World Lupus Day: What you need to know about the autoimmune condition

May 10th is dedicated to wolf, one autoimmunity condition which belongs to the family of rheumatic diseases.

More than 500,000 Europeans suffer from lupus. The 90% of patients are women and 80% of diagnoses is made between ages 15-45 years old.

The forms of the disease

There are two common forms of lupus: o discoid and systematic.

Lupus discoid is a disease of skin, which is often chronic and leads to scarring. It can be limited to the skin and not associated with diseases of other body systems. In other cases, discoid lupus may be one of the features of the systemic form of lupus.

Systemic lupus erythematosus (SLE) is a chronic, systemic, inflammatory condition. Chronic means that it lasts for a long time, possibly for the rest of life. However, almost all people with lupus experience fluctuations in disease activity, known as outbreaks and recessions. At times there may be no signs or symptoms of lupus at all (recessions). Some people have complete and long-lasting remissions. A systemic condition is one in which many different parts of the body can be affected. The term inflammatory refers to the body's reaction, leading to pain, heat, redness and swelling. Although lupus is a chronic condition, this does not mean that the patient cannot have a fulfilling life. Chronic conditions cannot be cured, but can be controlled with appropriate treatment.

This article focuses on systematic form of the wolf.

What causes SLE?

The etiology of the disease is unknown and it is impossible to predict who will develop it. However, there is a disorder of the body's immune system, which, while it normally protects the body from harmful agents, in the case of lupus produces autoantibodies, i.e. substances directed against the body itself. These substances circulate in the blood and cause a disturbance in the various organs.

The pathophysiology is multifactorial and not fully understood, with interaction of genetic predisposition (genes) with environmental factors. It affects women (usually of reproductive age) more than men and is more common in people of color and Asians. It can affect patients of any age, including neonates.

Certain drugs (procainamide, hydralazine, minocycline, diltiazem – calcium channel blockers, penicillamine, INH, quinidine, methyldopa, anti-TNF, and IFN-α) cause a reversible SLE-like syndrome (pharmacogenic lupus with positive ANA and anti-histone antibodies).

How is the diagnosis made?

Simple blood and urine tests, as well as special ones (ANA, anti-ds DNA, ENA, etc.), combine to help the doctor diagnose the condition, determine its severity and monitor the patient's course, as well as the effectiveness of the treatment he administers.

Diagnosis is based on clinical and serological criteria (ANA, anti-dsDNA in 70%, anti-Sm and anti-RNP in 40%, anti-SSA/Ro and SSB/La in 30% and antiphospholipid antibodies). Depletion of complement fractions (C3 & C4) is nonspecific, but is common in disease flares and is usually associated with renal involvement.

Other blood tests may be done to help diagnose and monitor disease activity. Biochemical blood tests will help determine whether vital organs such as the kidneys and liver are functioning normally. Because kidney problems often occur, a urinalysis, a 24-hour urine collection or, more rarely, a kidney biopsy, in which a small piece of tissue is removed from the kidney and subjected to special tests, may be needed.

What are the treatments available?

Treatment of SLE depends on the severity of the condition. Various drugs are used and research is done to find the most effective formulations so that the patient can be relieved of the symptoms and enjoy as normal a life as possible.

Today, the drugs used are mainly nonsteroidal anti-inflammatory drugs, antimalarials (for alopecia, rashes, arthralgias), corticosteroids and immunosuppressive drugs [cyclophosphamide, azathioprine, mycophenolate mofetil, methotrexate (for musculoskeletal and skin manifestations)] and rituximab, monoclonal antibody against the CD20 molecule on the surface of B-cells (for severe cases uncontrollable in conventional therapy). Sun protection is recommended for all patients with photosensitive rashes.

Benlysta, a drug given intravenously once a month, has also been approved in the US and Europe and appears to significantly and safely help a number of patients.

The course of the disease is usually variable and unpredictable. The choice of a specific drug and its dose depends on the patient's symptoms and its choice is the duty of the attending physician.

What is the prognosis for patients?

The outcome of SLE improves dramatically with the timely and correct use of glucocorticosteroids and immunosuppressive agents. Many patients with childhood-onset SLE do very well. But the disease can be serious and life-threatening, or it can remain active during adolescence and into adulthood.

The prognosis of SLE in childhood depends on the severity of the involvement of the internal organs. Children with severe kidney and central nervous system disease require aggressive treatment. In contrast, mild rash and arthritis can be easily controlled. However, the prognosis for each individual child is relatively unpredictable.

What symptoms does lupus cause in the initial stage?

The first symptoms of lupus usually appear in the early years of adulthood.

Clicking on the photos of below gallery you will see what are the 10 main symptoms caused by lupus in the initial stage.

Source: Hellenic Society of Rheumatology (http://www.tosomasoumilaei.gr), Hellenic Society of Anti-Rheumatism (EL.E.A.N.A.), Mayo Clinic, Healthline