Lupus Flashcards
What are the 5 types of Lupus?
Subacute Lupus Erythematosus Discoid Lupus Drug Induced Lupus Systemic Lupus Erythematosus Neonatal Lupus*
What is Lupus?
- Lupus is a systemic autoimmune disease in which the body loses tolerance to self
- Can affect virtually any organ in the body and initial symptoms are often nonspecific, making it very difficult to diagnose
- Typical autoantibodies seen are those reacting against nuclear components of the cell
- Most commonly seen in women of childbearing age but 10% of patients are men
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.
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
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
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.
What are the 11 ACR criteria for classification of SLE
- Malar rash 6. Serositis
- Discoid rash 7. Renal disorder
- Photosensitivity 8. Neurologic disorder
- Oral ulcers 9. Hematologic disorder
- Arthritis 10. Immunologic disorder
11. Antinuclear antibody
Malar Rash
Photosensitive
Spares nasolabial folds*
Fixed erythema, flat or raised, over malar eminences
May be hyperpigmented in darker skinned individuals
Ddx for malar rash
Glucocorticoid-induced atrophy Chloasma/ Melasma Benign flushing Rosacea Dermatitis
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
Lupus alopecia
Most SLE patients Localized or generalized Scalp, eyebrows, eyelashes, body hair Increased with disease activity
Subsides as disease subsides
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
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
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
Photosensitivity
Skin rash as an unusual reaction to sunlight
Mucocutaneous Ulcers
Oral or nasopharyngeal ulcerations
Must be observed by physician/ health care provider
Often painless