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Systemic Lupus Erythematosis
- 1. SYSTEMIC LUPUS ERYTHEMATOSES 1 Dr Jayakrishnan MP PG Resident, Internal Medicine MGM Medical College, Indore
- 4. Determine whether musculoskeletal complaint is 1. Articular or nonarticular. 2. Inflamatory or noninflamotory. 3. Acute or chronic. 4. Localised or widespread 4
- 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. 5
- 6. ARTHRITIS POLYARTHRITIS (> 5 joints) OLIGOARTHRITIS (2-4 joints) GOUT SARCOIDOSIS LYMES Ds BECHETS dS MONOARTHRITIS (1 joint) TRAUMA INFECTION CRYSTALS TUMOUR 6
- 7. POLYARTHRITIS INFLAMMATORY RHEUMATOID TYPES RA, SLE SSc, DM VIRAL INFECTIONS RHEUMATOID VARIANTS AS, ReA Ps A IBD DEGENERATIVE OA METABOLIC GOUT AMYLOIDOSIS 7
- 8. ACR Ad Hoc committee on clinical guidelines, Arthritis rheum 1996; 39: 1 8
- 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 10 Harrison 19th edition
- 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 11 J Indian Rheumatol Assoc 2002 : 10 : 80 - 96
- 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) 13 Harrison 19th edition
- 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 14 Harrison 19th edition
- 15. 8. Gastrointestinal 40 9. Thrombosis 15 Venous, Arterial 10. Occular 15 Sicca syndrome,Conjuctivitis, Episcleritis, Vasculitis CLINICAL MANIFESTATIONS 15 Harrison 19th edition
- 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 16 Harrison 19th edition
- 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 17 Harrison 19th edition
- 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. 18 Harrison 19th edition
- 19. ANA PATTERNS 19 A. Homogenous B. Speckled C. Centromere D. Nucleolar pattern
- 20. 20
- 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 21 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% 22 Petri M, et al. Arthritis and Rheumatism, Aug 2012 SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINIC (SLICC) CRITERIA FOR CLASSIFICATION OF SLE
- 23. 23 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) 24 Harrison 19th edition
- 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 25 Harrison 19th edition
- 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 26 Harrison 19th edition
- 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) 27 Harrison 19th edition
- 28. TREATMENT • Other drugs Chlorumbucil, Methotrexate, Leflunamide Cyclosporine & tacrolimus • Biological agents : used in resistent cases Rituximab (Anti CD20 Ab) Belimumab (Anti BLyS) 28
- 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 32
- 33. THANK YOU 33
- 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 34 Harrison 19th edition
- 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 35 Harrison 19th edition