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SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
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DEFINITION Systemic lupus erythematosus is a multi-system autoimmune disease which is characterized by production of antinuclear antibodies. SLE is a prototype of human immune complex disease.
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INCIDENCE AND PREVALENCE
Incidence – 4 to 7 new cases per 100,000 per year Prevalence – 1:2,000 population Age – Peak onset 15 to 45 years old Female:Male ratio – 4 to 10 :1 Black:White ratio – 3:1 Prevalence in all women ages 15 to 64 is 1/700 Prevalence in black women of same age is 1/245 Other populations commonly affected: Hispanics Chinese
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Pathogenesis and pathophysiology
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Familial/Genetics/HLA
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Common Clinical Manifestations Of SLE
Manifestation Approximate Frequency Arthralgia/Myalgia Fatigue Fever Central Nervous System Skin Changes Anemia Adenopathy Gastrointestinal Renal Changes Pericarditis Leukopenia Pleural Effusion Arthritis
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1982 CLASSIFICATION CRITERIA SLE
Serositis Oral ulcers Arthritis Photosensitive rash Blood dyscrasias Renal disorder ANA Immunologic disorder Neurologic disorder Malar rash Discoid rash ** SLE if 4 of 11 present serially or simultaneously
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SKIN INVOLVEMENT ACUTE SUBACUTE CHRONIC
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MALAR RASH Fixed erythema Flat or raised Spares the nasolabial folds
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MALAR RASH
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Lupus band test
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DLE Plugging atrophy scar telangiectasia erythematous raised rim
above trans-nipple line
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DISCOID RASH Raised Patches Adherent Keratotic Scaling
Follicular Plugging Older Lesions May Cause Scarring
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Balloon degeneration of epidermal cells
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LE-nonspecific Skin Lesions
Panniculitis Urticarial lesions (5-10%) Vasculitic lesions Raynaud’s phenomenon (50%)
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Lupus Paniculitis
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OTHER SLE RASHES Palmar Erythema and Rash
Subacute Cutaneous Lupus Erythematosus (SCLE) Bullous Lupus Dermatitis Neonatal Lupus Raynaud’s Phenomenon
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Subacute Cutaneous Lupus Erythematosus (SCLE)
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Bullous Lupus Dermatitis
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Neonatal Lupus
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ORAL ULCERS Can affect all mucosal surfaces
Painless (which distinguishes them from aphthous ulcers)
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ORAL ULCER
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ALOPECIA
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SEROSITIS Pleuritis, Pericarditis or Peritonitis.
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ARTHRITIS Clinically indistinguishable from RA Nonerosive Symmetric
Inflammatory > 2 peripheral joints
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Arthritis (Non-erosive, usually symmetric)
Bone & Joints in SLE Arthralgia Arthritis (Non-erosive, usually symmetric) Nodules (10%) Tenosynovitis Jaccoud’s joint Osteonecrosis Osteoporosis
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Jaccoud’s Arthropathy
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PHOTOSENSITIVITY Skin Rash From UV irradiation
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BLOOD DYSCRASIAS Hemolytic anemia with reticulocytosis or
Leukopenia- <4,000 WBC on > 2 occasions or Lymphopenia- <1,500 on 2 occasions or Thrombocytopenia- <100,000
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RENAL DISORDER Persistent proteinuria > 0.5 grams/day or
Cellular casts or > 10 RBC/HPF
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LUPUS RENAL HISTOLOGY WHO designation:
I: Normal light microscopy, but immuo globulin and /or complement deposits II: Mesangial Lupus Nephritis III: Focal Proliferative Glomerulonephritis IV: Diffuse Proliferative Glomerulonephritis V: Membranous Nephritis
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RENAL INVOLVEMENT 1.Msangial Glumerolonefritis(2)
2.Focal Proliferative Glumerolonephritis(3) 3.Diffuse Proliferative Glumerolonephritis(4) 4.Memberanous Nephritis(5) 5. Glumerolosclerosis(6) 7.Tubulointerstitial Nephritis 8.Infection 9.Drug-Induced Nephritis
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Lupus Nephritis: Manifestations
Proteinuria: ~100% Nephrotic syndrome: 50% Granular casts: 30% Red cell casts: 10% Microscopic hematuria: 80% Macroscopic hematuria: 1-2% Reduced renal function: 40-80% Hypertension: 15-50%
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Class II: Pure Mesangial
Mild to moderate proteinuria and hematuria.No HTN Little or no evidence of renal insufficiency.
