Field of

Medicine
Medicine And Surgery Program

Lecture : Systemic lupus erythromatosis

Dr. Rana Atef Khashaba Date : / 11 /2023

Systemic lupus erythromatosis
 Table of contents

2
Definition Diagnostic criteria Clinical finding
Risk factor (2019)
Pathogenesis

Investigation Treatment
 1
Definition
Risk factor
Pathogenesis
Definition:
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with relapsing and
remittion course.
Characterized by:
• Autoantibody response to nuclear and cytoplasmic antigens (SLE-induced
thrombocytopenia).
• Deposition of Ag-Ab complexes in the capillaries & small vessels leading to vasculities,
vasculopathy ((lupus nephritis & arthritis)
Organs affected:
• SLE can affect any organ system, but mainly involves the skin, joints, kidneys, blood cells,
and nervous system.
Incidence:
• SLE affects both males and female of all age groups
• The most commonly: female during their child -bearing years female to male 9: 1
• This ratio is approximately 3:1 in younger and older population.
Aetiology:
Unknown
Risk factors:
• Inherited factors: HLA B8, DR2 & 3, 10% have positive family history
• Congenital deficiencies of complement (C4, C2) are also associated with an increased
risk of SLE.
• Estrogen: SLE excerbates during early pregnancy and with contraceptive pills
• Infection: Viral infection mainly
• Drugs: Drug-induced lupus:
Anticonvulsants (phenobrabital)
Antihypertensives (hydralazine - Methyldopa)
Antiarrhythmics (procainamide)
Oral contraceptive pills induce exacerbations
Pathogenesis:
• Defective immune regulatory mechanisms, such as the clearance of apoptotic cells and
immune complexes.
• The loss of immune tolerance
• Defective B cell and T cell suppression,
 2
Diagnostic criteria
(2019)
Diagnostic criteria (2019):
❖ Suspect SLE in any (female) patient with multisystem disease + positive ANA
❖ The diagnosis of SLE is strongly suggested, when a person has 4 or more of these criteria
(4/11)
• 1. Malar rash
• 2. Discoid rash (plaque like)
• 3. Photosensitivity: Reaction to sunlight, resulting in the development of or increase in skin
rash
• 4. Painless oral ulcers
• 5. Serositis: Pleurisy or pericarditis
• 6. Neurological: Psychosis or seizures
• 7. Arthritis: Peripheral joints Non ereosive arthritis (2 or more)
• 8. Hematological: Pancytopenia/bicytopenia/hemolytic anemia
• 9. Renal: Proteinuria (>0.5 gm/day) or cellular cast
• 10. Positive anti-nuclear antibody (ANA)
• 11. Immunological investigations: Positive (+ve) Anti- ds DNA &/or anti-smith (sm) antibodies
 Malar rash

Discoid rash
 3
Clinical finding
Clinical finding:
Sex: Female : Male = 9 : 1
Age of onset: 14 – 40 years old
Course: episodic (remissions & exacerbations)
• General featurs: fever, headache, malaise, anorexia, loss of weight
• Articular (joint) symptoms: the earliest symptoms, in 90% of patients, they include:
❖ Arthritis and arthralgia:
✓ Symmetrical
✓ Involving (≥ 2) small or large peripheral joints
✓ May be associated with morning stiffness > 30 minutes (but less common than RA)
✓ Bone erosions & joint deformities are uncommon , less than 10% of patients (in contrast
to RA)
❖ Osteoporosis: due to corticosteroids, early menopause, +ve family history
❖ Avascular necrosis: (temporary or permanent loss of blood supply to the bone) due to
steroids, hyperlipidemia, Raynaud's phenomenon, antiphospholipid antibodies
Avascular necrosis of femoral neck at both
hip joints

