In this Power Point lecture I discuss how to diagnose lupus using the new 2012 classification criteria by SLICC as well as the importance of vitamin D, light protection, not smoking, and preventing human papilloma virus infection in people who have systemic lupus erythematosus. To learn more about the causes and symptoms of lupus as well as its complications such as arthritis, Sjogren's syndrome, and fibromyalgia, go to and click on "Like" to get daily tips and facts at http://www.facebook.com/LupusEncyclopedia

1. Donald Thomas, MD, FACP, FACR
Arthritis and Pain Associates of PG County
Assistant Professor of Medicine
Uniformed Services University of the Health Sciences, Bethesda

2. photo credit: africanleadershipacademy.org

3. -
LUPUS
Women of childbearing
age
More severe dz in
younger patients
1/200 African American
women of child bearing
age

4. -
-
LUPUS
Women of childbearing
age
More severe dz in
younger patients
1/200 African American
women of child bearing
age
“Invisible disease”
Average of 4-6 years
before diagnosis

5. -
-
LUPUS
Women of childbearing
age
More severe dz in
younger patients
1/200 African American
women of child bearing
age
“Invisible disease”
Average of 4-6 years
before diagnosis
- 5-10% die within
10 years of dx

6. Whitney
-
24 yo

7. -
Whitney
Born 12/14/88
Died 2/20/13 from SLE
photo credit: facebook.com/Lupus –Wall- Remembering- those- who- have- lost- their- Battle

8. - 95% of patients live 10 years or longer
- Most patients live a long normal life with proper treatment
- Best prognosis:
-
Early diagnosis
Proper medical care (doctors, medications, tests, educated)
photo credit: sometimesitslupus.com

9. - New “classification criteria” for systemic lupus
- What labs to order for lupus workup
- Correction of lupus triggers
-
Low vit D, UV light, smoking, sulfa antibiotics
- Ensure vaccines are obtained
- Resources to recommend to college students with lupus

10. - 4 out of 14 criteria = SLE
- Classification criteria = for research purposes only
-
Not recommended for diagnostic purposes
- 2004: embarked upon revision

11. - Missing in 1982 criteria
Low complements
Antiphospholipid antibodies
-
- 1982 weighted towards cutaneous dz (4 of 14 criteria)
Excluded biopsy proven lupus nephritis as sole manifestation
Neuro lupus only included psychosis and seizures
-
-
-
ACR lists 18 potential neurologic disorders in neuropsychiatric lupus
Could only use one type of low blood count
- LE cell prep no longer used

12. - Diagnosed SLE patients vs those meeting classification
Many patients with early SLE didn’t meet criteria
By the time they do they are:
-
Older
Had established disease longer
More end-organ damage

13. - SLE occurs if
-
Biopsy proven lupus nephritis + ANA or dsDNA
- OR
-
-
4 out of 17 criteria
At least 1 from “Clinical Criteria” and from “Immunologic Criteria”

14. -
Renal
Alopecia, nonscarring
Serositis
Hemolytic anemia
-
Oral and nasal ulcers
Neurologic
-
Synovitis
Chronic cutaneous lupus (discoid)
Acute and subacute cutaneous lupus
Leucopenia/lymphopenia
Platelets, low

15. - Random urine protein/creatinine ratio ≥ 0.500
- 25 hour urine protein ≥500 mg protein/24 hours
- Red blood cell casts on urine microscopy
photo credit: studyblue.com

16. - Diffuse thinning
- Hair fragility, broken hair
- “Lupus hair”
- Rule out alopecia areata, drugs, iron deficiency, androgenic
alopecia
- Grows back
CellCept

17. - Pleuritis
-
“Typical pleurisy” > 1 day
Pleural effusions
Pleural rub
- Pericarditis
-
“Typical pericardial pain” > 1 day (worse with lying, better sitting forward)
Pericardial effusion
Pericardial rub
+ ECG
Photo credit: clinicalcases.org

18. - Direct Coombs antibody positive
- High reticulocyte count
- Low haptoglobin
- Increased indirect bilirubin
Photo credit: commons.wikipedia.org

19. - Oral ulcers
-
Palate, buccal, tongue
Often painless
- Nasal ulcers
- Rule out:
-
-
Vasculitis
Behçet’s disease
Infections (HSV)
Inflammatory bowel disease
Reactive arthritis
Photo credit: de.wikipedia.org

20. - Seizures
- Psychosis
- Mononeuritis multiplex
-
in absence of a 1° vasculitis
- Myelitis
- Peripheral or Cranial neuropathy
-
R/o diabetes, infection (Lyme), 1° vasculitis
- Acute confusional state
-
R/o toxic, metabolic, uremia, infection, drugs
Photo credit: en.wikipedia.org

