This document discusses systemic lupus erythematosus (SLE), including its definition, pathogenesis, clinical manifestations, diagnostic criteria, treatment, and monitoring. SLE is an autoimmune disease that can damage any organ system via autoantibodies and immune complexes. It predominantly affects women of childbearing age. Common symptoms include fatigue, arthritis, rashes, and hematological abnormalities. Diagnosis requires meeting criteria from clinical manifestations and immunological tests. Treatment involves managing symptoms, preventing flares, and addressing organ-threatening disease with medications like antimalarials, steroids, immunosuppressants. Monitoring includes checking urine, blood tests, and managing complications like lupus nephritis.

1. SYSTEMIC LUPUS ERYTHEMATOSES
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Dr Jayakrishnan MP
PG Resident, Internal Medicine
MGM Medical College, Indore

2. APPROACH TO POLYARTHRITIS
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3. JOINT ANATOMY
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4. Determine whether musculoskeletal
complaint is
1. Articular or nonarticular.
2. Inflamatory or noninflamotory.
3. Acute or chronic.
4. Localised or widespread
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5. Arthralgia vs Arthritis
• Arthralgia - Pain in joints and is a symptom with an
underlying cause.
Causes: Injury,Infection,allergic reaction to medication,joint
diseases.
• Arthritis - Inflammation of one or more joints.
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6. ARTHRITIS
POLYARTHRITIS
(> 5 joints)
OLIGOARTHRITIS
(2-4 joints)
GOUT
SARCOIDOSIS
LYMES Ds
BECHETS dS
MONOARTHRITIS
(1 joint)
TRAUMA
INFECTION
CRYSTALS
TUMOUR
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7. POLYARTHRITIS
INFLAMMATORY
RHEUMATOID
TYPES
RA, SLE
SSc, DM
VIRAL INFECTIONS
RHEUMATOID
VARIANTS
AS, ReA
Ps A
IBD
DEGENERATIVE
OA
METABOLIC
GOUT
AMYLOIDOSIS
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8. ACR Ad Hoc committee on clinical guidelines, Arthritis rheum 1996; 39: 1
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9. SYSTEMIC LUPUS ERYTHEMATOSES
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10. DEFINITION
• Autoimmune disease with damage to organs and cells
mediated by autoantibodies and immune complexes.
• Mostly affects childbearing age group females.
• Female to male ratio 9:1
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11. INDIAN STATISTICS
• Prevalence is 3/ 100,000 population (Delhi)
• COPCORD Bhigwan Study(Pune) found incidence of
4 /100,000 population/ year.
• Mean age of onset is 24.5 years
• Female to male ratio 11:1
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J Indian Rheumatol Assoc 2002 : 10 : 80 - 96

12. PATHOGENESIS
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13. CLINICAL MANIFESTATIONS
PREVALENCE%
1. Systemic: fatigue ,malaise , fever, wt. Loss ,anorexia 95
2. Musculoskeletal 95
Arthralgia/myalgia 95
Nonerosive polyarthritis 60
3. Hematological 85
Anemia, Leucopenia(<4000), Lymphopenia(<1500)
Thrombocytopenia(<100000)
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14. 4. Cutaneous 80
Photosenstivity, Malar rash, Oral ulcers
Alopecia, Discoid rash.
5. Neurological 60
Cognitive disorders, Seizures, Psychosis
6. Cardiopulmonary 60
Pleurisy, pericarditis, effusions
Myocarditis, endocarditis, CAD
7. Renal 30-50
Proteinuria , Cellular casts, Nephrotic syndrome, ESRD
CLINICAL MANIFESTATIONS
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15. 8. Gastrointestinal 40
9. Thrombosis 15
Venous, Arterial
10. Occular 15
Sicca syndrome,Conjuctivitis, Episcleritis, Vasculitis
CLINICAL MANIFESTATIONS
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16. AUTOANTIBODIES IN SLE
Antibody % Clinical Utility
ANA 98 Best screening test
Anti dsDNA 70 Specific for SLE; Correlate with disease severity
Anti-Sm 25 Specific for SLE
Anti-RNP 40 MCTD
Anti-Ro(SS-A) 30 Predisposes to Subcutaneous Lupus, Neonatal
Lupus with Congenital Heart Blocks.
Decreased risk of Nephritis
Anti-La(SS-B) 10 Decreased risk of Nepritis
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17. Autoantibodies in SLE
Antibody % Clinical Utility
Anti Histone 70 Drug induced SLE
Antiphospholipid 50 Abortions, Thrombosis
Antierythrocyte 60 Hemolysis
Antiplatelet 30 Thrombocytopenia
Antineuronal 60 Active CNS lupus
Antiribosomal 20 Depression or Psychosis due to CNS lupus
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18. ANA
• Present in 4-5% of healthy population and upto 14% of elderly or
chronically ill patients.
• Its sensitivity is high for SLE (98%) but specificity is low
• Diseases associated : SLE, MCTD, Systemic sclerosis, Drug inudced
SLE, Inflammatory myopathies, RA,Sjogrens
Thyroid ds, AI hepatitis, PBC, Hep C
• Patterns of ANA : Peripheral or rim pattern
Homogenous pattern
Speckled pattern
Centromere pattern
Nucleolar pattern.
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19. ANA PATTERNS
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A. Homogenous B. Speckled C. Centromere D. Nucleolar pattern

