Lupura® is a professional grade nutraceutical with a unique and comprehensive blend of herbs, bioflavonoids and enzymes designed to promote a healthy inflammatory response.

1. Systemic LupusSystemic Lupus
ErythematosusErythematosus
LupuraLupura

2. Questions???Questions???
 1. What is compound E?1. What is compound E?
 2. Who is Phillip Hench?2. Who is Phillip Hench?
 3. What are the 11 criteria for lupus?3. What are the 11 criteria for lupus?
 4. What are the two most common drugs4. What are the two most common drugs
associated with drug induced lupus?associated with drug induced lupus?
 5. What is the risk of neonatal lupus in a5. What is the risk of neonatal lupus in a
patient with positive SSA or SSB?patient with positive SSA or SSB?

3. Questions???Questions???
 6. What is the most common organ6. What is the most common organ
system involved in lupus?system involved in lupus?
 7. How many FDA approved medicines7. How many FDA approved medicines
are there for lupus and what are they?are there for lupus and what are they?
 8. What is the 10 year life expectancy for8. What is the 10 year life expectancy for
lupus?lupus?

4. Case #1Case #1
 23 year old female comes to rheumatology c/o joint23 year old female comes to rheumatology c/o joint
pains in her PIPs and wrists, fatigue and a rash on herpains in her PIPs and wrists, fatigue and a rash on her
face. This has been going on for several months. Sheface. This has been going on for several months. She
denies any fevers, ulcers, CP, SOB, Raynaud’s. Shedenies any fevers, ulcers, CP, SOB, Raynaud’s. She
does have photosensitivity.does have photosensitivity.
 PMH: acne, endometriosisPMH: acne, endometriosis
 PSH: nonePSH: none
 Meds: OCP, minocyclineMeds: OCP, minocycline
 PE: VSS, malar rash, TTP to PIPs, no synovitisPE: VSS, malar rash, TTP to PIPs, no synovitis
 Labs: WBC 5, Hg 11, Plt 200, BUN 8, Cr 0.6, ANA posLabs: WBC 5, Hg 11, Plt 200, BUN 8, Cr 0.6, ANA pos
1:160, TSH 4.31:160, TSH 4.3
 What do you do?What do you do?

5. History of LupusHistory of Lupus
 Lupus means “wolf” in LatinLupus means “wolf” in Latin
 1010thth
century- case reports appeared in writingscentury- case reports appeared in writings
 Late 1800s- Sir William Osler initially described theLate 1800s- Sir William Osler initially described the
systemic nature and linked rashes to organ involvementsystemic nature and linked rashes to organ involvement
 1949- LE cell described by Malcolm Hargraves at Mayo1949- LE cell described by Malcolm Hargraves at Mayo
ClinicClinic
 1954- ANA described1954- ANA described
 1971- First set of classification criteria proposed for Lupus1971- First set of classification criteria proposed for Lupus
 1983- Antiphospholipid antibody syndrome described1983- Antiphospholipid antibody syndrome described

6. What exactly is Lupus?What exactly is Lupus?
 Autoimmune disease where one’s immune systemAutoimmune disease where one’s immune system
attacks itselfattacks itself
 Autoantibody production -> immune complexAutoantibody production -> immune complex
deposition -> inflammation -> damagedeposition -> inflammation -> damage
 Chronic disease, characterized by flares andChronic disease, characterized by flares and
remissionremission
 Pleomorphic with different phenotypic expressionsPleomorphic with different phenotypic expressions
 Multisystem involvementMultisystem involvement

7. Types Of LupusTypes Of Lupus
 Drug Induced LupusDrug Induced Lupus
 Neonatal LupusNeonatal Lupus
 Cutaneous LupusCutaneous Lupus
 Systemic Lupus ErythematosusSystemic Lupus Erythematosus

8. Drug- Induced LupusDrug- Induced Lupus
 Approximately 80 offending agents canApproximately 80 offending agents can
cause lupuscause lupus
 15,000- 30,000 cases reported annually15,000- 30,000 cases reported annually
 Production of autoantibodies more commonProduction of autoantibodies more common
than clinical symptomsthan clinical symptoms
 99% disappear within 3 months of stopping99% disappear within 3 months of stopping
the medicine.the medicine.

