Systemic Lupus Erythematosus Flashcards
How many criteria (symptoms) must be met for a patient to be diagnosed with Lupus?
Must meet at least 4 criteria
What constitutional symptoms predominate in lupus?
Fatigue
Fever
weight loss
Does Lupus usually affect men or women more?
Overwhelming female predominance, approximately 9:1
What age does lupus usually start showing symptoms?
Typical onset during reproductive years
T/F There is a strong minority representation with lupus?
True
What are some triggers of lupus?
Recent sun exposure Emotional stress Infection Certain drugs (Clopidogrel, Hydralazine, Isoniazid, Procainamide, Ticlopidine, Minocycline) Surgery
T/F a patient with a positive ANA definitely has Lupus?
False.
A patient with a positive ANA but no other symptoms does not have a lupus
However, if a patient meets at least 4 criteria for lupus then they MUST have a positive ANA
What is the mucocutaneous presentation of lupus?
malar rash
discoid lesions
photosensitivity
oral ulcers
What lab findings are seen in a patient with lupus?
Labs:
ANA
hematologic (e.g., lymphopenia, leukopenia, hemolytic anemia, thrombocytopenia),
immunologic (e.g., anti-ds-DNA, anti-Smith, antiphospholipid antibodies),
What are some of the criteria that must be met with lupus diagnosis? (at least four)
Criteria (must meet at least four):
Mucocutaneous: malar rash, discoid lesions, photosensitivity, oral ulcers
Symptomatic organs: arthritis, serositis, neuropsychiatric, renal
Labs: ANA, hematologic (e.g., lymphopenia, leukopenia, hemolytic anemia, thrombocytopenia), immunologic (e.g., anti-ds-DNA, anti-Smith, antiphospholipid antibodies), nephritis (e.g., RBC casts, proteinuria)
Which system is the most commonly affected by lupus?
Cutaneous system most commonly affected (80%–90%)
Which of the skin conditions is considered chronic in lupus?
discoid lesions are chronic
Which rash is considered the ACUTE rash of lupus?
typical butterfly rash
Is an erythematous and edematous eruption
Seen on chin and forehead, not nasolabial folds
Which rash is considered the ACUTE rash of lupus?
typical butterfly rash
Is an erythematous and edematous eruption
Seen on chin and forehead, not nasolabial folds
Involves erythematous patches on nose and medial cheeks (malar), butterfly rash
Is photosensitive, may also involve dorsal hands, arms, trunk
Which race is the only one that experiences the subacute rash?
Caucasians females
Describe the subacute lesions seen in lupus?
typically symmetric, widespread, superficial, nonscarring
Seen most often in sun-exposed areas
Involves erythematous annular or polycyclic plaques with scaling (psoriasiform)
In sun-exposed areas, is possibility of smaller plaques and papules
Progresses to internal disease in 10%–15% of patients
Is strongly associated with anti-Ro antibodies (60%–100%)
Is associated with HLA-A1, B8, DR3
Can be triggered by drugs (HCTZ, Terbinafine, Griseofulvin, ACE inhibitors, calcium channel blockers, NSAIDs, Etanercept, Thalidomide, antihistamines)
NO SCARRING
Describe the discoid (chronic) lesions:
Seen typically on face, scalp, pinnae, behind ears, neck
Seen in non–sun-exposed areas
Can exist as part of systemic disease or in isolation
Presents central atrophic scarring with active indurated erythema at periphery
Can involve irreversible alopecia from follicular destruction
Does not go away and is SCARRING
T/F there is NO scarring with acute skin lesions of lupus (malar rash)?
TRUE
Which type of skin lesions are typically triggered by drugs?
Acute and Subacute
Rarely chronic lesions
What are some other mucocutaneous conditions seen with lupus?
