1. Systemic Lupus Erythematosus
SLE
The term lupus, which is Latin for wolf, was developed in the 13th Century and is commonly used for the disease technically known as systemic lupus erythematosus or SLE for short. Lupus got its name because it was thought to look like the lesions found on patient’s faces, which resembled a wolf’s bite.
Dr. M Jokar

2. Definition Lupus: wolf Chronic Unknown etiology Connectie tissue dis.
Autoimmune
Multisystem

3. Lupus Types SLE DLE(Discoid lupus erythematosus) Drug induced lupus
Neonatal lupus

4. Epidemiology
Prevalence in
Urban area > rural areas

5. Epidemiology
Race
The prevalence of SLE is higher among Asians, Afro-Americans, Afro-Caribbeans, and Hispanic American

6. Epidemiology Sex: 95% in women Children 3:1 In adults 7-15:1
Older individuals 8:1

7. Epidemiology Age: all ages but more common in child- bearing ages
20% <16 years
65% years
15% >55 years

8. Pathogenesis Abnormal immune response B and T cell hyperactivity
Genetic background + Environmental factors
Abnormal immune response
B and T cell hyperactivity
Autoantibodies Immue complexes
Tissue damage

9. Genetic background Evidence for genetic predisposition:
concordance in monozygotic 24-58%
first degree relatives %
genes:
C4AQO HlA-DR2 HLA-DR3

10. Environmental factors
UV-B(sometimes UV-A)
Chemicals: hydrazines-hair dye?
Viruses
Drugs
Sex hormones
Stress
Foods

11. Autoantibodies ANA Anti-DNA Anti-Sm
Anti-RNP ,Anti-Ro=SS-A , Anti-La=SS-B
Anti-Histone , Antiphospholipid , Antiplatelet
Antierythrocyte , Antilymphocyte
Antineuronal

12. Clinical manifestations
Most patients experience exacerbations interspersed with periods of relative quiescence.
True remissions with no symptoms and requiring no therapy occur in up to 20% but are usually not permanent.
Systemic symptoms are usually prominent and include fatigue, malaise, fever, anorexia, and weight loss.

13. Clinical manifestations
Can affect any organ in the body including the joints, skin, lungs, heart, blood, kidney, or nervous system.
Can range from mild to life threatening.
No two people will have identical symptoms.
Systemic lupus erythematosus (SLE) can affect any system or organ in the body including the joints, skin, lungs, heart, blood, kidney, or nervous system. Symptoms of SLE can range from being minor to very serious or life threatening. A person may experience very little to no pain or they may experience extreme pain, especially in the joints. There may be no skin involvement or rashes that are disfiguring. They may have no organ involvement or extreme organ damage. Generally, no two people with systemic lupus will have identical symptoms. Most often when people mention "lupus," they are referring to the systemic form of the disease.

14. Musculoskeletal
Almost all patients experience arthralgias and myalgias
most develop intermittent arthritis
Joint deformities are unusual
Erosions are rare
Myopathy and myositis
Osteoporosis and AVN

15. Cutaneous Manifestations
Malar ("butterfly") rash

16. Cutaneous Manifestations
Discoid lupus erythematosus (DLE)

17. DISCOID LUPUS Affects the skin, hair or mucous membranes.
Identified by a rash or lesions.
Negative or low titer ANA.
Diagnosed by biopsy of rash.
10% will evolve into SLE.
Discoid or Cutaneous lupus (skin) lupus (DLE): affects primarily the skin, but may also involve the hair and mucous membranes. There is more than one type of cutaneous lupus, which can cause different looking rashes and symptoms. These include:
Acute cutaneous lupus erythematosus (ACLE)
Subacute cutaneous lupus erythematosus (SCLE)
Chronic cutaneous lupus erythematosus (CCLE) or Discoid lupus erythematosus (DLE)
Cutaneous lupus is usually identified by a rash or lesions that appear on the face, neck, and/or scalp (or other sun exposed areas). The lesions can appear patchy, crusty and cause scarring. Cutaneous lupus does not generally involve the body's internal organs. Therefore, the ANA test, which is a blood test used to help diagnose systemic lupus, may be negative in these patients. However, in some patients with discoid lupus, the ANA test is positive, but at a low level or "titer." Cutaneous lupus is diagnosed by examining a biopsy of the skin rash. In approximately 10 percent of patients, cutaneous lupus will evolve into the systemic form of the disease. This cannot be predicted or prevented. Treatment of cutaneous lupus will not prevent its progression to the systemic form, and individuals who progress to the systemic form probably had systemic lupus with the rash as their initial symptom. Topical and interlesional corticosteroids are usually effective for localized lesions, antimalarials or thalidomide may be needed for more generalized lesions.

18. Cutaneous Manifestations
Maculopapular rash
Subacute cutaneous lupus erythematosus (SCLE)
Alopecia
Photosensitivity
Other rashes:maculopapular,urticaria, bullae, erythema multiforme

20. Alpecia

21. Renal Manifestations One-half have clinical nephritis
Most have immunoglobulins deposited in glomeruli
Early in the disease most are asymptomatic
Urinalysis shows hematuria, cylindruria, and proteinuria

22. WHO classification of lupus nephritis
Class I: normal
Class II: mesangial lupus nephritis
Class III:focal proliferative nephritis
CLASS IV:diffuse proliferative nephritis
Class V : membranous lupus nephritis
Class VI: ESRD

23. Nervous System
CNS
PNS

24. CNS Any region of the brain can be involved in SLE
Mild cognitive dysfunction is the most
Seizures ,Headaches, demyelinating disorders, cerebrovascular disease, movement disorders, aseptic meningitis, myelopathy, subarachnoid hemorrhage
Psychosis,mood disorders, depression and anxiety