9. DIL Definite AssociationsDIL Definite Associations
 Procainamide (15-20%)Procainamide (15-20%)
 Hydralazine (7-13%)Hydralazine (7-13%)
 Enbrel/Remacaide/Humira (2/1000)Enbrel/Remacaide/Humira (2/1000)
 Minocycline (5/10,000)Minocycline (5/10,000)
 DiltiazemDiltiazem
 PenicillaminePenicillamine
 INHINH
 QuinidineQuinidine

10. ClinicalClinical DILDIL Classic SLEClassic SLE
Age of onsetAge of onset 5050 20-4020-40
F:M ratioF:M ratio 1:11:1 9:19:1
arthralgiaarthralgia 95%95% 90%90%
hepatomegalyhepatomegaly 25%25% 25%25%
RashRash 10-20%10-20% 74%74%
Renal diseaseRenal disease 5%5% 53%53%
CNS diseaseCNS disease 0%0% 32%32%
ANAANA 95%95% 95%95%
Anti-histoneAnti-histone 90%90% 80%80%
dsDNA, RNP,dsDNA, RNP,
SmithSmith
rarerare 80%/50%80%/50%
20%20%

11. Case #1Case #1
 23 year old female comes to rheumatology c/o joint23 year old female comes to rheumatology c/o joint
pains in her PIPs and wrists, fatigue and a rash on herpains in her PIPs and wrists, fatigue and a rash on her
face. This has been going on for several months. Sheface. This has been going on for several months. She
denies any fevers, ulcers, CP, SOB, Raynaud’s. Shedenies any fevers, ulcers, CP, SOB, Raynaud’s. She
does have photosensitivity.does have photosensitivity.
 PMH: acne, endometriosisPMH: acne, endometriosis
 PSH: nonePSH: none
 Meds: OCP, minocyclineMeds: OCP, minocycline
 PE: VSS, malar rash, TTP to PIPs, no synovitisPE: VSS, malar rash, TTP to PIPs, no synovitis
 Labs: WBC 5, Hg 11, Plt 200, BUN 8, Cr 0.6, ANA posLabs: WBC 5, Hg 11, Plt 200, BUN 8, Cr 0.6, ANA pos
1:160, TSH 4.31:160, TSH 4.3
 What do you do?What do you do?

12. Case #2Case #2
 32 year old female with known Lupus for32 year old female with known Lupus for
five years, on plaquenil 400mg a day andfive years, on plaquenil 400mg a day and
prednisone 3mg a day, wants to getprednisone 3mg a day, wants to get
pregnant. How do you advise her?pregnant. How do you advise her?
 Fertility?Fertility?
 Risk of neonatal lupus?Risk of neonatal lupus?
 Medications during pregnancy?Medications during pregnancy?

13. Neonatal LupusNeonatal Lupus
 Rare conditionRare condition
 not true lupus, passively transferred autoimmunenot true lupus, passively transferred autoimmune
diseasedisease
 Occurs when mother is SSA/SSB positiveOccurs when mother is SSA/SSB positive
 Transplacental transfer of IgG anti SSA or SSBTransplacental transfer of IgG anti SSA or SSB
antibodiesantibodies
 5-7% babies will have a transient rash, resolves by5-7% babies will have a transient rash, resolves by
6-8 months6-8 months
 2% of babies will have cardiac complications with2% of babies will have cardiac complications with
congenital heart blockcongenital heart block

14. Neonatal LupusNeonatal Lupus
 See erythematous,See erythematous,
 annular plaquesannular plaques
 tends to involvetends to involve
 scalp, face,scalp, face,
 periorbital areasperiorbital areas

