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Flashcards in Lupus Deck (40):
1

What are the 5 types of Lupus?

Subacute Lupus Erythematosus
Discoid Lupus
Drug Induced Lupus
Systemic Lupus Erythematosus
Neonatal Lupus*

2

What is Lupus?

1. Lupus is a systemic autoimmune disease in which the body loses tolerance to self

2. Can affect virtually any organ in the body and initial symptoms are often nonspecific, making it very difficult to diagnose

3. Typical autoantibodies seen are those reacting against nuclear components of the cell

4. Most commonly seen in women of childbearing age but 10% of patients are men

3

Systemic Lupus Erythematosus (SLE) diagnosis/ definition

Prototype of auto-immune disease
Onset maybe acute, episodic, or insidious
Anything can happen to any organ system at any time.
Antinuclear antibodies are almost always present
Serositis & Immune complexes can be the hallmark and pathogenesis of the disease.

4

What is the average time to diagnose SLE?

Average time of two years between initial symptomatology and definitive diagnosis¹.

Analysis of stored serum of 130 military recruits who later developed lupus revealed that autoantibodies were present in the serum up to 9 years prior to the onset of clinical disease²

5

Epidemiology of SLE

Prevalence rates 40-50 per 100,000 in U.S.

Incidence has tripled in the last 40 years

Incidence rate 2 to 8 per 100,000/year in NA, SA, and Europe

Women affected 9x more frequently- 20-40 year olds most frequent onset in age

African-Americans and Hispanics are affected more frequently than whites and have a higher disease mortality

Urban areas > rural areas

Cases of lupus increase the closer to the equator

65% of patients are between 16 and 55

20% prior to age 16, 15% after age 55

Men have a higher 1 yr mortality rate

The elderly tend to have milder SLE

6

ACR Criteria for Classification of SLE

Rules for using the criteria:

A patient who exhibits any 4 of these 11 criteria is classified as having SLE

Criteria need not be present simultaneously

SLE evolves over time.

7

What are the 11 ACR criteria for classification of SLE

1.Malar rash 6. Serositis
2.Discoid rash 7. Renal disorder
3.Photosensitivity 8. Neurologic disorder
4.Oral ulcers 9. Hematologic disorder
5.Arthritis 10. Immunologic disorder
11. Antinuclear antibody

8

Malar Rash

Photosensitive
Spares nasolabial folds*
Fixed erythema, flat or raised, over malar eminences
May be hyperpigmented in darker skinned individuals

9

Ddx for malar rash

Glucocorticoid-induced atrophy
Chloasma/ Melasma
Benign flushing
Rosacea
Dermatitis

10

Mucocutaneous Lesions Patterns

Acute - localized or generalized

Subacute – Annular or psoriaform

Chronic – Discoid localized or generalized

Nonspecific – Vasculitis, alopecia, livedo reticularis, Raynaud’s, urticaria, lichen planus

11

Lupus alopecia

Most SLE patients
Localized or generalized
Scalp, eyebrows,
eyelashes, body hair
Increased with disease
activity

Subsides as disease subsides

12

Discoid Lupus subset

Patients with DLE have 90-95% do not go on to SLE

5 to 10% risk of developing SLE

This form of SLE tends to be mild

The more numerous and widespread the lesions are the higher the risk of developing SLE

Occurs in 25% of lupus patients but can occur in patients without systemic lupus

13

How would you describe discoid rash

Erythematous raised patches
Adherent keratotic scale
Follicular plugging
Atrophic scarring may occur as
lesions age

LOOK IN EARS!
DLE on arm
DLE in ear

14

Subacute Cutaneous Lupus Subset

50% of patients with SCLE will have SLE
Positive anti –Ro (SS-A) and anti – LA (SS-B)
Photosensitive
Face usually spared
Nonscarring
Fatigue common
Arthralgia and arthritis

15

Photosensitivity

Skin rash as an unusual reaction to sunlight

16

Mucocutaneous Ulcers

Oral or nasopharyngeal ulcerations

Must be observed by physician/ health care provider

Often painless

17

Arthritis

Nonerosive arthritis involving 2
or more peripheral joints

Characterized by tenderness
swelling, or joint effusion.

18

Jaccoud’s Arthropathy

Reducible deformities
No erosions
Periarticular fibrosis and ligamentous laxity
Swan neck deformities

19

SLE - Musculoskeletal Disease

Arthralgia/Arthritis
Myalgia/Myositis
Ischemic necrosis of bone - AVN

20

Serositis

1. Pleuritis - convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion
or

2. Pericarditis - documented by ECG or rub or evidence of pericardial effusion

21

SLE - Pleuropulmonary disease

Pleuritis/Pleural effusion*
Infiltrates/Atelectasis
Acute lupus pneumonitis
Pulmonary hemorrhage
“Shrinking lung” - diaphragm dysfunction
Subclinical restrictive disease

22

SLE- Cardiac Involvement

Pericarditis
Myocarditis
Endocarditis, Libman-Sacks
Accelerated atherosclerosis

23

SLE- Renal Disorder

1. Persistent proteinuria greater than 0.5 grams per day or greater than 3+ if quantitation not performed. Protein in the urine
Usually asymptomatic
Gross hematuria
Nephrotic syndrome
Acute renal failure
Hypertension
End stage renal failure

24

SLE- Nephritis

Nephritis remains the most frequent cause of disease-related death.

