OVERVIEW

SYSTEMIC LUPUS
Definition and classification

ERYTHEMATOSUS
Pathophysiology

Causes
ECN Item # 117

Epidemiology

Signs and symptoms

Diagnosis
Jeanne-Marie xxx DCEM2
Toulouse Purpan medical school
Treatment
January 7th 2009

Lupus and pregnancy

DEFINITION
PATHOPHYSIOLOGY
CLASSIFICATION
Defect in apoptosis

Definition:
Chronic, inflammatory autoimmune disorder Increased cells death + disturbance in immune tolerance

Dysregulated lymphocytes: autoantibodies

Several types of lupus:
Formation of immune complexes in the microvasculature
- Drug-induced lupus erythematosus
- Lupus nephritis Complement activation
- Discoid lupus
- Neonatal lupus Chronic inflammation

CAUSES EPIDEMIOLOGY

Genetic factors
Prevalence: 40 cases per 100 000 population
Inherited genes (HLA region on chromosome 6)

Incidence: 5 cases per 100 000 population

Environmental factors
- Sun exposure
Women > > Men
- Female hormones
- Extreme stress
Between 10 – 50 years
- Infections

Africans, Americans and Asians more

Drugs affected
Hydralazine, quinidine, procainamide, phenitoin, isoniazid,
d-penicillamine

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 SIGNS AND SYMPTOMS SIGNS AND SYMPTOMS

Constitutional: fatigue, fever and weight changes.

Pulmonary: pleurisy, dyspnea, pulmonary

Musculoskeletal: arthralgia, myalgia, and arthritis.
hypertension, hemoptysis

Dermatologic: malar rash, a photosensitive rash,

Gastrointestinal: abdominal pain, nausea and
discoid lesions, alopecia, Raynaud phenomenon. dyspepsia, autoimmune hepatitis

Renal: glomerular disease, acute nephritic disease,
Cardiac: pericarditis, Libman-Sacks
nephrotic syndrome, acute or chronic renal failure endocarditis, myocarditis, coronary thrombosis

Neuropsychiatric: headache, mood disorders such
Hematologic: leukopenia,
as anxiety and depression, cognitive disorders, thrombocytopenia, anemia
psychosis, seizures, myelopathy.

DIAGNOSIS TREATMENT
CRITERIA of American College of Rheumatology (ACR):
1. Malar rash (rash on cheeks)
2. Discoid rash
Education: protective clothing, sunglasses,
3. Photosensitivity solar cream
4. Oral or nasopharyngeal ulcers
5. Non erosive arthritis of two or more peripheral joints
No oestro-progestative contraception!!
6. Pleurisy or pericarditis
7. More than 0.5g per day protein in urine or cellular casts seen in
Non steroidal anti-inflammatory medications
urine under a microscope
8. Seizures or psychosis

Corticosteroid creams
9. Hemolytic anemia, Leukopenia (white blood cell count<4000/µl),
Hydroxychloroquine
Lymphopenia (<1500/µl) or thrombocytopenia (<100000/µl) in the
absence of offending drugs.
Corticosteroids or cytotoxic drugs
10. Antinuclear antibody test positive
11. Immunologic disorder: Positive anti-Sm, anti-ds DNA,
antiphospholipid antibody, and/or false positive serological test for
syphilis

LUPUS AND PREGNANCY TAKE HOME MESSAGE

Carefully planned!!

SLE: chronic auto-immune disease

Advance in diagnosis and treatment
have improved survival

Lupus under control or in remission

The most common cause of death =
infection due to immunosuppression

Risks for both the mother and the fetus:
The most sensitive screening test:
- neonatal lupus
- SLE can flare during pregnancy ANA (anti-Sm +++)

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BIBLIOGRAPHY

http://www.nlm.nih.gov/medlineplus/e
ncy/imagepages/9069.htm

http://emedicine.medscape.com/articl
e/332244-overview

http://www.medicinenet.com/systemic
_lupus/article.htm

http://lupus.webmd.com/guide/pregna
ncy-lupus