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Systemic Lupus Erythematosus Sheelendra Shakya Dept. of Pediatrics, KMCTH
Introduction ,[object Object],[object Object]
Topics of Discussion ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pathogenesis ,[object Object],[object Object],[object Object],[object Object],[object Object]
Pathogenesis ,[object Object],[object Object],[object Object],[object Object]
Pathogenesis
Pathogenesis ,[object Object],[object Object],[object Object]
Case study ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
HPI ,[object Object],[object Object],[object Object],CRP >6mg/dl,  Urine RME : RBC and pus plenty, albumin + TC8400cumm  N82L17M1  Hb 13gm% ESR 52mm in 1 st  hr
O/E ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Investigations CBC: TC 3500 N63L36M1;Hb 6.9gm%; PLT 42000/cumm ESR 72mm in 1 st  hour Peripheral Smear:  normocytic, normochromic to mildly hypochromic e mild anisocytosis.   Plt reduced on smear Biochemistry: Urea 96mg/dl  Cr 1.1mg/dl;  Na 123meq/L,  K 4meq/L Total protein: 4.3g/dl Albumin: 3.6g/dl Total Cholesterol 210 mg/dl HDL: 27mg/dl Triglycerides: 697mg/dl  LDL could not be calculated.  ANA  , CRP, ASO, RA factor- negative Urine:   pus 6-8/hpf, RBC plenty; Albumin 2 +, cast 0-2 24 hour urine volume: 350ml 24 hour urinary protein 2717mg. Urine C/S- insignificant bacteriuria Montoux test: no induration after 72 hrs Reticulocytes: 4% Albumin repeated after 2 weeks: 1.8mg/dl Anti-dsDNA  by ELISA- positive (69.12 IU/ml) USG: B/L medicorenal disease B/L pleural effusion Minimal pericardial effusion. Ascites CXR:  B/L pleural effusion Repeat Urine RMEs:   persistent albumin, RBC and pus cells.
Diagnosis ,[object Object],[object Object],[object Object],[object Object]
Clinical Presentation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Muscles and bones ,[object Object],[object Object],[object Object],[object Object],[object Object]
Skin manifestations ,[object Object],[object Object],[object Object],[object Object]
Skin manifestations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Renal ,[object Object],[object Object],[object Object],[object Object],[object Object],Mesangial proliferative lupus nephritis Advanced sclerosis lupus nephritis Membranous lupus nephritis
Neuropsychiatric ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Neuropsychiatric syndromes in SLE as defined by American College of Rheumatology
Respiratory ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Cardiac
Cardiac ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Hematological ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Gastrointestinal ,[object Object],[object Object],Constitutional ,[object Object],[object Object],[object Object],[object Object],Ocular ,[object Object]
Diagnostic criteria ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Fixed erythema, flat or raised, over malar eminences, tending to spare nasolabial folds Erythematous raised patches of adherent keratotic scaling and follicular plugging. Atrophic scarring may be seen in older lesions. Skin rash as a result of unusual reaction to sunlight (UV rays) Oral or nasopharyngeal ulcers, usually painless, observed by a physician Non-erosive arthritis involving 2 or more peripheral joints, characterised by tenderness, swelling or effusion Pleuritis – pleuritic pain, rub heard or pleural effusion, or, Pericarditis – documented by ECG or rub or pericardial effusion Persistent proteinuria > 0.5g/day not > 3+. Cellular casts: red cell, haemoglobin, granular, tubular or mixed. Seizures or psychosis in the absence of offending drugs or metabolic derangement. Haemolytic anaemia with reticulocytes, or:  Leucopenia < 4000/mm 3  on 2 or more occassions, or: Lymphopenia <1500/mm 3  on 2 or more occasions, or: Thrombocytopenia<100000/mm 3  in absence of offending drugs Anti-DNA antibody, or: Anti-Sm antibody, or: Anti-phospholipid antibodies: i. abnormal anticardiolipin antibody IgG or IgM; ii. Positive lupus anticoagulant; iii. False positive for syphilis for > 6 months Abnormal titre of ANA at any point in absence of drugs known to be associated with drug induced lupus 4 out of 11 criteria gives a 96% sensitivity and 96% specificity for SLE. Criteria do not all have to be present at a defined time and can be cumulative.
Useful Investigations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Useful Investigations
Useful Investigations ,[object Object],[object Object],[object Object],TO GUIDE TREATMENT ON USE OF POTENTIALLY TOXIC DRUGS TO PREDICT OUTCOME
International Society of Nephrology 2003 classification of lupus nephritis Chronic lesions and sclerosis Advanced sclerosing Class VI Predominantly nephrotic disease Membranous Class V >50% of glomeruli involved Diffuse proliferative Class IV <50% of glomeruli involved Focal proliferative Class III Hypercellular on light microscopy Mesangial proliferative Class II Normal light microscopy findings, abnormal electron microscopy findings Minimal mesangial Class I
Principles of Management ,[object Object],[object Object],[object Object],[object Object]
Medications ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Medications ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Class specific Treatment ,[object Object],[object Object]
Class specific Treatment ,[object Object],[object Object]
immunosuppressive drugs indication ,[object Object],[object Object],[object Object],[object Object],[object Object]
Immunosuppressive regimen ,[object Object],[object Object],[object Object],[object Object]
Class specific Treatment ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Class specific Treatment ,[object Object],[object Object],[object Object]
Surgical Care ,[object Object],[object Object]
Prognosis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Prognosis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Prognosis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Complications: ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],J. Rheumatology 1990
Summary: ,[object Object],[object Object],[object Object],[object Object]
Thank you

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Systemic Lupus Erythematosus (SLE) Overview

  • 1. Systemic Lupus Erythematosus Sheelendra Shakya Dept. of Pediatrics, KMCTH
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  • 11. Investigations CBC: TC 3500 N63L36M1;Hb 6.9gm%; PLT 42000/cumm ESR 72mm in 1 st hour Peripheral Smear: normocytic, normochromic to mildly hypochromic e mild anisocytosis. Plt reduced on smear Biochemistry: Urea 96mg/dl Cr 1.1mg/dl; Na 123meq/L, K 4meq/L Total protein: 4.3g/dl Albumin: 3.6g/dl Total Cholesterol 210 mg/dl HDL: 27mg/dl Triglycerides: 697mg/dl LDL could not be calculated. ANA , CRP, ASO, RA factor- negative Urine: pus 6-8/hpf, RBC plenty; Albumin 2 +, cast 0-2 24 hour urine volume: 350ml 24 hour urinary protein 2717mg. Urine C/S- insignificant bacteriuria Montoux test: no induration after 72 hrs Reticulocytes: 4% Albumin repeated after 2 weeks: 1.8mg/dl Anti-dsDNA by ELISA- positive (69.12 IU/ml) USG: B/L medicorenal disease B/L pleural effusion Minimal pericardial effusion. Ascites CXR: B/L pleural effusion Repeat Urine RMEs: persistent albumin, RBC and pus cells.
  • 12.
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  • 27.
  • 28. International Society of Nephrology 2003 classification of lupus nephritis Chronic lesions and sclerosis Advanced sclerosing Class VI Predominantly nephrotic disease Membranous Class V >50% of glomeruli involved Diffuse proliferative Class IV <50% of glomeruli involved Focal proliferative Class III Hypercellular on light microscopy Mesangial proliferative Class II Normal light microscopy findings, abnormal electron microscopy findings Minimal mesangial Class I
  • 29.
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  • 34.
  • 35.
  • 36.
  • 37.
  • 38.
  • 39.
  • 40.
  • 41.
  • 42.
  • 43.