SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
Dr. Angelo Smith M.DWHPL
Autoimmune disease that affects multisystems
1.5 million cases of lupus Prevalence of 17 to 48 per
100,000 population Women > Men - 9:1 ratio 90% cases are women African Americans > Whites Onset usually between ages of
15 and 45 years, but Can occur in childhood or later
in life
Clinical Manifestations
Ranges from a relatively mild disorder to rapidly progressing, affecting many body systems.
Chronic with relapsing and remitting course.
Most commonly affects the skin / muscles, lining of lungs, heart, nervous tissue, and kidneys
Etiology
Etiology is unknown Most probable causes
Genetic influence Hormones Environmental factors Certain medications
Pathogenesis of SLE
Pathophysiology
Autoimmune reactions directed against constituents of cell nucleus, DNA
Antibody response related to B and T cell hyperactivity
General symptoms
The most common symptoms listed as initial complaints are fatigue, fever, and weight loss.
Fever: fever secondary to active disease was recorded from 50% to 86%. No fever curve or pattern is characteristic. It can be difficult, but very important to distinguish the fever of SLE from that caused by complicating infections.
Clinical Manifestations
Infection Increased susceptibility to infections
Fever should be considered serious
Infections such as pneumonia are a common cause of death
Fatigue is common in patients with SLE, especially during periods of disease activity. It is also often the only symptom that remains after treatment of acute flares.
Low grade fever, anemia, or any source of inflammation can result in fatigue.
Clinical Manifestations
Dermatologic Cutaneous vascular lesions Photosensitivity Butterfly rash (Malar Rash) Oral/nasopharyngeal ulcers Alopecia
Malar Rash
Discoid Rash
Maculopapular eruption
Oral Ulcers
Raynaud’s phenomenon is commonly found in lupus. It lack specificity.
(a triphasic reaction of distal digits to cold or emotion, in which the skin colour changes from white to blue to red)
Vasculitic skin lesion
Clinical Manifestations
Musculoskeletal (jaccoud arthropathy) Polyarthralgia with morning stiffness A vascular necrosis Arthritis bilateral – hands / wrists /
kneesSwan neck fingersUlnar deviationSubluxation with hyperlaxity of joints
Avacular necrosis of bone
Clinical Manifestations
Cardiopulmonary Tachypnea Pleurisy Dysrhythmias Accelerated CAD Pericarditis
Pulmonary manifestations Pleurisy it is the most common
manifestation of pulmonary involvement of SLE. The volume of pleural effusions usually is small to moderate and maybe unilateral or bilateral. Large pleural effusion are uncommon. It usually exudative in character.
Pleural effusions may also occur in SLE patients with nephrotic syndrome, infection, cardiac failure.
Lung 1) acute lupus pneumonitis:
fever, dyspnea, cough with scanty sputum, hemoptysis, tachypnea and pleuritic chest pain.
2) pulmonary hemorrhage 3) chronic diffuse interstitial
lung disease. the diagnosis should not be made
until infectious processes such as viral pneumonia, tuberculosis, and other bacterial, fungal and pneumocystis carinii infection have been completely excluded.
Cardiovascular manifestations
Pericarditis is the most common cardiac manifestation of SLE.
Myocarditis (the clinical features of lupus myocarditis resembles that of viral myocarditis)
Libman-Sacks endocarditis and valvular disease
Hypertension, cardiac failure
SLE can be associated with endocarditis.
Shown here is Libman-Sacks endocarditis in which there are many flat, reddish-tan vegetations spreading over the mitral valve and chordae.
Clinical Manifestations
Renal Lupus nephritisRanging from mild proteinuria to glomerulonephritis
Primary goal in treatment is slowing the progression
Haematuria Proteinure (>0.5g protein/d or
3+ ) Cast
Lupus nephritis
Class I Minimal mesangial Normal light microscopy; abnormal electron microscopy
Class II Mesangial proliferative
Hypercellular on light microscopy
Class III Focal proliferative <50% glomeruli involved
Class IV Diffuse proliferative >50% glomeruli involved; segmental/global
Class V Membranous Predominantly nephrotic disease
Class VI Advanced sclerosing Chronic lesions and sclerosis
Clinical Manifestations
Nervous system Generalized/focal seizures Peripheral neuropathy Cognitive dysfunction
DisorientationMemory and reasoning deficitsPsychiatric symptoms – severe depression / psychosis
Clinical Manifestations
Red blood cells a normochromic, normocytic
anemia is frequently found in SLE. They appears to be related to chronic inflammation, drug-related haemorrhage.
haemolytic anemia as detected by the Coombs’ test is the feature of SLE.
on rare occasion, a serum antibody may be produced which impairs red cell production.
Platelets thrombocytopenia (<100*109/L)
appears to be mediated by anti-platelet antibodies or/and anti-phospholipid antibodies.
White blood cell leucopenia (<4.0*109/L), its cause
is probably a combination of destruction of white cells by autoantibodies, decreased marrow production, increased or marginal splenic pooling, and complement activation.
it should also noted that the immunosuppressive drugs used in the treatment of SLE may cause a marked leucopenia.
