Systemic Lupus Erythematosus Disease

DESCRIPTION

A multi-system, autoimmune inflammatory condition characterized by a fluctuating, chronic course. Varies from mild to severe and may be lethal (CNS and renal forms).

System(s) affected: Hemic/Lymphatic/Immunologic, Nervous, Renal/Urologic, Endocrine/Metabolic, Skin/Exocrine, Gastrointestinal, Musculoskeletal
Genetics: Markers: HLA-B8; HLA-DR2; HLA-DR3
Incidence/Prevalence in USA: 20/100,000
Predominant age: All ages, but 30-50 are most common
Predominant sex: Female > Male (10:1)

SIGNS AND SYMPTOMS

Arthritis
Fever
Anorexia
Malaise
Weight loss
Skin lesions
Oral ulcers
Eye pain and/or redness
Chest pain and/or shortness of breath
Pallor
Nausea, vomiting, diarrhea
Muscles – tenderness, aching and stiffness
Headaches and visual problems
Psychosis/delirium

CAUSES

Most cases are idiopathic
Drugs – drug-induced lupus is clinically different from idiopathic SLE

RISK FACTORS

Race – blacks, Hispanics, Asians, and Native Americans have higher prevalence than whites
Genetic markers – HLA-B8, HLA-DR2, HLA-DR3
Hereditary complement deficiency especially C1q, C1r, C1s, C4, and C2
Polymorphisms in the Fc gammaRIIa and Fc gammaRIIIa genes may be important risk factors in SLE

LABORATORY

Positive antinuclear antibody (ANA)
Anti-double stranded DNA (dsDNA), anti-Sm, false-positive VDRL, or positive LE preparation. These tests have either high sensitivity (ANA, false-positive VDRL) or specificity (anti-dsDNA, anti-Sm, and LE preparation) and are included as American Rheumatology Association (ARA) criteria for the diagnosis of SLE along with the clinical features.
Sedimentation rate is nonspecific, but valuable in assessing activity of SLE
Anemia
Anticardiolipin antibody
Leukopenia
Lymphopenia
Abnormal urinary sediment
Proteinuria
Increased prothrombin time
Hypoalbuminuria
Thrombocytopenia
Increased serum creatinine
Positive Coombs test

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Connective tissue disorders affecting skin, blood vessels, serous and synovial membranes

Collagenous swelling
Fibrinoid change
Cellular necrosis
Periarterial sclerosis
Granulomatous reaction
Infiltration of polymorphonuclear leukocytes, plasma cells, lymphocytes in walls of small vessels, arterioles of skin, spleen, glomeruli, endocardium, pericardium, brain
Hematoxylin bodies resembling those in LE cells
Vegetation on heart valves

SPECIAL TESTS

Complement levels, immune complex assays (cryoglobulins, Raji cell test, C1q precipitins)
Coagulation studies (lupus anticoagulant)
Biopsy of skin, kidney, and peripheral nerves may reveal typical histopathology

IMAGING

Cerebral angiography in CNS lupus
Chest x-ray for pulmonary infiltration, pleural effusion
MRI to detect CNS lupus
Echocardiogram for pericardial effusion

DIAGNOSTIC PROCEDURES

American Rheumatology Association (ARA) criteria are a combination of any 4 manifestations of the 11 listed
- Malar (butterfly) rash
- Discoid rash
- Photosensitivity
- Oral/nasopharyngeal ulcers
- Nonerosive arthritis
- Pleuritis or pericarditis
- Renal disorder – proteinuria or cylindruria
- Neurologic disorder – psychosis or seizures
- Hematologic disorder – hemolytic anemia, leukopenia (less than 4,000), lymphopenia (less than 1,500), thrombocytopenia (less than 100,000)
- Immunologic disorder
- Positive antinuclear antibody (ANA) in absence of drugs known to cause positive ANA

Note: While the above criteria are required for proper epidemiologic classification of SLE, in practical situations, the combination of a multi-system inflammatory illness, positive antinuclear antibody (ANA) and absence of a better diagnosis often represents the most practical way to make a clinical diagnosis

APPROPRIATE HEALTH CARE

Outpatient with regular monitoring

GENERAL MEASURES

Avoidance of or protection from ultraviolet light by using sunscreens, hats, etc.
Early intervention when infections occur
Energy conservation
Stress avoidance/management

SURGICAL MEASURES

N/A

ACTIVITY

As active as possible
Those with arthritis may be limited by their pain, but active exercises are to be encouraged

DIET

No special diet unless for complications such as renal failure

PATIENT EDUCATION

Printed materials available on lupus from the Arthritis Foundation, 1314 Spring Street N.W., Atlanta, GA 30309, (404)872-7100; and from the Lupus Foundation of America, 1717 Massachusetts Avenue, NW, Suite 203, Washington, DC 20036, (800)558-0121

PREVENTION/AVOIDANCE

Avoiding sun exposure is only necessary for approximately one sixth of SLE patients (those who self-report such sensitivity)
Routine vaccinations are safe and appropriate for SLE patients
Drugs known to induce SLE in normal individuals are not necessarily contraindicated in patients who have idiopathic SLE

POSSIBLE COMPLICATIONS

Fever, vasculitis, panniculitis, myositis, avascular necrosis of bone, endocarditis, pulmonary fibrosis, renal failure, organic brain syndromes, peripheral neuropathy, stroke syndromes, pancreatitis and elevated liver enzymes, infertility, ascites, venous thrombosis, seizures

EXPECTED COURSE AND PROGNOSIS

Most patients with lupus follow a course of remissions and exacerbations. Many experience spontaneous permanent remission.
Treatment of renal lupus (the most serious form) with immunosuppressors, renal dialysis, and renal transplantation has increased the five-year life expectancy to over 90%. For those patients surviving the first two years of disease, life expectancy is essentially normal.
In patients with drug-induced lupus, symptoms should gradually decrease upon discontinuation of the suspected agent.

ASSOCIATED CONDITIONS

Other autoimmune diseases - rheumatoid arthritis, hypothyroidism, diabetes

AGE-RELATED FACTORS

Pediatric: Stroke syndromes frequently seen in children
Geriatric:

Higher percentage of males involved among the elderly
Since “false-positive ANA” reaches 15% in the elderly, caution in interpretation is required in this age group

Others: N/A

PREGNANCY

Onset of lupus and lupus flares are more common during pregnancy
Fetal loss is increased for mothers with lupus
Newborns of mothers who have lupus are more likely to have cardiac arrhythmias
Specialists' collaboration during pregnancy is indicated

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.