25. PNS Mononeuropathy Polyneuropathy Mononeuritis multiplex
Acute demyelinating polyneuropathy (Guillain-Barre)

26. Vascular System Thrombosis, emboli vasculitis Atherosclerosis
Raynauds phenomenon

27. Hematologic Manifestation
Anemia: Anemia of chronic disease,hemolytic anemia
Leukopenia (usually lymphopenia)
Thrombocytopenia
Antiphospholipid antibodies and lupus anticoagulant
Bleeding syndromes
Splenomegaly,lymphadenopathy

28. Heart Pericarditis Myocarditis
Valvular insufficiency (usually aortic or mitral)
Libman-Sacks endocarditis
Myocardial infarcts

29. Respiratory tract Pleurisy and pleural effusions pneumonitis
Interstitial pneumonitis leading to fibrosis
Pulmonary hypertension
Respiratory distress syndrome
Intraalveolar hemorrhage
Infections

30. Gastrointestinal System
Nausea, diarrhea, and vague discomfort
Peritonitis
Vasculitis of the intestine
Acute pancreatitis
Hepatitis
Hepatomegaly

31. Ocular Manifestation Retinal vasculitis conjunctivitis episcleritis
optic neuritis
sicca syndrome

32. LABORATORY MANIFESTATIONS
CBC:anemia, leukopenia, lymphopenia, and thrombocytopenia
ESR,CRP
Urinalysis :proteinuria, hematuria, and cellular or granular casts

33. LABORATORY MANIFESTATIONS
Immunologic tests:
ANA
AntiDNA
AntiSm
Anticardiolipin
C3,C4,CH50

34. PREGNANCY
Fertility rates are normal
Spontaneous abortion and stillbirths are frequent (10 to 30%), especially in women with LA and/or aCL
Disease flares in a small proportion, especially during the 6 weeks postpartum

35. Neonatal lupus
Caused by transmission of maternal anti-Ro across the placenta
Transient skin rash
Permanent heart block
Transient thrombocytopenia

36. Renal and CNS involvement are rare
DRUG-INDUCED LUPUS
Drugs:procainamide, hydralazine, isoniazid, chlorpromazine, D-penicillamine, methyldopa, quinidine, interferon a, and possibly hydantoin, ethosuximide, and oral contraceptives
Clinical manifestations: systemic complaints arthralgias; polyarthritis and pleuropericarditis
Renal and CNS involvement are rare
Lab:All patients have ANA and most have antibodies to histones,

37. DRUG-INDUCED LUPUS Anti dsDNA and hypocomplementemia are rare
Anemia, leukopenia, LA, aCL, thrombocytopenia,,, false-positive VDRL, and positive direct Coombs' tests can occur
Symptoms rarely persist more than 6 months; ANA may persist for years

38. PROGNOSIS Survival : 90 to 95% at 2 years 82 to 90% at 5 years
Poor prognosis factors:
high serum creatinine,Black Race ,hypertension,nephrotic syndrome thrombocytopenia, serious CNS involvement antibodies to phospholipids

39. Causes Of Death Cardiac involvement Pulmonary involvement
CNS involvement
Renal involement
Infections

40. DIAGNOSIS Medical history (including family history)
Complete physical examination
Laboratory tests
Skin or kidney biopsy

41. ACR DIAGNOSTIC CRITERIA
Skin criteria
1. Butterfly rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
Systemic criteria
5. Arthritis
6. Serositis
7. Kidney disorder
8. Neurologic disorder
To assist the physician in the diagnosis of lupus, the American College of Rheumatology (ACR) has developed a set of classification criteria used to help distinguish lupus from other diseases. A person should have four or more of these symptoms to suspect lupus. It is important to remember that the symptoms do not have to occur at the same time, and the criteria do not include all possible symptoms of lupus like fever, fatigue, hair loss, or Raynaud's phenomenon. The criteria are meant only to help with diagnosis.
Butterfly or Malar Rash
Rash over the cheeks and/or nose.
Discoid Rash
Red raised patches anywhere on the skin, but usually in the sun exposed areas of the face, arms, neck, hands.
Photosensitivity
Reaction to sunlight, resulting in the development or increase in skin rash or general feeling of illness.
Oral Ulcers
Ulcers in the nose or mouth, usually painless.
Arthritis
Non-erosive arthritis involving two or more peripheral joints (arthritis in which the bones around the joints do not become destroyed).
Serositis
Pleuritis or pericarditis – pain in the chest with deep breathing.
Renal Disorder
Excessive protein and/or cellular casts (abnormal elements the urine, derived from red and/or white cells and/or kidney tubule cells) in the urine.
Neurologic Disorder
Seizures (convulsions) and/or psychosis.
Hematologic Disorder
Hemolytic anemia (low red blood cell count), leukopenia (low white blood cell count) or thrombocytopenia (low platelet count).
Immunologic Disorder
Positive LE prep test, positive anti-DNA test, positive anti-Sm test or false positive syphilis test (VDRL).
Antinuclear Antibody
Positive test for antinuclear antibodies.
Laboratory criteria
9. Hematologic abnormalities
10. Immunologic disorder
11. Antinuclear antibody

42. COMMON LUPUS MEDICATIONS
NSAIDs
Antimalarials
Corticosteroids
Immunosuppressants
Investigational (research)
Medications play an important role in the care of people with SLE. For most people with lupus, effective treatment can minimize symptoms, reduce inflammation, and maintain normal body functions. Treatment approaches are based on the specific needs and symptoms of each person. The medication prescribed usually depends on which organ(s) are involved and the severity of the involvement. The choice of drugs is highly individualized and typically changes often during the course of the disease. The medications used in the management of lupus include:
·        NSAIDs
·        Antimalarials
·        Corticosteroids
·        Immunosuppressants
·        Investigational (research)