation of immune & complement system promotes the release of myriad chemical substances including cytokines, chemotaxins, vaso-active peptides, destructive enzymes etc and all these above developments result in the cell damage.

Body involvement in SLE

SLE is one of those diseases that can affect majority of the tissues and organs in the body. Some of common organs involved and the resulting disease or clinical manifestations are highlighted below.

* Neurological involvement

Diffuse lupus cerebritis
Seizures (fits)
Strokes
Bleedings
Psychosis
Depression etc.

Lung related;

Inflammation of pleura (lung covering)
Pleural effusion
Lung infiltrates
Interstitial inflammation an fibrosis
Bleeding into lung tissue etc.

Heart related;

Inflammation of pericardium (heart covering)
Myocarditis
Libman-Sacks fibrinous endocarditis
Heart attacks etc.

Blood vessel (vascular) manifestations;

Vasculitis (inflammation of blood vessels)
Strokes
Heart attacks etc.

Skin manifestations

Lupus dermatitis etc.

Kidney manifestations

Lupus nephritis
Nephrotic syndrome
ESRD (end stage renal disease) etc.

Hematological & vascular manifestation

Anemia
Hemolysis
Lymphocytopenia
Thrombocytopenia
Venous & arterial clots etc.

GI (gastrointestinal) manifestations

Nausea, vomiting & diarrhea
Autoimmune peritonitis
Intestinal vasculitis etc.

Eye manifestations

Sicca syndrome etc.

Diagnosis

The diagnosis of SLE requires both clinical and investigational inputs.

The Investigations arranged during the diagnosis & evaluations of SLE include:

* Blood tests
* Urine analysis
* Imaging studies (e.g. Ultrasound, Echo, x-rays, CT, and MRI) etc.

The blood tests are done mainly to look for abnormal antibodies. ANA (antinuclear antibodies) are highly sensitive (about 98%) but not specific. Antibodies to double strand DNA (Anti-dsDNA) and to smooth muscle antigen (anti-sm) are highly specific for SLE although not as sensitive as ANA.

The other antibodies some times tested during the diagnosis of SLE (less sensitive & specific than the three antibodies mentioned above) include;

* Antiphospholipid
* Anti-RNP
* Antihistone
* Anti-Ro (SS-A)
* Anti-La (SS-B)
* Anti-platelet
* Anti-erythrocyte
* Anti-ribosomal P
* Anti-neuronal antibodies etc.

Other blood tests include ESR (sed rate) or CRP (c reactive protein) and these tests come frequently abnormal especially during active flare ups although they are not specific for this condition.

Diagnostic criteria for SLE

There are 11 points listed as below that are included for helping with the diagnosis of SLE. If ?4 are present then the diagnosis of SLE is highly likely with specificity approximately 95% and sensitivity approximately 75%.

1. Malar rash
2. Discoid rash
3. Light sensitivity (photosensitivity)
4. Ulcers in the mouth & naso-pharynx
5. Arthritis
6. Serositis
7. Kidney diseases
8. Neurological diseases
9. Hematological disorders
10. Immunological disorders
11. Antinuclear antibodies

Management

The management of SLE generally includes;

* Stabilization of the patient
* Hospitalization if necessary
* Symptomatic management (pills for pain etc)
* Specific treatment;

Steroids
Hydroxychloroquinone
Methotrexate
Cyclophophamide
Azathioprine
Mycophenolate etc.

There is no curative treatment as of now so the treatment is focused on controlling the acute attacks, prevention of future attacks, and symptomatic treatments.

Generally when a patient presents with a SLE flare especially a severe one the patient needs to be admitted to the hospital an treated wit a course of intravenous methyl prednisolone (a steroid) and this is generally followed by a course of tapering dose of oral steroids like prednisolone and a small dose ma be continued as maintenance dose.

If the attacks are serious, life threatening or involving vital organs like kidney etc then generally a combination of steroid and one of the immunosuppressive agents (cyclophosphamide, mycophenolate etc) are used.

The chronic usage of steroids and immunosuppressive agents unfortunately produce a host of complications including;

* Infections
* Gastrointestinal ulcers
* Hypertension
* Diabetes mellitus
* Osteoporosis (thinning of bone)
* Develop of tumors like lymphomas, leukemia etc.