Gastrointestinal and hepatic manifestation
Esophagitis, dysphagia, nausea, vomiting: (drug related in most cases)
Chronic intestinal pseudo-obstruction, mesenteric vasculitis, protein-losing enteropathy
Pancreatitis Lupus hepatitis
Diagnostic Studies
No specific test SLE is diagnosed primarily
on criteria relating to patient history, physical examination, and laboratory findings
On examination Constitutional – lymphadenopathy,
hepatosplenomegaly Musculoskeletal – Jaccoud
arthropathy Dermatologic - capillaroscopy Renal Neuropsychiatric Cardiopulmonary – friction rubs,
pulmonary embolism, Libman-Sacks endocarditis
GIT – peritonitis, pancreatitis, mesenteric vasculitis
Diagnostic Studies
Antinuclear antibodies ANA and other antibodies indicate
autoimmune disease Anti-DNA and anti-Smith antibody
tests most specific for SLE LE prep can be positive with other
rheumatoid diseases ESR & CRP are indicative of
inflammatory activity
Radiological studies
Joint x-rays: no erosions, periarticular osteopenia + soft tissue swelling
CXR/CT chest: interstitial lung disease, pneumonitis, pulmonary emboli, alveolar hemorrhage
CTBrain or Brain MRI ± angiography: lupus white matter changes, vasculitis or stroke
Echo: pericardial effusion, pulmonary hypertension or Libman-Sacks endocarditis
Additional work-up
- Serum cr. and albumin- CBC w/ diff- U/A- ESR- Complement levels- Renal profile if warranted
Invasive procedures
LP – nonspecific ↑cells + protein, ↓ glucose
Renal biopsy – prognosis and Rx
Skin biopsy
Diagnostic criteria
American College of Rheumatology
4/11 criteria (sens 85%, specif 95%)
“SOAP BRAIN MD” Serositis – heart, lung,
peritoneum Oral ulcers – painless esp
palate Arthritis – non-erosive Photosensitivity
Diagnostic criteria Blood disorders - ↓RBC (Coombs +),
PLT, WCC, Lymphocytes Renal involvement – proteinuria /±
casts ANA – titer > 1:160 Immunologic phenomena – LE cells,
anti-dsDNA Ab, anti-Sm Ab, antiphospholipid Ab, false WR +
Neurological disorders – seizures/ psychosis
Malar rash – cheeks + nasal bridge Discoid rash – rimmed with scaling,
follicular plugging
Treatment Treatment plans are based on patient age,
sex, health, symptoms, and lifestyle and on disease severity Fever, skin, musculoskeletal and serositis =
milder disease CNS and renal involvement – aggressive Rx
Goals of treatment are to: -prevent flares -treat flares when they occur -minimize organ damage and
complications
Collaborative Care
Drug therapy NSAIDs Antimalarial drugs Steroid-sparing drugs Corticosteroids Immunosuppressive drugs
Conservative management
For those w/out major organ involvement. NSAIDs: to control pain, swelling, and
fever Caution w/ NSAIDS though. SLE pts are at
increased risk for aseptic meningitis Antimalarials: Generally to treat fatigue
joint pain, skin rashes, and inflammation of the lungs
Commonly used: Hydroxycholorquine Used alone or in combination with other
drugs
Corticosteroids (Mainstay of SLE treatment)
To rapidly suppress inflammation Usually start with high-dose IV
pulse and convert to PO steroids with goal of tapering and converting to something else.
Commonly used: prednisone, hydrocortisone, methylprednisolone, and dexamethasone
Immunosuppressives Primarily for CNS/renal involvement Mycophenolate mofetil (cellcept) Azathioprine (imuran): requires several
months to be effective, effective in smaller percentage of patients
MTX: for treatment of dermatitis and arthritis, not life-threatening disease
Cyclosporine: used in steroid-resistant SLE, risk of nephrotoxicity
Cyclophosphamide (cytoxan) Almost all trials performed on patients with nephritis
Differential diagnosis
Drug induced lupus erythematosis
Vasculitis Leukemia HIV Multiple sclerosis Parvovirus or other viral
infections
Prognosis
Benign to rapidly progressive Better for isolated skin + musculoskeletal
disease vs renal and CNS Death rate 3X age-comparable general
population
Mortality
Nephritis (most within 5 yrs of symptoms) Infectious (active SLE + Rx – most common) CVS disease (50X more MI than other woman) Malignancy (chronic inflammation + Rx)
SLE disease activity index (SLEDAI)Clinical feature score
seizure , psychosis , organ brain syndrome 8
visual disturbance, cranial nerve disorder 8
lupus headache, cerebrovascular accidents, 8
vasculitis 8
arthritis 4
myositis 4
urinary casts, hematuria, proteinure, pyuria 4
rash, alopecia, mucosal ulcers, 2
pleurisy, pericarditis 2
low complement, increased DNA binding 2
fever 1
thrombocytopenia, leucopenia 1
Other therapy
Plasma exchange Intravenous Immunoglobulin Stem cell transplantation Immune therapy ( anti-IL10,
anti-CD20, and immune tolerance therapy)
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