systemic lupus erythematosus (s l e) - كلية الطب · 2019-10-29 · systemic lupus...

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Systemic Lupus Erythematosus (S L E)


.Epidemiology:-

- varies from country to country.

1/250 ----- African American women

1/1000------------1/10.000

F: M -------9:1

Peak age onset 20-40


Aetiology

- Autoimmune: cause unknown but there are several predisposing

factor's

* Heredity: - Monozygotic twins 25%

- Dizygotic 3%

- First degree 3%

* Genetic: HLA-B3----DR3---DR2

* Complement: -C4, C2

* Sex Hormones:

* Immunological Factor's "loss of self-tolerance"

. B cell activation

. Failure to remove immune-complexes

. Impaired T cell regulation of the immune response

Environmental trigger's

Drug's:- Ultraviolet light-viral


Clinical Feature

1-General Features

. Fever 50%

. Malaise and tiredness


2- The joint and muscles

. Arthritis > 90%

. RA like symptom's(symmetrical, small joint arthrlgia)

. Non erosive

. Rarely Deformity " Jaccoudarthropathy"

. Aspetic necrosis

. Myalgia 50% --- true myositis rare.


Systemic lupus erythematosus: Jaccoud’s arthropathy

(clinical and radiograph)


3- The Skin 75%

Acute : . Butterfly

.photosensitivity

.Raynoud

Sub acute: subacute cutanous lupus erythematosus

- Photosensitive ,nonscaring rash that occurs in both

psoriasform and an unnular / poly cyclic form

Chronic : discoid lupus, face, neck, scalp and forearms

.Maculopapular rash

.Alopecia

.Vasculitis


Systemic lupus erythematosus: butterfly rash


Systemic lupus erythematosus: malar rash, face


Mixed connective tissue disease: nasal septal perforation


Subacute cutaneous lupus erythematosus


Systemic lupus erythematosus: alopecia, scalp


4- The Lung's --50%

. Pleurisy---pleural effusion

. pheumonitis and atelectasis

. Shrinking lung syndrome

. Pulmonary fibrosis

. lung hemorrhage


5- Heart and Cardio vascular system-----25%

. Pericarditis

. Myocarditis (arrythemia)

. Aortic value lesion---cardiomyopathy

. Endocarditis (libman-sacks syndrome)

. Vascuitis

. Arterial and vein thromboses


Systemic lupus erythematosus: Libman-Sacks endocarditis

(gross specimen)


Systemic lupus erythematosis: vasculitis, hands


Systemic lupus erythematosus: digital gangrene, hands


Vasculitis: fingers


Systemic lupus erythematosus: bullous rash


6-The Kidneys-----50%

- Lupus nephritis: - . Type 1 : N

. Type 11 mesangial glomero nephritis

. Type 111 focal proliferative glomero nephritis

. Type 1V diffuse GN

. Type V membranous GN

. Type V1 advance sclerosing lesion

- Renal vein thrombosis


Systemic lupus erythematosus: classification of nephritis

Normal glomeruli

a) Nil by all techniques

b) Normal by light but deposits on EM or IF

Mesangial glomerulonephritis

Focal glomerulonephritis

Diffuse glomerulonephritis

Diffuse membranous glomerulonephritis

Advanced sclerosing glomerulonephritis


7- Nervous System

. Depression (psychosis)

. Epilepsy

. Migraines

. Encephalopathy

. Coma

. Meningitis

. Stroke

. Mono neuritis multiplex

. Peripheral and cranial Neuropathy


Systemic lupus erythematosus: nervous system

manifestations

Seizures Ataxia

Headache Rigidity, tremor

Stroke syndromes Chorea

Subarachnoid hemorrhage Hemiballismus

Transverse myelitis Coma

Multiple sclerosis-like disorder Dementia

Optic neuritis Psychiatric disorders

Aseptic meningitis

Peripheral neuropathy

Cranial neuropathy

Mononeuritis multiplex

Guillain-Barré syndrome


8- The Eyes:

. Retinal vasculitis Can cause infarcts (cytoid bodies)

. Episcleritis conjunctivitis

. Optic neuritis

. Secondary siogren 15%


Systemic lupus erythematosus: cytoid bodies


Systemic lupus erythematosus: retinal occlusive disease


9-The Gastro Intestinal System:

