systemic lupus erythematosus manal al mashaleh. systemic lupus erythematosus autoimmune multisystem...
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SYSTEMIC LUPUS ERYTHEMATOSUS
Manal Al Mashaleh
SYSTEMIC LUPUS ERYTHEMATOSUS
Autoimmune multisystem disease characterized by widespread inflammation and production of autoantibodies
This means wide spectrum of presentations
EPIDEMIOLOGY
Age:peak 15-40 years but any age can be affected
Sex :more women affected ,10:1 during childbearing age
Clinical features
General Severe fatigue Fever Weight loss Anorexia Lymphadenopathy
Dermatological features
MALAR RASH
Fixed erythema, flat or raised, over the malar eminences
Tending to spare the nasolabial folds
30-60 %
MALAR RASH
Photosensitivity
Rash over the sun exposed areas.Face,neck and V shaped area of chest.See rash varies in severity depending on exposure.Less under the orbit protected areas.
Discoid lupus
Erythematous hyper pigmented margins and flat scarred hypo pigmented centers
This can be seen in SLE and pure cutaneous lupus
DISCOID RASH
Erythematous raised patches with adherent keratotic scaling and follicular plugging
Atrophic scarring may occur in older lesions
Subacute Cutaneous Lupus
Acute Cutaneous: Malar RashNote Sparing of Nasolabial Folds
Chronic Cutaneous:DiscoidNote Scarring, Hyperpigmentation
Livedo Reticularis
SLE - VASCULOPATHY
Small vessel vasculitis
Raynaud’s phenomenon
Antiphospholipid antibody syndrome
Alopecia
Oral lesions of SLE
Erythema of hard and soft palate, papules ,vesicles and petechiae
Erythematous rash of the tongue.
Oral Ulcers
Oral or nasopharyngeal ulceration, usually painless, observed by physician
SLE ARTHROPATHY
Non erosive arthritis Hand may show
diffuse soft tissue swelling,ulnar deviation,swan neck deformity,MCP subluxation.
Musculoskeletal
Synovitis-90% patients, often the earliest sign
Osteoporosis From SLE itself and therapy (usually steroids)
Osteonecrosis (avascular necrosis) Can occur with & without history of steroid
therapy
Ocular
ConjunctivitisPhotophobiaMonocular blindness transient or permanentBlurred visionCotton-Wool spots on retina due to occlusion retinal blood vessels
Serositis
Pleuritis : convincing history of pleuritic pain ,pleural
rub heard by a physician or evidence of pleural effusion or
Pericarditis: documented by ECG ,pericardial rub or
evidence of pericardial effusion
Pulmonary
Pleuritis the most common 30% in life time of SLE patient
Peumonitis, pulmonary embolism, pulmonary hypertension
Pulmonary hemorrhage: 50 % mortality with treatment
Cardiovascular
Pericarditis the most common Aortic insufficiency the most common
valvular lesion Endocarditis antibiotic prophylaxis indicated for
dental and surgical procedures Accelerated atherosclerosis with 10
times higher mortality from myocardial infarction from age and sex matched
Lupus - Endocarditis
Noninfective thrombotic endocarditis involving mitral valve in SLE.
Note nodular vegetations along line of closure and extending onto chordae tendineae
(libman_sacks syndrome).
Renal
50% of all lupus patients will have kidney involvement during their life
of these, 50 % will have serious kidney disease
Persistent proteinuria greater than 0.5 grams per day or greater than 3+
Cellular casts--may be red cell, hemoglobin, granular, tubular, or mixed
Impaired kidney function Lupus nephritis predict out come
(prognosis) Major cause of mortality
How does lupus damage the kidneys?
Autoantibodies are formed against antigens in the glomerulus basement membrane
Circulating immune complexes bind to the basement membrane of the glomeruli
These result in inflammation of the glomeruli (glomerulonephritis)
How does lupus damage the kidneys?
The basement membrane is damaged by the inflammation
Appearance of protein, white and red blood cells and ‘casts’ in the urine
Low albumin levels in the blood resulting in leakage of fluid from the vessels into the tissues (edema)
Accumulation of waste proteins (uremia) Hypertension
Do I really need a biopsy?
