SYSTEMIC LUPUS ERYTHEMATOSUS

What is it?

Systemic Lupus Erythematosus (SLE) is an inflammatory disease of unknown cause.

Disease outbreak is triggered by unknown external agents (virus, bacteria, chemical agents, ultraviolet radiation) entering the immune system of an individual having various genes that wrongly induce an inadequate production of antibodies. .

These antibodies are directed against normal structures (self-antibodies) provoking lesions in tissues and also alterations in the blood cells.

It is a reasonably common disease in the rheumatologist´s office.

Better medical knowledge and advancement in the medical diagnostics must be the reasons for the SLE to be diagnosed with more frequency and its prognostic is much better than 15 years ago.

It affects principally women (9:1) in the reproductive age, initiating more commonly between 20 and 40 years old. Its spectrum ranges from quite benign to extremely severe to even fatal.

How does one feel?

The clinical manifestations are very different among the patients. The most frequent complaints are: malaise, fever, fatigue, weight loss and lack of appetite, which may precede other alterations for weeks or months. Patients may already be feeling mild joint or muscular pain, and showing red skin rashes often mistaken for urticaria, the most frequent alterations occurring on the skin and joints.

Skin and mucous membrane

There are many types of SLE-related skin lesions. The most known one is the butterfly-shaped rash, which is a raised erythema across the cheeks and back of the nose. Erythematosus rashes, flat or raised, may appear on any part of the body.

Many patients with SLE react to the sunlight (photo-sensitivity). So, these rashes can be prominent or limited to sun-exposed areas only. Other times, the lesions are more deep-rooted, leaving a scar (discoid lupus).

They start with a desquamation process over the erythematosus rash. Over time, the central area becomes atrophied and the skin eventually looses its color, leaving a scar that can be very uncomfortable.

There are cases of discoid lupus in which there will never be other problems, that is, there will not be systemic lupus. These patients must be monitored attentively because there is no way to follow up the evolution without physical exam and additional laboratory tests.

Hair loss is very frequent. Lots of hair falling out in bunches and many times found on the pillow. It is a signal that the disease is active.

The appearing of scars on the nose, tongue and oral mucous membrane, even though they are not frequent, is helpful for the diagnosis.

Locomotor Apparatus

The great majority of the patients has arthritis. It is usually mild and gets better rapidly with the treatment. However, there are a few cases showing destructive lesions t hat can be very severe.

The long-time tretament with corticoids, which is sometimes inescapable, can provoke, in around 5% of the patients, necrosis in the long bones extremities, principally the femur.

Tendinitis occurs with frequency and may follow the crises of arthritis or have an isolated manifestation. Not very common areas like the Achilles´ tendon may hurt for a long time. There are but a few cases with severe lesions.

Chronic Lupus can cause hand deformities resembling rheumatoid arthritis. Myositis (inflammation of the muscular fibers) is not a common event, but it can be severe and mistaken for other muscular diseases. A discreet muscular pain may happen but it is not worrying.

Kidneys

Very often lupica glomerulonephritis may develop. Fortunately, the majority of the patients suffer of non-progressive mild lesions, showing alterations in the urinalysis, as its only discreet evidence, or presenting renal lesions that respond very well to the treatment. When there are proteins, red or white blood cells, and various types of urinary casts in the urinalysis, in addition to the increase of creatinine in the blood, we are facing a severe situation but not one without solution. Also, The increase of the blood pressure is indicative of the severity of the condition.

The Nervous System

More than half of the patients with SLE have both their peripheral nerve roots and the central nervous system (CNS) compromised.

Migraine type headache is the most common symptom when there is inflammation of the CNS. But, in fact, as it is a very frequent complaint among ordinary people, it is very often overlooked. Not rarely, other manifestations may appear such as:

Peripheral neuritis (feeling of burning, tingling, and loss of strength).
Behavior disorders such as irritability, crying easily, more severe depression and even psychosis.
Seizures (may be the first manifestation with children).
Chorea (uncoordinated involuntary movements of the upper and lower limbs), is a much rarer condition.

There is a golden rule to be obligatorily followed in neurolupus: rule out the possibility of infection.

Other conditions to be observed, occurring in patients with chronic disease, are anxiety and depression, that may be severe, in addion to aesthetic problems provoked by dermatitis or the use of corticoid.

The antiphospholipid syndrome can be an isolated condition or follow SLE. Thrombi occurs in blood vessels and arteries of any size, causing embolism. The development of micro-thrombi in the brain causes small infarcts, with manifestations little observed at the beginning. It might be a cause of severe repercussion of the SLE on the CNS. Thrombi lodging in the placenta can provoke miscarriage.

Heart

Isolated inflammation of the membrane that surrounds the heart (pericarditis) is not rare and is easily solved. Severe valve lesions, myocardial and the coronaries inflammation are not frequent. Palpitations, lack of air and precordial pain are warning signs. They can be present since the onset of the condition.

Lungs

More than a half of the patients feel backaches or ribcage pain due to inflammation of the pleura. When it is mild, it only appears when one takes a deep breath and the radiography can be normal, that is, without effusion. As it gets worse, the pain becomes stronger and the respiration more difficult and followed by a dry cough.