. Mouth ulcers

. Mesenteric vascuitis

.Liver enlargement

. Splenomegaly is found in 10% of adult30% of children


Systemic lupus erythematosus: bullous lesions, palate


Investigation

.C B C - Leucopenia

- Lymphopenia

- Thrombocytopenia

- Auto immune hemolytic anemia

-E S R raised

- C R P normal

.R F positive 25%

. C3, C4 low

. Anti cardiolipin antibody are present in 35-45% of the patients

. Lupus anti coagulant


Autoantibody-disease associations: SLE and drug-induced

lupus

Antigen SLE Drug-Induced LE

dsDNA 40% No

ssDNA 70% 75%-80%

Histone 70% >95%

Sm antigen 30% No

Nuclear RNP 30% No

Ribosomal RNP 10%

SS-A/Ro 35% No

SS-B/La 15% No


Diagnostic imagine

- CTS Brain: . Infarction

. Hemorrhage

- MRI: White matter


Systemic lupus erythematosus: brain (MRI)


Drug-induced lupus: definite drug associations

Hydralazine

Procainamide

Minocycline

Chlorpromazine

Isoniazid

Penicillamine

Methyldopa

Interferon-alpha


Drug-induced lupus: possible drug associations

Anticonvulsants

Quinidine

Propylthiouracil

Sulfonamides

Lithium

Beta-blockers

Nitrofurantoin

Sulfasalazine

Diltiazem

Hydrazine

Interferon-gamma

TNF inhibitors


Preliminary classification criteria for antiphospholipid

syndrome

APS is present if more than 1 clinical and 1 lab criteria met.

Vascular thrombosis

> 1 arterial, venous, or small-vessel thrombosis and

Thrombosis confirmed by imaging or Doppler or histopathology and

Without evidence of inflammation in vessel wall on histpathologic confirmation

Pregnancy morbidity

> 1 fetal death > 10th week gestation or

> 1 premature birth > 34th week with preeclampsia or placental insufficiency or

> 3 consecutive spontaneous abortions

< 10th week gestation

Laboratory criteria

Anticardiolipin antibody IgG or IgM present medium or high titers > 2 times at

least 6 weeks apart or

Lupus anticoagulant present > 2 times at least 6 weeks apart


Antiphospholipid antibody syndrome: clinical

manifestations

Arterial thrombosis

Venous thrombosis

Valvular abnormalities

Pregnancy loss and infertility

Livedo reticularis

Neurologic complications

cerebrovascular thrombosis

chorea

Catastrophic syndrome

Thrombocytopenia


Antiphospholipid antibody syndrome: tests

Partial thromboplastin time (PTT)

Lupus anticoagulant

Anticardiolipin antibodies by ELISA

Anti-B2-glycoprotein 1 antibody

False-positive biologic test for syphilis

Platelet count

Antinuclear and anti-DNA antibodies


Management:

. Arthrlgia, arthritis, fever and serositis (mild disease)

- NSAIDS

- Antimalaria . Chloroquine

. Hydroxychloroquine

- Small or medium dose of steriod


. Severe disease (Lupus nephritis –vascuitis CNS or blood dyscrasias)

- Corticosteroid orally or pulse therapy

- Immunosuppression therapy

. Azathioprine

. cyclophosphamide

. mycophenolate

- Depleted B lymphocyte anti CD20 "Rituximab“

-APS .anti coagulant


. Infection: S L E increased risk of infection, influenza

vaccine is recommended yearly

. Cancer:S L E are at increased risk of some forms of

cancer (colonoscopies, pap smears, mammograms

should be enforced)

. Osteoperosis (steroid induced): CA+vit D

. Hypertension + hypercholesremia : must be

controlled.


Pregnancy and Contraception

- Pregnancy in lupus considered high risk

- The incidence of preclampsia is increased

- If the pregnancy woman has anti RO or anti LA antibody -2-5% risk of

congenital heart block lupus rash in baby

- four chamber fetal cardiac ultrasound are recommended weekly from

the 16 through 32 week of gestation

- Barrier methods of contraception rather than pill are advisable.

systemic lupus erythematosus (s l e) - كلية الطب · 2019-10-29 · systemic lupus...

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