Most likely - YES
Kidney biopsies are important to dictate how to treat predict how long to treat predict the chance for kidney function recovery
The biopsy helps determine treatment
Inflammation can occur: Diffuse Focal
Types of Lupus Nephritis
6 classes based on a WHO classification
Class I (normal) mild
Class II (mesangial) mild
Class III (focal proliferative glomerulonephritis) moderately severe
Class IV (diffuse proliferative glomerulonephritis) severe Class V (membranous glomerulonephritis) Class VI (glomerulosclerosis) irreversible changes
Neurological
Seizures & Psychosis
in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance
Cranial nerve lesions
Gastrointestinal & Hepatic
Uncommon SLE manifestations Severe abdominal pain syndromes in SLE often
indicate mesenteric vasculitis, resembling medium vessel vasculitis (PAN)
Diverticulitis may be masked by steroids Hepatic abnormalities more often due to therapy
than to SLE itself
Laboratory Findings
Complete blood count Anemia Leukopenia Lymphopenia Thrombocytopenia
Complete blood count
Hemolytic anemia--with reticulocytosis Leukopenia :less than 4,000/mm total on 2
or more occasions
Lymphopenia: less than 1,500/mm on 2 or more occasions
Thrombocytopenia: less than 100,000/mm, in the absence of offending drugs
Immunological findings
ANA - 95-100%-sensitive but not specific for SLE
Anti -ds DNA-specific(60%) for SLE, but positive to other non lupus conditions
4 RNA associated antibodies Anti-Sm (Smith) Anti Ro/SSA-antibody Anti La/SSB-antibody Anti-RNP
Antiphospholipid antibody Lupus anticoagulant Anti-B2 glycoprotein 1 AB Anti-cardiolipin
Depressed serum complement ESR , CRP
CXR
Pleural effusion and pleural thickening Pericardial effusion,cardiomegaly Lung infiltrate from infection,pulmonary
embolism,Peumonitis,Interstitial lung disease or hemorrhage
CLASSIFICATION
1. Malar rash 2. Discoid rash3. Photosensitivity4. Oral ulcers5. Arthritis6. Serositis7. Renal disease. 8. Neurologic disease.
9. Hematologic disorders: -Hemolytic anemia -Leukopenia (Lymphopenia) -Thrombocytopenia 10. Immunologic
abnormalities: -Anti-ds- DNA -Anti- Sm -Antiphospholipid -False +ve VDRL
11. Positive ANA
CRITERIA FOR CLASSIFICATION OF SLE
CLASSIFICATION CRITERIA
Must have 4 of 11 for Classification Sensitivity 96% Specificity 96%
Not all “Lupus” is SLE Discoid Lupus Drug induced lupus Subacute Cutaneous Lupus
DIFFERENTIAL DIAGNOSIS
Rheumatic: RA, Sjogren’s syndrome, systemic sclerosis, dermatomyositis
Nonrheumatic: HIV, endocarditis, viral infections, hematologic malignancies, vasculitis, ITP, other causes of nephritis
LUPUS RELATED SYNDROMES
Drug Induced Lupus Classically associated with
hydralazine, isoniazid, procainamide Male:Female ratio is equal Nephritis and CNS abnormalities rare Normal complement and no anti-DNA
antibodies Symptoms usually resolve with
stopping drug
LUPUS RELATED SYNDROMES
Antiphospholipid Syndrome (APS) Hypercoagulability with recurrent thrombosis of either
venous or arterial circulation Thrombocytopenia-common Pregnancy complication-miscarriage in first trimester Lifelong anticoagulation warfarin is currently
recommended for patients with serious complications due to common recurrence of thrombosis
Antiphospholipid Antibodies Primary when present without other SLE feature. Secondary when usual SLE features present
SLE – treatment I.
Mild cases (mild skin or joint involvement): NSAID, local treatment, hydroxy-chloroquine
Cases of intermediate severity (serositis, cytopenia, marked skin or joint involvement):
corticosteroid , azathioprine, methotrexate
SLE – treatment II.
Severe, life-threatening organ involvements (carditis, nephritis, systemic vasculitis, cerebral manifestations):
high-dose intravenous corticosteroid + iv. cyclophosphamide + in some cases: plasmapheresis or iv. immunoglobulin, or, instead of cyclophosphamide: mycophenolate mofetil
Some cases of nephritis (especially membranous), myositis, thrombocytopenia cyclosporine
TREATMENT
Antiphospholipid Syndrome Anticoagulation with warfarin (teratogenic) subcutaneous heparin and aspirin is usual
approach in pregnancy Lupus and Pregnancy
No longer “contraindicated” No changes in therapy other than avoiding
fetal toxic drugs Complications related to renal failure,
antiphospholipid antibodies
TREATMENT
ESR, CRP probably useful as general markers of disease activity
Complement and anti-DNA antibodies may correlate to disease activity
PROGNOSIS
Unpredictable course 10 year survival rates exceed 85% Most SLE patients die from infection,
probably related to therapy which suppresses immune system
Recommend smoking cessation, yearly flu shots, pneumovax q5years