systemic lupus erythematosus (s l e) - كلية الطب · 2019-10-29 · systemic lupus...
TRANSCRIPT
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic Lupus Erythematosus (S L E)
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
.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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Clinical Feature
1-General Features
. Fever 50%
. Malaise and tiredness
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: Jaccoud’s arthropathy
(clinical and radiograph)
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: butterfly rash
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: malar rash, face
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Mixed connective tissue disease: nasal septal perforation
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Subacute cutaneous lupus erythematosus
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: alopecia, scalp
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
4- The Lung's --50%
. Pleurisy---pleural effusion
. pheumonitis and atelectasis
. Shrinking lung syndrome
. Pulmonary fibrosis
. lung hemorrhage
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
5- Heart and Cardio vascular system-----25%
. Pericarditis
. Myocarditis (arrythemia)
. Aortic value lesion---cardiomyopathy
. Endocarditis (libman-sacks syndrome)
. Vascuitis
. Arterial and vein thromboses
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: Libman-Sacks endocarditis
(gross specimen)
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosis: vasculitis, hands
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: digital gangrene, hands
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Vasculitis: fingers
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: bullous rash
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
7- Nervous System
. Depression (psychosis)
. Epilepsy
. Migraines
. Encephalopathy
. Coma
. Meningitis
. Stroke
. Mono neuritis multiplex
. Peripheral and cranial Neuropathy
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
8- The Eyes:
. Retinal vasculitis Can cause infarcts (cytoid bodies)
. Episcleritis conjunctivitis
. Optic neuritis
. Secondary siogren 15%
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: cytoid bodies
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: retinal occlusive disease
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
9-The Gastro Intestinal System:
. Mouth ulcers
. Mesenteric vascuitis
.Liver enlargement
. Splenomegaly is found in 10% of adult30% of children
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: bullous lesions, palate
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Diagnostic imagine
- CTS Brain: . Infarction
. Hemorrhage
- MRI: White matter
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: brain (MRI)
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Systemic lupus erythematosus: brain (MRI)
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Drug-induced lupus: definite drug associations
Hydralazine
Procainamide
Minocycline
Chlorpromazine
Isoniazid
Penicillamine
Methyldopa
Interferon-alpha
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Drug-induced lupus: possible drug associations
Anticonvulsants
Quinidine
Propylthiouracil
Sulfonamides
Lithium
Beta-blockers
Nitrofurantoin
Sulfasalazine
Diltiazem
Hydrazine
Interferon-gamma
TNF inhibitors
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Management:
. Arthrlgia, arthritis, fever and serositis (mild disease)
- NSAIDS
- Antimalaria . Chloroquine
. Hydroxychloroquine
- Small or medium dose of steriod
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
. 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
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
. 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.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.