Systemic Lupus Erythematosus is chronic inflammatory multisystem disease of unknown etiology. It is an autoimmune disease where body’s immune system (antibodies in this case referred to as autoantibodies) mistakenly attacks its own tissues, causing multi-organ inflammation and diverse clinical manifestations with domination of peripheral symmetric polyarthritis of small and large joints. SLE is characterized with periods of exacerbation and remission.

Origins of the name: Systemic Lupus Erythematosus

“Lupus” is Latin for wolf, “Erythro” in Greek stands for red, and Systemic is English word meaning that multiple organs are involved. One theory explains that this disease has gotten its name because it is similar to the attacks of a wolf on humans with its severity, random spots of attack and repetitiveness.

SLE results in tissue damage caused by attack of autoantibodies and immune complexes. It involves polyclonal and antigen-specific T and B lymphocyte hyperactivity. T cell help in production of autoantibodies is critical for development of full-blown disease.

Proposed Etiology:

Definitive etiology is still unknown. We could only hypothesize what causes this disease is: genetics, environmental factors (sun exposure to UV light), estrogen (prepubertal and postmenopausal women have similar incidence to men; men with SLE have higher concentration of estrogenic metabolites), infection (viral: non-specific stimulant of immune response, medications (Dilantin-anticonvulsant), oral contraceptive pills are associated with exacerbation (they should be avoided in SLE patients). 25% of SLE patients have experienced false-positive tests for syphilis due to circulating lupus anticoagulant in the blood.

Diagnostic criteria updated by American College of Rheumatology in 1997 states that at least 4 or more of 11 (7 clinical and 4 laboratory) criteria must be present serially or simultaneously:

Diagnostic Criteria Description

Clinical

1. Malar rash Classic “butterfly rash”; sparing of nasolabial folds, no scarring
2. Discoid rash; May cause scarring since invasion of basement membrane
3. Photosensitivity; Skin rush in reaction to sunlight
4. Oral/nasal ulcers; Usually painless
5. Arthritis; Symmetric, involving 2 or more small or large peripheral joints, non-erosive
6. Serositis; Pleuritis or Pericarditis
7. Neurologic disorder; Seizures or Psychosis

Laboratory

8. Renal disorder; Proteinuria
9. Hematologic disorder; Hemolytic anemia, Leukopenia, Lymphopenia, Thrombocytopenia
10. Immunologic disorder; Anti-double stranded DNA Ab (50% of patients), anti-Sm Ab (25-60% of patients), anti-phospholipid Ab.
11. Antinuclear antibody –(ANA); Most sensitive test (present in 90%of the patients)

Laboratory tests that will determine diagnosis of Lupus:

• Serologic diagnosis made by high titre of ANA detected by immunofluorescence, but this test doesn’t determine diagnosis because many other autoimmune disease have positive ANA test
• Anti-dsDNA antibodies detected by Crithidia test and anti-Sm antibodies are specific for SLE (95-98% of the cases) and these tests determine the diagnosis of Lupus.

Signs and Symptoms:

1. Musculoskeletal
The most common manifestations of SLE are Arthralgias and Nonerosive Arthritis occurs in 95% of patients, it is symmetric and involves small joints of hands, wrists, and feet).There is also avascular necrosis (cause of pain, along with arthritis) and myositis.
2. Dermatologic
Abnormalities of the skin, hair or mucous membranes are second most common manifestation of SLE, occurring in 85% of patients.
The most common skin manifestation is the classic malar butterfly rash, an erythematous rash covering both cheeks and the bridge of the nose, with sparing of the nasolabial folds.
The second most common skin manifestation is maculopapular rush that can be located anywhere on the body. Also are present: nasal/genital ulcers, panniculitis, alopecia, urticaria and purpura.

First symptoms to occur: 1. Fatigue 2. Myalgias 3. Arthtritis

Radiographic characteristics:

Radiographically, the arthritis of SLE is non-erosive. This is helpful for differential diagnosis with Rheumatoid Arthritis where there is bone erosion on radiographs.

Three histological lesions are most characteristic of SLE:
1. Onion-skin lesions – found in arteries of the spleen, which consist of concentric layers of fibrosis surrounding the vessel
2. Libman-Sacks verrucous endocarditis: vegetations on heart valves
3. Hematoxylin bodies: globular masses of bluish, dense, homogenous material seen on hematoxylin and eosin stain.

Microscopic features of oral lesions:

Histologically, lesions reveal lichenoid mucositis with perivascular exudate and thickening of basement membrane.

Long term use of medications to control Lupus can induce significant intra-oral pathology.
Corticosteroids: can lead to root canal calcification, delay of tooth eruptions and root dilaceration.
Steroids: can cause necrotizing ulcerative gingivitis.
NSAID’s: can induce gingival bleeding, but because there is a possibility NSAID’s to inhibit alveolar bone resorption, periodontal health in some patients with Lupus has been found improved due to intake of these medications.
Cyclosporine: is a common cause of gingival enlargement (hyperplasia).
Immunosuppressive treatment: fights against intra-oral infections but promotes Candidiasis and Herpes Simplex Virus infections.

Preventive dental hygiene care in Lupus patients is very important. Chlorhexidine mouthwashes could help contain periodontal disease. Mucous membrane ulcers can be treated with hydrogen peroxide gargle, buttermilk gargle, or steroid impregnated gel. Intralesional injection of corticosteroids are also effective. Bacterial, viral and fungal infection should be treated with conventional, proven therapy specific for the infection present. Dental procedures should not be undertaken on patients with active Lupus, or if necessary, antibiotic premedication is advised, due to high incidence of bacterial endocarditis.

Survival in patients with SLE is 90 to 95% at 2 years, 82 to 90% at 5 years, 71 to 80% at 10 years, and 63 to 75% at 20 years. Disability in SLE patients is common. 20% of patients experience remissions. Infections due to immunosuppressive therapy and renal failure are the leading causes of death in the first decade of disease. Thromboembolic events are frequent causes of death in the second decade.