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Class III: Focal Proliferative GN
Less than 50% of glomeruli and less than 50% of each glomeruli are involved. Subendothelial deposits clinically:
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Focal Proliferative Glomerulonephritis
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Class IV: Diffuse Proliferative GN
The most severe form of lupus nephritis. Along with class III, makes >50% of cases subendothelial deposits clinically:
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Diffuse Proliferative Glomerulonephritis
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Class V: Diffuse Membranous GN Subepithelial deposits
5 - 10 % of all • lupus nephritides. Presents • mainly with massive proteinuria. Subepithelial deposits
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Membranous Glomerulonephritis
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Subepithelial deposit in Membraneous GN
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SLE & Respiratory System
Pulmonary Disease Acute lupus pneumonitis Alveolar hemorrhage Chronic ILD Lymphocytic pneumonia Bronchiolitis obliterans Respiratory Muscle Disease Shrinking lung syndrome Pleural disease Pleuritis Upper Airway disease Epiglotitis Subglotic stenosis Laryngeal edema Laryngeal ulceration Cricoarythenoid arthritis Necrotizing vasculitis Vascular Disease Pulmonary hypertension Pulmonary embolism Acute reversible hypoxemia
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GI INVOLVEMENT Peritonitis Vasculitis Pancreatitis
IBD( collagenous colitis)
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NEUROLOGIC DISORDER Seizures Psychosis
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NEUROPSYCHIATRIC MANIFESTATIONS
9.Aseptic Meningitis 10.MS-like Disorder 11.Guillan-Barre Syn. 12.SAH 13.Ataxia 14.Dementia 15.Psychiatric Disorders 1.Headache 2.Seizure 3.Stroke 4.Chorea 5.Neuropathy 6.Vertigo 7.Tinnitus 8.Transverse Myelitis
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CNS LUPUS
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Antinuclear antibodies
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ANTINUCLEAR ANTIBODY Significant titer is > 1:80
Found in 95% SLE patients but also- Interstitial pulmonary fibrosis MCTD PSS Polymyositis Procainamide/Hydralazine/Dilantin RA Sjogrens syndrome Normal population
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ANA - RIM PATTERN
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ANA - HOMOGENOUS
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ANA - SPECKLED
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ANA - NUCLEOLAR
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LE-cell LE-cell is an in-vitro phenomenon where a PMN phagocytoses the antibody-coated nucleus of a cell. The technique is an insensitive way to detect anti-nuclear antibody in a serum specimen.
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Hematoxyllin Body Hematoxyllin body is the in-vivo counterpart of LE cell. It is considered pathognomonic for SLE lesions.
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IMMUNOLOGIC DISORDER anti-ds DNA antibody (anti-native DNA ab) or
anti-SM antibody or antiphospholipid antibody based on either: abnormal serum IgG or IgM anticardiolipin ab or lupus anticoagulant or false positive RPR or VDRL for > 6 months and confirmed by t. Pallidum immobilization or fluorescent treponemal antibody (FTA) absorption test.
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APS IgG ,IgM APL LA assay VDRL hypercoagulable state
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LEIBMAN-SACHS ENDOCARDITIS
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Prognosis Creatinin > 1.4 HTN NS(> 2.6 g/24h) anemia
low C and albomin low socioeconomic state major organ involvement APS+ thrombocytopenia
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Mortality Infection & renal involvement in 1st decade
Thromboembolic phenomena in the 2th.
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Treatment NSAIDs antimalarials sunscreens(SPF >15) androgens (DHEA)
immuno-suppression anti-thrombotics & anti-platelets IvIG BMT
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THERAPY Serositis NSAID Arthritis NSAIDs Blood dyscrasias
Corticosteroids Immunosuppressive Arthritis NSAIDs Hydroxychloroquine Immunosuppressive Drugs Blood dyscrasias
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THERAPY cont. Renal Corticosteroids Neurologic Symptomatic
Immunosuppressive Neurologic Symptomatic Corticosteroids Rash Topical Corticosteroids Systemic Steroids Hydroxychloroquine Immunosuppressive Drugs
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