16. Who get’s Lupus?Who get’s Lupus?
 Prevalence is over 1.5 million AmericansPrevalence is over 1.5 million Americans
 Incidence difficult due to lack of strictIncidence difficult due to lack of strict
definitiondefinition
 Bimodal peak presentation: ages 20-40 andBimodal peak presentation: ages 20-40 and
again after age 60again after age 60
 Prevalence is higher in African Americans,Prevalence is higher in African Americans,
Asians, HispanicsAsians, Hispanics
 Female to male predominanceFemale to male predominance

17. Does your sex really matter?Does your sex really matter?
 90% of patients with lupus are female90% of patients with lupus are female
 Before puberty F:M ratio is 2:1Before puberty F:M ratio is 2:1
 During reproductive years ratio 8:1During reproductive years ratio 8:1
 Post-menopausal ratio 2.3:1Post-menopausal ratio 2.3:1
 Increased frequency in women attributed toIncreased frequency in women attributed to
the hormonal effect of estrogenthe hormonal effect of estrogen

18. Does your sex really matter?Does your sex really matter?
 Nurses Health StudyNurses Health Study
 use of estrogen-containing contraceptive agentsuse of estrogen-containing contraceptive agents
associated with an 50 percent increase in risk ofassociated with an 50 percent increase in risk of
developing SLEdeveloping SLE
 either early onset of menarche (age ≤ 10 years) oreither early onset of menarche (age ≤ 10 years) or
administration of estrogen to postmenopausaladministration of estrogen to postmenopausal
women doubles their riskwomen doubles their risk
 Treatment of clinically stable SLE with oralTreatment of clinically stable SLE with oral
contraceptives for one year does not increasecontraceptives for one year does not increase
disease flaresdisease flares

19. What causes Lupus???What causes Lupus???
Genetically
susceptible
individual
Environmental
factors
Auto
antibody
production
Inflammation
Damage

20. Genetics of LupusGenetics of Lupus
 High concordance in monozygotic twinsHigh concordance in monozygotic twins
 5-12% or relatives with lupus have the disease5-12% or relatives with lupus have the disease
 No single lupus geneNo single lupus gene
 Disease is polygenicDisease is polygenic
 At least 30 susceptiblility genes identifiedAt least 30 susceptiblility genes identified
 HLADR2, HLADR3, HLADR4, HLADR8 (presentHLADR2, HLADR3, HLADR4, HLADR8 (present
in 75%)in 75%)
 Homozygous deficiency of C1q complementHomozygous deficiency of C1q complement

21. Environmental FactorsEnvironmental Factors
 UVA and UVB light can stimulate/ up-regulateUVA and UVB light can stimulate/ up-regulate
autoimmunityautoimmunity
stimulating keratinocytes to produce cytokines -> activate Bstimulating keratinocytes to produce cytokines -> activate B
cells to produce abcells to produce ab
 Viruses/Bacteria: molecular mimicryViruses/Bacteria: molecular mimicry
SLE patients have higher titers of antibodies to Epstein-BarrSLE patients have higher titers of antibodies to Epstein-Barr
virus (EBV), increased circulating EBV viral loads; SSA abvirus (EBV), increased circulating EBV viral loads; SSA ab
has a sequence similar to EBV nuclear ag 1has a sequence similar to EBV nuclear ag 1
Parvovirus B19Parvovirus B19
 DrugsDrugs
 Silica exposure, tobacco smoke, emotional stressSilica exposure, tobacco smoke, emotional stress

22. LupusLupus
Genetically
susceptible
individual
Environmental
factors
Auto
antibody
production
Inflammation
Damage

23. Immune dysregulationImmune dysregulation
 Upregulation of innate immunityUpregulation of innate immunity
 Delayed clearance of apoptotic cells, resulting inDelayed clearance of apoptotic cells, resulting in
antigenic stimulationantigenic stimulation
 Loss of tolerance via failed elimination ofLoss of tolerance via failed elimination of
autoreactive T lymphocytesautoreactive T lymphocytes
 Abnormalities in B cellsAbnormalities in B cells
 Abnormalities in T regulatory cells (CD4+/CD25+Abnormalities in T regulatory cells (CD4+/CD25+
cells down regulate immune system responses)cells down regulate immune system responses)