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21. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING
CLINIC (SLICC) CRITERIA FOR CLASSIFICATION OF SLE
A.Clinical Manifestations
1. Acute cutaneous Lupus
2. Chronic cutaneous Lupus
3. Oral or nasal ulcers
4. Non scarring alopecia
5. Arthritis
6. Serositis
7. Renal
8. Neurological
9. Hemolytic anemia
10. Leucopenia
11. Thrombocytopenia
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Petri M, et al. Arthritis and Rheumatism, Aug 2012

22. B. Immunological manifestations
1. ANA
2. Anti- dsDNA
3. Anti Sm
4. Antiphospholipid Ab
5. Low serum complement (C3,C4,CH50)
6. Positive direct coombs test
• Requirements: >= 4 criteria (atleast 1 in each category) OR
Biopsy proven Lupus Nephritis in presence of ANA or Anti ds DNA
• Sensitivity 97%, Specificity 84%
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Petri M, et al. Arthritis and Rheumatism, Aug 2012
SYSTEMIC LUPUS INTERNATIONAL COLLABORATING
CLINIC (SLICC) CRITERIA FOR CLASSIFICATION OF SLE

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Malar Rash Oral Ulcers

24. LUPUS NEPHRITIS -ISN &RPS CLASSIFICATION
• Class I Minimal mesangial LN
• Class II Mesangial proliferative LN
• Class III Focal LN (<50% of glomeruli)
• Class IV Diffuse LN (≥50% glomeruli)
• Class V Membranous LN
• Class VI Advanced sclerosing LN(>90% globally sclerosed
glomeruli without residual activity)
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25. TREATMENT
• Goals of treatment:
- prevent flares
- treat flares when they occur
- minimize organ damage and complications
• Treatment plans are based on patient age, sex, health,
symptoms and disease severity
– Fever, skin, musculoskeletal and serositis - milder disease
– CNS and renal involvement - Lifethreatening SLE
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26. TREATMENT - NON LIFE THREATENING SLE
• NSAIDS
• Antimalarials – Hydroxychloroquine
• Resistent cases – Low dose steroids (prednisolone 0.07 to 0.3
mg/kg)
- systemic immunosuppressants
• Dermatitis: Topical sunscreens, steroids, antimalarials or
Tacrolimus.
Systemic Dapsone or Thalidomide in resistent
cases
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27. TREATMENT - LIFE THREATENING SLE
• Glucocorticoids :
- Prednisolone - 0.5-1mg/kg orally or
- Methylprednisolone 1g/d for 3 days f/b oral therapy 4-6 wks
- Maintenance dose 5-10 mg/day
• Cytotoxic therapy :
- Induction therapy :
Cyclophosphamide - 500-750 mg/mt2 monthly for 6 months
Mycophenolate mofetil(MMF) 2-3 gm/day
- Maintanence therapy :
Azathioprine(2mg/kg/d) or MMF(1.5-3 gm/d)
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28. TREATMENT
• Other drugs
Chlorumbucil, Methotrexate, Leflunamide
Cyclosporine & tacrolimus
• Biological agents : used in resistent cases
Rituximab (Anti CD20 Ab)
Belimumab (Anti BLyS)
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31. MONITORING OF LUPUS NEPHRITIS
Blood
Pressure
Urine
Analys
is
Prot/Cr
Ratio
Serum
Creatinine
C3/C4
levels
Anti DNA
Active Nephritis 1 1 1 1 2 3
Previous active
nephritis,none
currently
3 3 3 3 3 6
No prior or
current nephritis
3 6 6 6 6 6
31Arthritis Care Res (Hoboken). 2012 June ; 64(6): 797–808. doi:10.1002/acr.21664

32. POOR PROGNOSTIC MARKERS
• Male sex
• Anaemia
• Hypoalbuminemia, Hypocomplementemia
• Nephrotic syndrome, Raised serum Cr
• HTN
• aPL Ab
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33. THANK YOU
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34. RHEUMATOID FACTOR
• RA FACTOR is not specific for RA
• Sensitivity in RA 75- 80%
• May not be positive in early disease.
• It is not a screening test for RA.
• Conditions associated with RA Factor :
- Rheumatologic diseases:
RA, SLE, sjogren’s, MCTD, myositis, cryoglobulinemia
- Infections:
SABE, TB, syphilis, hepatitis B&C
- Present in 1-5% of healthy population
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35. ANTI-CCP ANTIBODIES
• Sensitivity 75-80%, Specificity 95%
• 40% of seronegative RA are anti-CCP +ve
• Predictor of disease severity
• Present months prior to disease
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