Photosensitivity
rash
• Extra-articular symptoms:
❖ C.V.S:
✓ Libman-Sacks endocarditis: non-infective endocarditis of mitral or tricuspid valves
✓ Pericarditis: the most common cardiac symptom
✓ Myocarditis
✓ Hypertension (HTN) & ischemic heart disease (IHD)
✓ Vasculitis: affecting small vessels of visceral organs
❖ Chest:
✓ Lung: interstitial fibrosis, pulmonary hypertension, pulmonary embolism
✓ Pleura: Pleurisy - Pleural effusion
❖ Abdomen:
✓ Esophagitis, gastritis, pancreatitis, hepatosplenomegaly (HSM)
✓ Renal (Lupus nephritis): Common, characterized by edema, hypertension, proteinuria &
haematuria
❖ Nervous system: Psychosis, convulsions, stroke & peripheral neuropathy
❖ Mucocutaneous:
✓ Malar (butterfly) rash: (30%) characterized by erythema & induration of cheeks & nasal
bridge
✓ Discoid rash: raised, erythematous plaques with adherent scales and occur most
commonly on the face, scalp, and neck
✓ Photosensitivity: skin rash on sun-exposed areas as a result of unusual reaction to sunlight
✓ Alopecia: patchy or diffuse alopecia and thin, friable hair
✓ Oral ulcers: usually painless and can occur on the hard palate, tongue
✓ Purpura, urticaria rash, Rhynaud’s phenomenon
❖ Eye: Conjunctivitis, scleritis (blue sclera)
❖ Blood: hemolytic anemia, bicytopenia or pancytopenia with or without lymphadenopathy
❖ Complications:
.Renal failure .Pericardial/ pleural effusion .Heart/respiratory failure
.Stroke .Permanent alopecia .Pathological fractures
.Anti-phospholipid antibody syndrome
Alopecia
 4
Investigations
Investigation:
1- Serological tests:
✓ Antinuclear antibody (ANA) test: the most sensitive test, positive in 90% of cases
✓ Anti- double stranded (ds) DNA antibody: the most specific test for diagnosis & disease
activity
✓ Anti-Smith (sm) antibody: also important specific test for diagnosis, not disease activity
✓ Anti-cardiolipin antibody test: positive in Antiphospholipid syndrome (15-50 percent of SLE
patients, increase the risks for blood clots and may be responsible for narrowing of and
irregularities in blood vessels, Antiphospholipid antibodies are linked with miscarriages
and other complications such as strokes, heart attacks and blood clots in almost any part
of the body, including kidneys, legs, lungs, and eyes)
✓ Anti-histone antibody: indicates drug-induced lupus
✓ Complement levels: serum C3 & C4 levels are decreased
during disease activity
✓ LE cells: leukocytes with large inclusion bodies
(Ag-Ab complexes).
Investigation:
2- CBC: anemia, bi-or pancytopenia
3- Urine analysis: haematuria, proteinuria, cell casts
4- Kidney functions tests
5- ESR: > 100
6- Biopsy: Skin biopsy
Kidney biopsy: (tubular atrophy and interstitial fibrosis, and extensive
glomerular arteritis)
5
Treatment
Treatment:
General measures:
• Use of sunscreen
• Regular exercise & healthy diet
• Avoidance of estrogen-containing contraceptive pills

Specific therapeutic options:

Mild-to-moderate disease: (no major organ involvement)
• NSAIDs: for mild arthritis, serositis
• Corticosteroids: topical creams for skin lesions
• Antimalarial agents: such as hydroxychloroquine for rash & arthritis
Severe disease (major organ involvement):
glomerulonephritis, vasculitis, hemolytic anemia, severe thrombocytopenia,
myocarditis, seizures, pulmonary interstitial fibrosis or alveolar hemorrhage)
• High doses of systemic corticosteroids: IV methylprednisolone 500-1000 mg/day for
3 days or oral prednisone 40-60 mg/day>>>> to induce remission
• Plasmapheresis: if corticosteroids alone are not effective
• Immunosuppressive drugs:
✓ Cyclophosphamide, methotrexate, azathioprine and mycophenolate
For long-term control of the disease (maintenance of remission)
✓ Intravenous immunoglobulins: such as Belimumab, in lupus nephritis, vasculitis.
THANK YOU