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Lupus Flashcards

1
Q

What are the 5 types of Lupus?

A 
Subacute Lupus Erythematosus
Discoid Lupus
Drug Induced Lupus
Systemic Lupus Erythematosus
Neonatal Lupus*
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2
Q

What is Lupus?

A
  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
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3
Q

Systemic Lupus Erythematosus (SLE) diagnosis/ definition

A

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.

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4
Q

What is the average time to diagnose SLE?

A

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

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5
Q

Epidemiology of SLE

A

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

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6
Q

ACR Criteria for Classification of SLE

A

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.
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7
Q

What are the 11 ACR criteria for classification of SLE

A
  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
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8
Q

Malar Rash

A

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

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9
Q

Ddx for malar rash

A 
Glucocorticoid-induced atrophy
Chloasma/ Melasma 
Benign flushing
Rosacea
Dermatitis
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10
Q

Mucocutaneous Lesions Patterns

A

Acute - localized or generalized

Subacute – Annular or psoriaform

Chronic – Discoid localized or generalized

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

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11
Q

Lupus alopecia

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

Subsides as disease subsides

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12
Q

Discoid Lupus subset

A

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

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13
Q

How would you describe discoid rash

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

LOOK IN EARS!
DLE on arm
DLE in ear

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14
Q

Subacute Cutaneous Lupus Subset

A 
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
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15
Q

Photosensitivity

A

Skin rash as an unusual reaction to sunlight

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16
Q

Mucocutaneous Ulcers

A

Oral or nasopharyngeal ulcerations

Must be observed by physician/ health care provider

Often painless

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17
Q

Arthritis

A

Nonerosive arthritis involving 2
or more peripheral joints

Characterized by tenderness
swelling, or joint effusion.

18
Q

Jaccoud’s Arthropathy

A

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

19
Q

SLE - Musculoskeletal Disease

A

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

20
Q

Serositis

A
  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
Q

SLE - Pleuropulmonary disease

A 
Pleuritis/Pleural effusion*
Infiltrates/Atelectasis
Acute lupus pneumonitis
Pulmonary hemorrhage
“Shrinking lung” - diaphragm dysfunction
Subclinical restrictive disease
22
Q

SLE- Cardiac Involvement

A

Pericarditis
Myocarditis
Endocarditis, Libman-Sacks
Accelerated atherosclerosis

23
Q

SLE- Renal Disorder

A 
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
Q

SLE- Nephritis

A

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

Rheumatology (9 decks)

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