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Etiology:
(1) Genetic factors:
- familial incidence
- HLA-DR2 and DR3
Mechanism:
(a) Autoimmune disease: antinuclear antibodies
(ANAs) against several nuclear antigens (DNA,
RNA, nucleoproteins) react with nuclei of
damaged cells (cannot penetrate intact cells)
(b) Formation of immune complexes and their
deposition in different tissues
Pathological changes:
(1) Blood vessels:(acute necrotizing vasculitis)
- acute vasculitis of small arteries and arterioles
due to deposition of immune complexes
- necrosis & fibrinoid deposits within vessel wall
- later, fibrous thickening with luminal narrowing
- perivascular lymphocytic infilterate
(2) Heart:
- pericarditis
- myocarditis
- endocarditis (Libman-Sacks):
multiple, small, sterile, flat vegetations,
detachable
embolization
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(4) Skin:
- deposition of immune complexes along the
dermo-epidermal junction
- activation of complement
skin rash
inflammation
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(5) Joints:
- arthritis (most common presenting symptom)
resembles rheumatoid arthritis but:
- involves large and small joints
- mild (rarely causes destruction of cartilage)
(6) C.N.S:
- vasculitis
(7) Lung:
- pleurisy with pleural effusion
- alveolitis and fibrosis
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(8) Spleen:
- enlarged slightly
- capsule is thickened
- follicular hyperplasia
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Clinical features:
- females
- arthritis (larger joints)
- fever
- skin eruption (butterfly rash over face)
- hypertension
- hematuria, albuminuria, nephrotic syndrome
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Diagnosis :
- antinuclear antibodies (ANAs) in the serum
- presence of LE cells (nuclear debris ingested by
blood neutrophils)
Prognosis:
- chronic course with repeated exacerbations and
remissions
- survival rate is 90% at 10 years (death mostly due
to renal failure)
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