25

SLE - NEUROPSYCHIATRIC INVOLVEMENT

Behavior/Personality changes
Depression
Cognitive dysfunction
Psychosis
Seizures

26

Thrombocytopenic Purpura

Non blanching
Purplish discoloration
Purpura is not a disease but indicates underlying bleeding disorder
Usually lower extremities
< 1 cm is petechiae

27

Hematologic D/O

Hemolytic anemia with reticulocytosis
OR
Leukopenia—less than 4000/mm 3 total on two separate occasions
OR
Lymphopenia—less than 1500/m 3 on two or more occasions
OR
Thrombocytopenia—less than 100,000/m 3 in the absence of
offending drugs

28

Immunologic D/O

1. Anti-dsDNA: antibody to native DNA in abnormal titer
or
2. Anti-Sm: presence of antibody to Sm nuclear antigen
or

3. Antiphospholipid antibodies by positive IgG or IgM anticardiolipin antibodies (not IgA) or a positive test for lupus anticoagulant, or a false positive test for syphilis (RPR) for 6 months

29

SLE labs

Antibodies to dsDNA are present in 70% of lupus patients and are >95% specific for lupus
Anti-Sm (Smith) + in up to 30% of lupus patients and is pathognomonic
Decreased C3 and C4 indicate activity
Anti-Ro (SS-A) congenital heart block

30

Antinuclear Antibodies---ANA

Low titer positive ANA in normal population increases with age

Patterns of ANA immunofluorescence vary.

-SLE is commonly associated with peripheral or speckled patterns

-Homogeneous patterns are associated with drug induced lupus.

-First degree relatives of patients with SLE are likely to be ANA positive

32% of relatives had antinuclear antibodies, compared with 1.5% of the healthy controls

31

ANA sensitivity and specificity

Sensitivity 95%
Specificity very low
Positive in many autoimmune diseases
10 to 35% of adults aged >65 have + ANAs
and do not have Lupus or other autoimmune disease
Negative ANA occurs in less then 3% of lupus patients, (+ SS-A or + RNP)

32

ANAs do not?

ANAs do not correlate with disease activity

Do not forget other causes of a positive ANA

A patient with a negative ANA has less than a 3% chance of developing SLE

33

Other causes for a positive ANA

RA, Sjogren’s, Scleroderma, Myositis
Infections (viral or bacterial)
Thyroiditis or Graves
Cancers such as melanoma, breast, lung
GI ( hepatitis, UC, Crohn’s, Primary Biliary Cirrhosis
Lung (pulmonary fibrosis, TB)
Medications

34

Common manifestations not a part of ACR criteria

Fever and Constitutional Symptoms 42%
Raynaud's Phenomenon 22%
Alopecia 18%
Cutaneous Vasculitis

35

SLE clustering symptoms

Cutaneous, articular and renal manifestations tend to appear “together”

CNS thrombotic and muscular symptoms also cluster, although to a lesser extent

36

Summary of Lupus

Do not order ANAs for broken bones
Most ANAs do not mean Lupus
Know what you are going to do with the result of an ordered lab test.
Numerous potential complications loom behind the scenes and must be anticipated and monitored
Lupus can be treated
Lupus patients can live and accomplish goals just like everyone else

37

Management Goals for SLE

Counseling, education

Recommend adequate rest and activity (30 to 60 min pm nap)

Decrease inflammation; prevent end-organ injury failure

Preserve renal function; provide HBP Rx; prevent flare

Provide photo protection

Maintain up-to-date immunizations

Management of infection

Minimize osteoporosis

Eye examinations for Plaquenil toxicity (q 6 to 12 months)

Identify patients at risk of thrombo-occlusive events

Evaluate and treat ASHD risk; dyslipoproteinemia, etc.

Family planning/contraceptive issues

Lupus support groups and Arthritis Foundation

38

Medications for SLE

Steroid sparing agents
****Hydroxychloroquine (Plaquenil)
Thalidomide (for mucocutaneous manifestations)
Dapsone (for panniculitis, vasculitis)
Danazol (for thrombocytopenia)
General Immunosuppressants
****Azathioprine (Imuran)
Cyclophosphamide (Cytoxan)
Leflunomide (Arava)
Methotrexate
***Mycophenolate Mofetil (CellCept)
Biologics
Abatacept (Orencia)
Rituximab (Rituxan)
*** Belimumab (Benlysta) NEW
Other
IVIg

39

Drug induced lupus

Minocycline (Minocin)
Procainamide
Isoniazid (INH)
Phenytoin (Dilantin)
Carbamazepine (Tegretol)
Hydralazine (Apresoline)
Quinidine (Quinaglute)
Infliximab, Etanercept, Adalimumab, Simponi™, Cimzia™

40

Dx and treatment of drug induced lupus

15,000 to 20,000 new cases/yr in U.S.
Positive ANA and one clinical feature of Lupus
Antihistone antibody in 95% of cases
Fever, myalgias, rash, arthritis, serositis
Stop drug, consider anti-histone ABs
Rx with NSAIDS, steroids