For the management of skin conditions including the facial rash topical steroids, sunscreen agents etc are of help.

Not so with HIV. Spread primarily through sexual contract or infected blood products, HIV turned out to be a relentless insurgent, ravaging the immune systems of most people who came in contact with it. Over time, by destroying the cells that direct the rest of the immune system (the helper T-cells or CD4 cells), HIV makes it harder and harder for an infected body to fight off certain cancers and infections. As the disease progresses, bacteria, viruses and other microbes that would rarely harm a healthy person can explode into severe, sometimes fatal, infections.

Technically, a diagnosis of HIV infection becomes an AIDS diagnosis only after blood tests show that an infected person has fewer than 200 helper T-cells per microlitre of blood. (Healthy adults usually have 1000 or more t-cells per microlitre of blood.) Without treatment, the number of CD4 cells drops over time, the patient becomes more and more immunosuppressed and develops severe infections. Many of the more than 35 million or so people worldwide now infected with HIV are so debilitated they can’t hold down a job or even do household chores. Some of them experience periods of intense, life-threatening illness followed by periods of normal functioning. But a very small number of patients with HIV show no outward evidence of infection at all, even 18 years or so after contracting the virus.

What does the future holds AIDS / HIV patients?

Protease inhibitors, a new class of anti-HIV drugs, have increased life expectancy and reduced illness episodes for many AIDS patients. Scientists are now trying to find out how long-term survivors are different and why they are protected. On other fronts, a flurry of research is focusing on creating the next generation of antiviral drugs, including ‘fusion inhibitors’ that cripple HIV’s ability to attach to and disable white blood cells. Although there are no absolute answers or solutions yet, the fervent desire to stop the rapid spread of HIV has shed much light on the secrets of the immune system – knowledge that is already helping to prolong life and promote health for all, not just those with HIV.

This immune-system disaster typically ravages the body in three main ways:
1. As a direct attack on an organ, such as the skin (which occurs in psoriasis, for example);
2. As a domino effect – for instance, when lupus causes inflammation in the kidneys that leads directly to kidney damage; and
3. As a response that starts in one spot, then spreads. The swellings of rheumatoid arthritis affects joints first but can move on to involve other tissues.

What allows the immune system to run amok in this way is quite a mystery. It seems that many factors from viruses, to certain drugs, to sunlight – may play a role. Heredity appears to be a strong influence, but, oddly when these disorders cluster in families, they can surface as different illnesses. A mother may have lupus; her daughter, juvenile diabetes; and her grandmother, rheumatoid arthritis. Although ‘auto-immune disease’ refers to more than 80 illnesses, only a few affect lots of people.

Among those that do are:
- Hashimoto’s thyroiditis
Continued attack by the immune system destroys the thyroid gland, an organ that helps control the body’s metabolism, leading to an underproduction of thyroid hormone. Luckily, the deficiency can be made up with medication.
- Graves’ disease
This is another auto-immune problem involving the thyroid, but this time, it has the opposite effect, with the gland producing too much of the thyroid hormone.
- Lupus
This disorder (the full name is systemic lupus erythematosus) can involve inflammation of the different tissues and organs, such as the joints, skin, kidneys and even the brain, in different people.
- Rheumatoid arthritis
This immune system attacks the tissue that lines and cushions the joints – most often in the knees, wrists and hands – causing scarring within the joint that eventually disables one in ten sufferers.
- Multiple sclerosis
A disorder in the which the immune system methodically destroys myelin, the coating that protects the nerve fibres of the eye, brain and spinal cord. For some reason, people who grow up in tropical climates are much less likely to suffer from this disease.

Women & Autoimmune Diseases

Scientists don’t know for certain why, but the vast majority of auto-immune diseases occur in women.
Below are the female to male ratios of autoimmune diseases:

• Hashimoto’s thyroiditis – 50:1
• Systemic lupus erythematosus – 9:1
• Antiphospholipid syndrome – 9:1
• Sjögren’s syndrome – 9:1
• Primary biliary cirrhosis – 9:1
• Autoimmune hepatitis – 8:1
• Graves’ disease – 7:1
• Rheumatoid arthritis – 4:1
• Scleroderma – 3:1
• Juvenile diabetes – 2:1
• Multiple scleroris – 2:1