7: Lupus Flashcards
T/F SLE is an organ-specific disease like thyroiditis and diabetes.
False. It is a multisystem autoimmune disease that affects many organs.
Does SLE favor men or women?
Women (9:1)
When is the typical onset of SLE?
Reproductive years
T/F SLE has a strong minority representation.
True
What are constitutional symptoms of SLE (3)?
- Fatigue
- Fever
- Weight loss
What are SLE triggers (5)?
- Recent sun exposure.
- Emotional stress.
- Infection
- Drugs (sulfonamides, Hydralazine).
- Surgery
What is the criteria for diagnosing SLE (12)?
Must meet 4: Mucocutaneous: 1. Malar rash 2. Discoid lesions 3. Photosensitivity 4. Oral ulcers Symptomatic Organs: 5. Arthritis 6. Serositis 7. Neuropsychiatric 8. Renal Labs: 9. ANA 10. Hematological (lymphopenia, leukopenia, hemolytic anemia, thrombocytopenia) 11. Immunologic (anti-ds-DNA, anti-Smith, antiphospholipid) 12. Nephritis (RBC casts, proteinuria)
T/F SLE patients who die within 5 years usually have active disease.
True. Over 90% survive at least 2 years after diagnosis. Currently 80-90% survive 10 years. Late deaths are often d/t CVD.
T/F SLE is curable.
False. Remission is possible, but not a cure.
Which system is the most commonly affected?
Cutaneous (80-90%)
What are the 3 most common types of skin lesions with SLE?
- Acute
- Subacute
- Discoid (chronic)
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Rash is erythematous and edematous. Seen on chin and forehead, but not nasolabial folds.
Acute Cutaneous Lupus (Butterfly Rash)
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Primarily affects Caucasian females. It is typically symmetric, widespread, superficial, and non-scarring. Seen most often in sun-exposed areas.
Subacute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Typically on the face, scalp, pinnae, behind ears, and neck. Seen in non-exposed areas. Can exist as part of systemic disease or in isolation.
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Central atrophic scarring with active indurated erythema at periphery. It can involve irreversible alopecia from follicular destruction.
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Erythematous patches on nose and medial cheeks (malar).
Acute Cutaneous Lupus
What drugs can trigger SLE (6)?
- Clopidogrel
- Hydralazine
- Isoniazid
- Procainamide
- Ticlopidine
- Minocycline
Is a malar rash scarring?
No
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Involves erythematous annular or polycyclic plaques with scaling (psoriasiform).
Subacute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Rash is photosensitive and may involve dorsal hands, arms, trunk.
Acute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Strongly associated with active SLE.
Acute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
In sun-exposed areas, is possibility of smaller plaques and papules.
Subacute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Progresses to internal disease in 10%–15% of patients.
Subacute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Is strongly associated with anti-Ro antibodies (60%–100%). Is associated with HLA-A1, B8, DR3.
Subacute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Can be triggered by drugs (HCTZ, Terbinafine, Griseofulvin, ACE inhibitors, calcium channel blockers, NSAIDs, Etanercept, Thalidomide, antihistamines).
Subacute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
On biopsy, inflammatory infiltrate in upper dermis.
Subacute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
On biopsy, inflammatory infiltrate in superficial dermis.
Acute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Also known as chronic cutaneous lupus.
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Presents on head and neck.
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Progresses to SLE in 5%–15% of patients. Can be increased risk if lesions below neck (20%).
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Represents 15%–30% of SLE patients.
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Rarely triggered by drugs (Fluorouracil).
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
On biopsy, inflammatory infiltrate from superficial to deep dermis, involvement with adnexal structures.
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Rash is scarring.
Discoid (Chronic) Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Rash is not scarring.
Acute Cutaneous Lupus
Subacute Cutaneous Lupus
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Alopecia that can be diffuse or patchy. If caused by discoid, is irreversible. This type can be reversible.
Mucocutaneous Involvement
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement? Mucosal lesions (mouth, nose, anogenital area). Typically painless. Can be painful if central depression occurs.
Mucocutaneous Involvement
Acute cutaneous lupus, subacute cutaneous lupus, discoid lupus, or mucocutaneous involvement?
Vasculitis (urticaria, palpable purpura, nail fold/digital ulcerations, papules of pulps of fingers/palms, splinter hemorrhages).
Mucocutaneous Involvement
What areas are important to inspect with SLE (6)?
- Scalp
- Pinnae
- Behind Ears
- Palate
- Fingertips
- Palms
T/F SLE can present with symmetrical arthritis just like RA.