21. - ≥ 2 joints
-
Swelling or effusion OR
Tender joints + AM stiffness ≥ 30 minutes
Photo credit: cdaarthritis.com

22. - Discoid lupus
- Hypertrophic (verrucous) lupus
- Lupus panniculitis (profundus)
- Discoid lupus/lichen planus overlap
- Lupus erythematosus tumidus
- Chilblains lupus
- Mucosal lupus
Photo credit: entindia.info

23. - Malar rash (don’t count discoid)
- Toxic necrolysis variant of SLE
- Maculopapular lupus rash
- Photosensitive lupus rash
- Bullous lupus
- SCLE:
-
Non-indurated psoriasiform
Annular polycyclic
Photo credit: globalskinatlas.com

24. - WBC < 4000/mm3 (once)
-
R/o Felty’s syndrome, drugs, portal hypertension
- Lymphs < 1000/mm3 (once)
-
R/o steroids, drugs, infections (virus)

25. - Platelets< 100,000 (once)
-
R/o TTP, drugs, portal hypertension

26. - ANA
- Anti-ds DNA
- Antiphospholipid antibodies
-
-
Lupus anticoagulant
False positive RPR
Anticardiolipin antibody
Beta-2 glycoprotein antibody
- Low complements (C3, C4, CH50)
- Direct Coombs’ test (in absence of hemolytic anemia)

27. - Out of 702 patient scenarios……….
- Misclassified patients: 7% vs 10%
- Sensitivity: 94% vs 86%
- Specificity: 92% vs 93% (not statistically different)

28. “… if you use the
classification criteria to
diagnose SLE... I
promise not to tell
anyone.”
Michelle Petri, MD: Medical Director Lupus Clinic
Johns Hopkins

29. -
Renal (proteinuria)
Alopecia
Serositis (pleuritic chest pain)
Hemolytic anemia (all low blood counts)
-
Oral and nasal ulcers
Neurologic problems
-
Synovitis (joint pains)
Chronic cutaneous lupus (discoid)
Acute cutaneous lupus (malar rash, rash with sun exposure)
Leukopenia/lymphopenia and Platelets, low
-
Blood clots
Raynaud’s phenomenon

30. - Basic/Initial
-
ANA by IFA (indirect fluorescence assay)
-
CBC
Urinalysis with reflex microscopy
Random urine protein/creatine ratio
ESR, CRP, SPEP
25-OH vitamin D
-
-
- If pleuritic chest pain
-
CXR
ECG
Echocardiogram

31. - If positive ANA by IFA
ds-DNA
ENA (Smith, RNP)
Sjögren's panel (SSA/SSB)
Ribosomal-P antibody
C3, C4, CH50 complements
Direct Coombs’ test
Antiphospholipid antibodies
-
-
RPR with reflex FTA
Anticardiolipin antibodies (IgM, IgG, IgA)
Lupus anticoagulant
Beta-2 glycoprotein I antibodies (IgM, IgG, IgA)
- Inflammatory arthritis:
-
CPK, RF, CCP, Lyme, HLA-B27, ASO, IgM Parvovirus

32. - Low vitamin D levels
- UV light
- Smoking
- Sulfa antibiotics

33. - White blood cell membranes have Vit D receptors
- Higher prevalence of low Vit D in SLE patients
- More severe SLE at presentation associated with lower Vit D
- Lower Vit D levels occur during SLE flares
- Low vitamin D correlated with flares

34. - Petri M et al, Vitamin D and SLE, Arthr & Rheum;65(7):1865-71
1006 patients, 128 weeks
25[OH]D < 40 ng/mL
TX = 50,000 IU ergocalciferol (vit D2) + daily calcium with 200 IU vit D3
-
- Results:
- ≥ 20 ng/mL increase 25[OH]D associated with:
-
.22 decrease in SELENA/SLEDAI (P = .032)
21% decrease in having a SELENA/SLEDAI ≥ 5
Random urine/protein decreased by 2% (P = .0001)
15% decrease in odds of having urine/prot > .5

35. - Treat patients with 25[OH]D < 40 ng/mL
- Aim for a level of around 40 ng/mL or higher

36. Ultraviolet light

37. Ultraviolet light
Skin
NUCLEUS
cell

38. Ultraviolet light
Skin
NUCLEUS
cell
damage
cell
NUCLEUS

39. Ultraviolet light
Skin
NUCLEUS
cell
Antinuclear antibodies
Cause increased lupus activity

40. X 15 minutes
Dose of UV light = Strength X Time

41. X 15 minutes
Dose of UV light = Strength X Time
X all day long

42. - Wear sunscreen daily even if don’t go outside
- Reapply if go outside
- Use sunscreen vs UVA and UVB + waterproof + high SPF
- Wide brimmed hat
- UV protectant clothes
- Add Rit Sunguard to wash
- Avoid outside 10 AM – 3 PM

