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Systemic Lupus Erythematosis (SLE) (Pathophysiology: Type III…
Systemic Lupus Erythematosis (SLE)
What is SLE?
Chronic, multisystem autoimmune disease which targets connective tissue
Treatment modalities
NSAIDs
Antimalarial drugs reduce flares
Corticosteroids
Immunosuppressants
Biologics
Control inflammation/immune response, treat acute symptoms as needed
Lifestyle changes
Avoid sun, wear sunscreen
Smoking cessation
Regular exercise
Proper diet, focusing on vegetables, fruits, whole grains
Supplementation (vitamin D/calcium)
Regular doctor checkups
Stress management
Acupuncture to help joint/muscle pain?
Support groups
Weight loss if indicated
Pathophysiology: Type III hypersensitivity reaction
B & T cells fail tolerance but still get released into body; self-reactive B & T cells circulating
Damaged cell releases DNA or nucleoprotein
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IgG antibodies combine with soluble antigens (DNA and nucleoproteins in lupus); become self-reactive
Immune complexes
are small and evade detection/phagocytosis
Plasma cells secrete IgG antibodies
Deposition in blood vessel walls triggers complement system --> increase in vascular permeability
Antigen-antibody (immune) complexes
deposit in blood vessel walls
Inflammation results in tissue damage
Resulting edema activates chemokines, draws neutrophils to site of deposition
Neutrophils unable to phagocytize and degranulate, releasing lysozymes and reactive oxygen species
Vasculitis results in more inflammation and ultimately more damage to cells and more DNA antigen release
Degranulation causes vasculitis
Clinical manifestations
Malaise
Headache
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Fatigue
Rashes
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Symmetrical butterfly rash on face
Diagnosis can be difficult, as signs/symptoms are often general and can be attributed to other conditions
Joint pain and swelling, especially in hands/wrists
Fever
Muscle pain
Anorexia
Edema
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Pleural effusion
Pericardial effusion
Epidemiology
Women 9 times more likely to be diagnosed with SLE
Exact prevalence unknown (not a reportable condition)
Estimated prevalence in US is 160,000 (definite) to over 320,000 (definite/probable)
Generally diagnosed at younger ages; 20% diagnosed after age 50
Altered systems and complications
Gastrointestinal
Malabsorption
Dyspepsia, nausea
Oral ulcers
Vasculitis bowel ischemia
Renal
Glomerular disease
Lupus nephritis
Renal failure (common cause of death)
Hematuria, proteinuria
Respiratory
Pulmonary hypertension
Right-side heart failure
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Pulmonary fibrosis
Pleurisy, pleuritis
Pulmonary embolism
Gynecological
Higher risk of miscarriage
IUGR (intrauterine growth restriction)
Premature menopause
Cardiovascular
Myocarditis
Endocarditis
Pericarditis/effusion
Higher risk of atherosclerosis
Vasculitis
Heart failure
Neurological
Seizures
Cerebellar ataxia
Stroke, ITA
Neuropathy
Dementia
Cognitive decline
Demyelination disorders
Headaches
Dermatological
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Scarring from rashes
Alopecia
Psychiatric
Anxiety
Psychosis
Depression
Musculoskeletal
Bone necrosis
Jaccoud's arthropathy
Arthritis, arthralgia
Prognosis
Renal failure or cardiovascular disease most common causes of death
Reduced survival versus non-lupus patients
Etiology/risk factors
Genetic factors
Involved genes often influence/control immune system
Accumulated polymorphisms
Family history of autoimmune diseases
Sex hormones, esp estrogen
No single cause
Infections, esp viral
Poor diet
Stress
Chemical exposure
Diagnostic tests/criteria
Family history of lupus or other autoimmune diseases
Physical exam
Skin or kidney biopsy
Medical history
D.O.P.A.M.I.N.E. R.A.S.H.
Discoid lupus: circular, red macules
Oral aphthous ulcers
Photosensitivity
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Arthritis: hand wrist, knee
Malar rash: butterfly macules face
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Immunologic antibodies: anti-ds (anti-double stranded) DNA, anti-Smith (ribonucleoprotein), anti-Ro, anti-La
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Neurological: psychosis, seizures
ESR (erythrocyte sedimentation rate) increase
Renal disease
ANA (anti-nuclear antibody) positive test
Serositis: pleurisy, pericarditis
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Hematologic: hemolytic anemia, thrombocytopenia, leukopenia
Blood and urine tests
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