Chapter 112 Inflammatory Connective Tissue Disorders

SYSTEMIC LUPUS
ERYTHEMATOSUS (SLE)
osms.it/systemic-lupus-erythematosus

▪ Antiphospholipid syndrome
PATHOLOGY & CAUSES ▫ Hypercoagulable state; individuals
prone to develop clots (e.g. deep vein
▪ Chronic systemic autoimmune disorder; thrombosis, hepatic vein thrombosis,
wide range of clinical, serological features stroke)
▪ Periods of flare-ups, remittance
▪ Environmental triggers damage DNA →
apoptosis → release of nuclear bodies SIGNS & SYMPTOMS
▪ Clearance of apoptotic bodies ineffective
due to genetic defects → increased amount ▪ Fever, joint pain, rash in sun-exposed areas
of nuclear antigens in bloodstream → ▪ Typical rashes
initiates immune response → production of ▫ Malar rash (butterfly rash): over cheeks
antinuclear antibodies → bind to antigens,
▫ Discoid rash: plaque-like/patchy
form immune complexes
redness, can scar
▪ Complexes deposit in tissues (e.g.
▫ General photosensitivity: typically lasts
kidneys, skin, joints, heart) → Type III
few days
hypersensitivity reaction
▪ Individuals may develop antibodies
targeting molecules (e.g., phospholipids) of
red, white blood cells → marking them for
phagocytosis → Type II hypersensitivity
reaction

RISK FACTORS
▪ Genetic defects associated with SLE
▪ UV radiation
▪ Smoking
▪ Viral, bacterial infections
▪ Medications (e.g. procainamide,
hydralazine, isoniazid, estrogens)
▪ More common in individuals who are
biologically female, of reproductive age

COMPLICATIONS
Figure 112.6 A butterfly rash on the
▪ Cardiovascular disease
face of an individual with systemic lupus
▫ Libman–Sacks endocarditis, myocardial erythematosus.
infarction (MI)
▪ Serious infections; renal failure;
hypertension

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 ▪ Weight loss
▪ Ulcers in oral/nasal mucosa
DIAGNOSIS
▪ Serositis (e.g. pleuritis/pericarditis) OTHER DIAGNOSTICS
▪ Libman–Sacks endocarditis: formation of
nonbacterial vegetations on ventricular, Diagnostic criteria (4 of 11)
atrial valve surfaces; mitral, aortic valves ▪ Malar rash
(most common) ▪ Discoid rash
▪ Myocarditis ▪ General photosensitivity
▪ Renal disorders ▪ Oral/nasal ulcers
▫ Abnormal levels of urine protein, diffuse ▪ Serositis
proliferative glomerulonephritis
▪ Arthritis in ≥ two joints
▪ Neurologic disorders
▪ Renal disorders
▫ Seizures, psychosis
▪ Neurologic disorders
▪ Hematologic disorders
▪ Hematologic disorders
▫ Anemia, thrombocytopenia, leukopenia
▪ Antinuclear antibodies
▫ Very sensitive, not specific
▪ Other antibodies
▫ SLE specific: anti-Smith, anti-dsDNA
▫ Anti-phospholipid: anticardiolipin
(false-positive test for syphilis); lupus
anticoagulant (lupus antibody); anti-beta
2 glycoprotein I

TREATMENT
▪ Goal: prevent relapses, limit severity

MEDICATIONS
▪ Long term therapy
▫ Antimalarial agents
▪ Mild to moderate manifestations
▫ Non-steroidal anti-inflammatory drugs
(NSAIDs), low doses of corticosteroids
▪ Severe/life-threatening manifestations
Figure 112.7 An MRI scan of the head of ▫ High doses of corticosteroids, intensive
an individual with SLE who presented with immunosuppressive drugs
altered mental status and seizures. There
a numerous small infarcts suggestive of
cerebral vasculitis. The individual improved OTHER INTERVENTIONS
after treatment with steroids. ▪ Avoid sun exposure
▪ Physical exercise
▪ Balanced diet
▪ Smoking cessation
▪ Immunizations

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 Chapter 112 Inflammatory Connective Tissue Disorders

Figure 112.8 A histological section of a Figure 112.9 Histological appearance of the

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