Inflammation of the alveoli may also develop (be careful with infections at the same time), in addition to the arteries (rare and very severe).

Vessels

SLE very often have cold hands, which when in contact with extremely cold surfaces, or when the room temperature is low, change from pale to purple (cyanosis) and at times with pain in the fingertips. It is called the Raynaud´s phenomenon. It can happen with people who will never have the disease but may precede by years the other manifestations of SLE or other inflammatory autoimmune diseases.

Inflammation of the vessels is called vasculitis. Depending on the intensity of the inflammation there will be from erythematous rashes to gangrene spots on the area irrigated by the compromised vessels.

Eyes

Conjunctivitis or other manifestations are little common. One severe complication are thrombi in the back of the eye when the antiphospholipid syndrome is present.

Digestive System

Severe complications are very rare. Medications are the most frequent cause of complaints such as heartburn, abdominal pain and lack of appetite.

Few patients have an increase of the hepatic enzymes, showing the presence of liver inflammation. In this case, one must always rule out the simultaneity of two diseases and look for viral infection.

Blood

Mild anemia is very common and it is controlled with the regular treatment of the disease. It gets worse in patients with a severe condition and when there is renal insufficiency.

Antibodies directely targeted against the red blood cells can be of difficult control; they occur in less than 20% but can be one form of SLE onset and, as the high-dose corticoid treatment can mask other manifestations, the diagnosis might be missed.

There can be an important reduction in the white blood cells (risk of infection) and in the platelets (risk of bleeding).

How is the diagnosis made?

The diagnosis of SLE is made through the association of both the clinical and laboratory data.

The doctor must consider SLE, and there are some clues that are very helpful when the clinical manifestations are poor: women during the reproductive period (children and women after the menopause also have lupus) with joint pain, ill-feeling, weight loss, relapsing urticaria, hair loss, the Raynaud´s phenomenon, old blood tests with alterations such as low white count, alterations in the urine, unexplained anemia can be early manifestations of disease onset.

Research on autoantibodies is used for diagnosis and some of them are used for disease follow-up:

ANA – (antinuclear antibody) is the most frequent
Anti-dsDNA – sign of active disease and usually with renal disease
Anti-SM – not very frequent but, when present, they confirm the diagnosis

The clinical use of the presence of these autoantibodies and of several others is extremely helpful. It must be made by the reumathologist because they are not specific, that is, they appear in more than one disease and the association of the presence of one or more autoantibodies with the clinical analysis is what allows the physician to reach a diagnosis.

SLE -Diagnoses Criteria

In the table that follows are the1982-revised criteria of the American School of Rheumathology, to be used by physicians:

Malar rash: Fixed erythema, flat or raised, over the malar eminences and back of the nose
Discoid rash: erythematous patches with adherent scaling, follicular plugging and atrophic scarring
Photosensitivity: skin rash as a result of sunlight exposure
Oral ulcers: Oral or nasopharyngeal ulceration, observed by physician
Arthritis: nonerosive arthritis involving two or more pheripheral joints
Serositis: Pleuritis – documented by physician; pericarditis documented by ECG ou physician
Kidney disorder: proteinuria higher than 0.5 grams per day or higher than 3+ in regular tests; cellular casts – red cell, granular, tubular or mixed
Neurological disorder: seizures or psychosis in the absence of other offending cause
Hematologic disorders: hemolytic anemia – less than 4,000 leucocytes/mm3 on two or more occasions; less than 1,500 lyphocytes/mm3 on two or more occasions; less than 100,000 platelets/mm3 in the absence of other offending cause
Immunologic disorders: positiv Anti-DNA or anti-Sm or false positive test for lues (syphilis) for more than 6 months with normal FTA-ABS
positive ANA: in the absence of drugs to be associated withg “drug-induced lupus”.

In order to reach a SLE-diagnosis, four or more of the eleven criteria are necessary. For the purpose of identifying patients in clinical studies, we should strictly observe the sum of the criteria. In practical terms, if we have two or three “strong” criteria such as arthritis, dermatitis and ANA, and do not detect any other disease, we make the diagnosis and proceed with the treatment, as an early efficacious treatment always leads to a more successful prognostic.

How should it be treated?

Notwithstanding the international protocol guidelines for the treatment of complexe diseases such as SLE, each patient has his/her own history.

We certainly know what works best for cerebritis, nephritis, dermatitis, but the outcomes are individual. SLE-treatment is not a ready-made plan to be executed and the characteristics of each case will indicate how we should address it, in fact turning the treatment into an artisanal procedure.

The medication used may cause important side effects and should be handled by qualified professionals only.

Patients must be on the alert to the symptoms of the disease and to its complications that, though rare, may result. If they are treated promptly they can be readily resolved.

Question you can ask your doctor

Is there a cure for SLE?

What is the purpose of the treatment?

Is the treatment complete with this prescription or should I repeat it?

Is there any interference with other medications I am currently taking?

What are the side effects? Should I get routine control-tests?

Are there any problems with obesity?

How important are physical exercices and rest?

What concerns should I have about my daily, professional and leisure habits?

Are there any problems with contraceptive use? And pregnancy?

Are there any problems with sun or fluorescent light?

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