Systemic Lupus Erythematosus, ANA’s, etc. Hermine Brunner, MD MSc Assistant Professor of Pediatrics Division of Rheumatology Cincinnati Children’s Hospital Medical Center. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)- DEFINITION/DIAGNOSIS. Prototype of auto-immune, multi-system disease
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Systemic Lupus Erythematosus, ANA’s, etc.
Hermine Brunner, MD MSc
Assistant Professor of Pediatrics
Division of Rheumatology
Cincinnati Children’s Hospital Medical Center
Adults tend to die secondary to complications
Tsao, BP, Curr Opinion Rheum, 2004; 16: 513-521
Discoid rashRenal disorder
Oral ulcersHematologic disorder
A)Pleuritis - convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion
B)Pericarditis - documented by ECG or rub or evidence of pericardial effusion
A)Persistent proteinuria greater than 0.5 grams per day or greater than 3+ if quantitation not performed
B)Cellular casts - may be red cell,hemoglobin, granular, tubular, or mixed
A)Seizures - in the absence of offending drugs or known metabolicderangements, e.g., uremia,ketoacidosis, or electrolyte imbalance
B)Psychosis - in the absence of offending drugs or known metabolic derangements, e.g. uremia, ketoacidosis, or electrolyte imbalance
A)Hemolytic anemia - with reticulocytosis
B)Leukopenia - less than 4,000/mm3 total on 2 or more occasions
C)Lymphopenia - less than 1,500/mm3 on 2 or more occasions
D)Thrombocytopenia - less than 100,000/mm3 in the absence of offending drugs
A)Anti-dsDNA: antibody to native DNA in abnormal titer
B)Anti-Sm: presence of antibody to Sm nuclear antigen
C) Antiphospholipid antibodies by positive IgG or IgM anticardiolipin antibodies or positive test for lupus anticoagulant
Tissue Specific Nuclear
Cabral, DA, et al Pediatrics 1992, 89(3):441-444
10 (9%) were lost to F/U, 31 (27%) had no AID,
Deane, PMG, et al, Pediatrics 1995, 95:892-895
Most common signs/symptoms
vasculitis in SLE
Behavior/Personality changes, depression
Total of 19 manifestations described
Nephritis remains the most frequent cause of disease-related death.
Class IIIFocal and segmental proliferative glomerulonephritis
Class IVDiffuse proliferative glomerulonephritis
Class VMembranous glomerulonephritis
Class VIGlomerular sclerosis
ACLA - Anticardiolipin antibodies
LAC - Lupus anticoagulant
short stature, cataracts, osteoporosis
Contreras, G et al: NEJM 350(10): 971-980, 2004
Houssian FA, J Am Soc Neprol, 2004; 15: 2694-2704
Ro (SSA) , La (SSB) , RNP , Sm ,
ds DNA 1:5120, APA negative
Monastero R, et al, J of the Neurological Sci 2001; 184:33-39
Stichweh, D , Curr Opin Rheumatol 16:577-587, 2004
Schanberg LE, Sandborg C, Current Rheum Reports 2004;6:425-433
9.3U/A: 1.015, pH 6.0,
9.7 137>300 mg protein,
ALC – 1360
ESR - >140; CRP – 5.26
C3 – 153; C4 - 21.2
[Thrombotic Profile – normal]
[DAT – positive]
ANA – positive at 1:2560; other autoantibodies all negative
[APA Profile – positive]
Light Microscopy with Silver Stain showing epimembranous deposits.
Electron Microscopy showing epimembranous deposits.
Light Microscopy showing increased mesangial cells.
Seaman DE, et al, Pediatrics. 1995; 96: 1040-5
Petri,M, Arthritis Rheum 2004, 50(9): S239, abstract 523
Brunner, HI, et al. Arthritis and Rheumat.2002;46:436-44.
Hagelberg, S. J Rheumatol. 2002;29:2635-42.
Brunner et al, Lupus 2006, in press