Definition

Acute or insidious in its onset, it is chronic, remitting and relapsing, often febrile illness, characterized principally by injury to skin, joints, kidney and serosal membrane

Definition
Acute or insidious in its onset, it is chronic, remitting and relapsing, often febrile illness, characterized principally by injury to skin, joints, kidney and serosal membrane

Definition
Acute or insidious in its onset, it is chronic, remitting and relapsing, often febrile illness, characterized principally by injury to skin, joints, kidney and serosal membrane

Definition
Acute or insidious in its onset, it is chronic, remitting and relapsing, often febrile illness, characterized principally by injury to skin, joints, kidney and serosal membrane

Definition
Acute or insidious in its onset, it is chronic, remitting and relapsing, often febrile illness, characterized principally by injury to skin, joints, kidney and serosal membrane

Criteria of the ARA for the classification of SLE

1. Malar rash: Fixed erythema over malar areas, sparing nasolabial folds 2. Discoid rash: Erythematous raised patches with keratotic scaling and follicular plugging 3. Photosensitivity: Skin rash after exposure to sunlight, history or physical exam 4. Oral ulcers: Oral or nasopharyngeal, painless, by physical exam

5. Arthritis:Tenderness, swelling, effusion in 2 or more peripheral joints

6. Serositis: A) pleuritis or B) pericarditis 7. Renal disorder A) proteinuria>0.5g/24hour or 3+ or B) cellular casts 8. Neurological disorder: A) seizures or B) psychiatric disorder (having excluded other causes, e.g. drigs) 9. Haematological disorder: A) haemolytic anaemia or B) leucopenia or C) thrombocytopenia 10. Immunologic disorder: A) positive LE cells or B) raised anti-native DNA antibdy binding or C) anti-Sm antibody or D) false positive serological test for syphilis. 11. Positive antinuclear antibody:

1. Malar rash
This is a "butterfly-shaped" red rash. It is present over the cheeks below the eyes and across the bridge of the nose. It may be a flat or a raised rash. The rashes are made worse by sun exposure.

2. Discoid lupus
These are red, raised patches with scaling of the overlying skin. Atrophic scarring may occur in older lesions.

3. Photosensitivity
4. Oral Ulcers
Painless sores in the nose or mouth.

5. Arthritis
Nonerrosive arthritis. Involve two or more pripheral joints. Characterized by tenderness, swelling and effusion.

6. Renal disorders
It includes Persistent proteinuria > 0.5 gm/dl or > 3, if quantitation not Cellular casts

performed

7. Serositis
It can be in two forms. Pleuritis Pericarditis

 Pleuritis
History of pleuritic pain or rub heard by a physician or evidence of pleural effusion.

 Pericarditis
Documented by ECG or rub or evidence of pericardial effusion.

8. Neurological disorders
It include seizures and psychosis in the absence of offending drug and known metabolic derangements.

9. Hematologic disorders
It includes Hemolytic anemia Leukocytopenia (<4.0*109/L) Lymphopenia (<1.5*109/L) Thrombocytopenia (<100*109/L)

10. Immunological disorders

It includes the presence of Anti DNA antibody Anti Sm antibody Anti phospholipid antibody

11.Antinuclear antibody

Autoantibodies in SLE
Targets of autoantibodies are Nuclear components ANA, anti-dsDNA, antibodies to extracellular nuclear antigen (ENA, anti-Sm, anti-RNP, anti-Jo1) Cytoplasmic components anti-SSA, anti-SSB Cells surface antigens lymphocytotoxic antibodies, anti-neurone antibodies, antierythrocyte antibodies, anti-platelet antibodies

Anti-nuclear antibodies
They are directed against nuclear antigens and are divided into four categories. 1. Against DNA 2. Against histone 3. Against non histone proteins bound to RNA 4. Against nucleolar antigens

Detection of Antinuclear Antibodies

It is done by indirect immunoflourescence technique. Four type of patterns are detected.

1. Homogenous pattern
This reflects antibodies to chromatin, histones, and occasionally DNA.

2. Rim (peripheral ) pattern This indicates antibodies to DNA.

3. Speckled pattern
This is most commonly observed and least specific pattern. It shows presence of antibodies to non-DNA nuclear constituents. Examples: Sm antigen, SS-A and SS-B reactive antigens.

4. Nucleolar pattern
It represents antibodies to RNA.

Secondary Antiphospholipid syndrome

It is characterized by recurrent arterial and /or venous thrombosis, fetal loss and thrombocytopenia. High titer of Antiphospholipid antibody can be found in APS patients. Antiphospholipid antibody: are present in 40-50% of lupus patients. are directed aginst epitopes of plasma proteins. These proteins include prothrombin, annexin V, B2-glycoprotein I, protein S and protein C. Some of these antibodies are termed as lupus anticoagulant.

Etiology of SLE
1. 2. 3. Three factors are involved in SLE. Genetic Factors Immunological factors Encironmental factors

Genetic factors
There is a high rate of concordance in monozygotic twins (25%) versus dizygotic twins (1% to 3%). Family members have an increased risk of developing SLE, and up to 20% of clinically unaffected first-degree relatives may reveal autoantibodies. Some lupus patients (about 6%) have inherited deficiencies of complement components. there is a positive association between SLE and class II HLA genes, particularly at the HLA-DQ locus.

Immunological factors
Defective elimination of self reactive B cells. Escape tolerance of CD4+ helper T cells. Increased production of Autonuclear antibodies by B cells. Exposure to type 1 interferons.

Environmental factors
Ultraviolet (UV) radiation (sun exposure) exacerbates the lesions of SLE. UV radiation causes apoptosis of host cells, leading to an increased burden of nuclear fragments. An example of nongenetic (e.g., environmental) variables in initiating SLE is the occurrence of a lupus-like syndrome in patients receiving certain drugs, including procainamide and hydralazine. Most patients treated with procainamide for more than 6 months develop ANAs, with clinical features of SLE appearing in 15% to 20% of them. Sex hormones also seem to exert an important influence on the occurrence of SLE.