43. - Tobacco contains hydrazine
-
Hydrazine known to increase lupus activity
- Smoking decreases effectiveness of Plaquenil
- Smoking is associated with increased lupus prevalence
- Smoking associated with more severe lupus

44. - Increased risk for lupus flares
- Ask patients to include Bactrim and Septra in allergies

45. - Make sure all patients get
yearly flu shot

46. - Dreyer L et al, High Incidence of Potentially Virus-Induced
Malignancies in SLE, Arth & Rheum, 2011;63(10):3032-37
Increased HPV-associated cancers
-
Anal cancer
Vulvovaginal
Cervical
Non-melanoma skin cancer
- Nath R et al, High risk of Human Papillomavirus Type-16 infections
and of development of squamous intraepithelial lesions in
systemic lupus erythematosus patients, A&R, 2007;57(4):619-25
-
High levels of HPV-16 infection and abnormal colposcopy in newly
diagnosed SLE women

47. photo credit: beasleyallen.com

48. - Lupus Foundation of America DC/MD/VA chapter
Patient Navigator service
www.lupus.org/dmv
888-787-5380
-
- “Lupus Secrets” handout (last page)
- Social Media:
Facebook: Lupus Encyclopedia
-
-
-
www.facebook.com/LupusEncyclopedia
Daily tips and facts about lupus
I answer questions posted by patients
Numerous Facebook patient support groups

49. - SLICC new SLE classification criteria
-
-
4 out of 17
at least 1 from “clinical” and 1 from “immunologic”
- Basic initial workup: ANA, CBC, UA
-
Do additional labs if ANA+
Refer to rheumatologist ASAP
- Begin tx: Vitamin D, Sunscreen, no cigarettes
- Vaccines:
-
Annual flu shot
Gardasil series
- Resources are available

50. photo credit: customink.com

51. References 1:
 Agmon-Levin N et al. International recommendations for
the assessment of autoantibodies to cellular antigens
referred to as anti-nuclear antibodies. Ann Rheum Dis.
2014;73:17-23
 Amital H et al. Serum concentration of 25-OH vitamin D in
patients with SLE are inversely related to disease activity.
Ann Rheum Dis.2010,69:1155-57.
 Birmingham DJ et al. Evidence that abnormally large
seasonal declines in vitamin D status may trigger SLE flare
in non-African Americans. Lupus. 2012;21(8):855-64
 Bonakdar ZS et al. Vitamin D deficiency and its association
with disease activity in new cases of systemic lupus
erythematosus. Lupus.2011;20:1155-60

52. References 2:
 Boeckler P et al. Association of cigarette smoking but
not alcohol consumption with cutaneous lupus
erythematosus. Arch of Derm. 2009;145(9):1012-16
 Cooper G et al. Occupational and environmental
exposures and risk of systemic lupus erythematosus:
silica, sunlight, solvents. Rheum (Oxford).
2010;49(11):2172-80
 Dreyer L et al. High incidence of potentially virusinduced malignancies in systemic lupus
erythematosus. Arth & Rheum. 2011;63(10):3032-37

53. References 3:
 Ghaussy NO et al. Cigarette smoking and disease
activity in systemic lupus erythematosus. J of Rheum.
2003;30:1215-21
 Isenberg DA et al. The Systemic Lupus International
Collaborating Clinics (SLICC) group – It was 20 years
ago today. Lupus. 2011;20:1426-32
 Mok CC et al. Vitamin D deficiency as marker for
disease activity and damage in systemic lupus
erythematosus. Lupus. 2012;21:36-42

54. References 4:
 Nath Ret al. High risk of human papilloma virus type
16 infections and of development of cervical squamous
intraepithelial lesions in systemic lupus erythematosus
patients. Arth & Rheum. 2007;57(4):619-25
 Petri M et al. Vitamin D in SLE. Arth & Rheum.
2013;65(7):1865-71
 Petri M et al. Derivation and validation of the systemic
Lupus International Collaborating Clinics
classification criteria for SLE. Arthr & Rheum.
2012:2677-86

55. References 5:
 Petri M & Magder L. Classification criteria for SLE.
Lupus. 2004;13:829-37
 Pons-Estel GJ et al. The ACR and the SLICC criteria for
SLE in two multiethnic cohorts. Lupus. 2014;23:3-9
 Rahman P et al. Smoking interferes with efficacy of
antimalarial therapy in cutaneous lupus. J of Rheum.
1998;25:1716-19
 Ruiz-Irastorza G et al. Changes in vitamin D levels in
patients with SLE. Arthr Care & Research.
2010;62(8):1160-65