Alopecia:
Can be diffuse or patchy
Can be reversible or permanently scarring due to discoid lesions
Mucosal lesions:
Can affect the mouth, nose, anogenital area
Are typically painless, but may not be
Can involve painful ulcerations if central depression occurs
Vasculitis:
May manifest as:
Urticaria
Palpable purpura
Nailfold or digital ulcerations
Erythematous papules of pulps of fingers or palms, splinter hemorrhages
Which organ is considered the “signature” organ affected by lupus?
Kidneys
What other systems are affected by lupus?
Musculoskeletal (painful joints) Kidneys Neurological Cardiovascular Pleura and Lungs
What is the most frequent cardiac complication from lupus?
Pericarditis
Substernal or pericardial pain aggravated by inspiration, coughing, swallowing
Duration can be hours or days.
What are the pulmonary manifestations seen with lupus?
Pneumonitis pulmonary hemorrhage pulmonary hypertension shrinking lung syndrome Acute lupus pneumonitis: Pleuritic chest pain, cough with hemoptysis and dyspnea when infection ruled out
What are some nervous system manifestations with lupus?
Psychiatric
Mood disorders, anxiety, psychosis
Cognitive
Attention deficit, lack of concentration, impaired memory or word finding issues
Neurologic
Acute confusional state, disturbance in consciousness or level of arousal
Seizures and headaches that are disabling, persistent, and nonresponsive to narcotic analgesics
What eye conditions occur with lupus?
Visual defects ptosis nystagmus vertigo peripheral neuropathy of motor and sensory function
What is the most important treatment in lupus?
SUNBLOCK
Sunburns activate lupus
What is the #1 medication for treatment in lupus?
Plaquenil (hydroxychloroquine)
used for skin and joint manifestations.
What is toxicity concern with the use of plaquenil?
Retinal toxicity
Must see ophthalmologist first before beginning this medicine and then again at 6 months, 1 year and then yearly follow ups (looking for plaque deposits on the retina).
What do the corticosteroids target with lupus treatment?
Use topical and oral steroids with lupus
Topical uses
Skin rashes, except on face
Systemic uses
Low dose for skin rashes and joint involvement
Moderate dose combats aggressive skin disease, serositis, and hematologic abnormalities
High dose for nephritis, cerebritis, vasculitis, and life threatening hematologic abnormalities
Why are immunosuppressants used in treatment of lupus
Because they are steroid sparing
Which immunosuppressants are used?
Shown to improve outcomes for severe lupus Are superior to steroids alone Include: Methotrexate Cellcept (mycophenolate mofetil) Azathioprine Cytoxan
What is the newest drug for lupus?
Benlysta
First FDA-approved treatment for lupus in 50 years
T/F Lupus may be active if C3 and C4 numbers are low
TRUE
Which autoantibody is associated with Raynaud phenomenon if present?
Anti-RNP
Which autoantibody is associated with nephritis if present?
Anti-dsDNA
Which autoantibody is associated with dry eye, dry mouth, SCLE, neonatal lupus if present?
Anti-SSA/Ro
Anti-SSB/LA
Which autoantibody is associated with clotting issues if present?
Antiphospholipid
The presence of which antibody almost always is present in lupus diagnosis
Smith antibody
What lupus rashes cause scarring of the skin?
A. All rashes/lesions cause scarring of the skin.
B. Subacute lupus lesions cause scarring.
C. Discoid or chronic lesions cause scarring.
D. Acute butterfly lesions cause scarring.
C. Discoid or chronic lesions cause scarring.
T/F Lupus is a constellation of diverse signs and symptoms that change over time. A butterfly rash means the disease is active.
TRUE
What characteristics are most common in lupus?
A. There is an overwhelming female predominance (approx. 9:1).
B. The typical onset of lupus is during the reproductive years.
C. There is a strong minority representation.
D. All of the above
D. All of the above
Monitoring lupus activity is important especially to watch for involvement of other systems. What autoantibodies should be monitored routinely? A. SSA and SSA B. dsDNA, C3, and C4 C. Antiphospholipid antibody and ANA D. Anti Sm
B. dsDNA, C3, and C4