True, but it can be brief or persistent, unlike RA.
The _____ is the signature organ affected by SLE.
Kidney
Studies all show _____ as key predictor of bad outcome.
Lupus nephritis
_____ affects 50-66% of patients.
Renal disease
What leads to diagnosis of renal disease in SLE?
Proteinuria
What is a very important test in SLE for health maintenance?
Urinalysis for protein
_____ manifestations in 66% of SLE patients.
Neuropsychiatric
Which psychiatric disorders are common SLE (3)?
- Mood disorders
- Anxiety
- Psychosis
Which cognitive disorders are common in SLE (3)?
- Attention deficit
- Lack of concentration
- Impaired memory or word finding issues
Which neurological complications are common in SLE (4)?
- Acute confusional state
- LOC or arousal issues
- Seizures/headaches that are non-responsive
- Visual defects, ptosis, nystagmus, vertigo, peripheral neuropathy
_____ is the most frequent cardiac complication (6-45%).
Pericarditis
T/F Primary myocardial involvement is rare.
True. Less than 10%.
T/F There can be severe pain from pleuritis.
True. Seen in more than 30%. Not as deadly as cardiac and renal issues.
_____ is pleuritic chest pain with cough, hemoptysis, and dyspnea and no infection is present.
Acute lupus pneumonitis
_____ is the most important treatment.
Sunblock. Sunburns activate lupus.
_____ is a key indicator of lupus.
Photosensitivity
_____ alleviate pain from arthralgias and serositis.
NSAIDs.
Kidney involvement and risk of thrombosis are associated with _____.
NSAIDs
_____ is used for skin and joint manifestations.
Plaquenil
_____ can occur and should be monitored on Plaquenil.
Retinal toxicity
_____ is topically used for skin rashes, except on face.
Corticosteroids
_____ is systemically used in low doses for skin rashes and joint involvement.
Corticosteroids
Moderate doses of corticosteroids combat aggressive skin disease, _____, and hematologic abnormalities.
Serositis
High doses of steroids are used for what (4)?
- Nephritis
- Cerebritis
- Vasculitis
- Life-threatening hematologic abnormalities
These drugs are superior to steroids alone and are shown to improve outcomes in severe lupus.
Immunosuppressants (Methotrexate, Cellcept, Azathioprine, Cytoxan)
_____ is the first FDA-approved treatment for lupus in 50 years.
Benlysta (Belimumab). Long-term effectiveness unknown.
What pharm category is Benlysta (Belimumab) and why is this important?
B-lymphocyte Stimulator Inhibitor. This is impotant b/c B lymphocytes are overexpressed.
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Frequency is greater than 90%. Nonspecific. Diagnostic only.
ANA
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Frequency is 40-60%. Found in nephritis. May predict flare.
Anti-dsDNA
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Frequency is 30-40%. Found in Raynaud’s. Not related to SLE activity.
Anti-RNP
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Frequency is 30-40%. Found in MSK disease. Not related to SLE activity.
Anti-RNP
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Frequency is 10-45%. Associated with dry eye/mouth, SCLE, neonatal lupus, and photosensitivity. Not related to SLE activity.
Anti-SSA/Ro
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Frequency is 10-15%. Associated with dry eye/mouth, SCLE, neonatal lupus, and photosensitivity. Not related to SLE activity.
Anti-SSB/LA
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Frequency is 30%. Associated with clotting issues. Varied relationship to SLE activity.
Antiphospholipid
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Presence almost always indicates SLE.
Anti-SM
ANA, anti-dsDNA, anti-RNP, anti-SSA/Ro, anti-SSB/LA, antiphosholipid, anti-SM, or complements C3/4?
Inflammation from SLE severely reduces these. Low numbers may indicate active SLE.
Complements C3/4
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?
- There is an overwhelming female predominance (approx. 9:1).
- The typical onset of lupus is during the reproductive years.
- There is a strong minority representation.
- All of the above.
- All of the above.
What lupus rashes cause scarring of the skin?
- All rashes/lesions cause scarring of the skin.
- Subacute lupus lesions cause scarring.
- Discoid or chronic lesions cause scarring.
- Acute butterfly lesions cause scarring.
- Discoid or chronic lesions cause scarring.
Monitoring lupus activity is important especially to watch for involvement of other systems. What autoantibodies should be monitored routinely?
- SSA and SSA
- dsDNA, C3, and C4
- Antiphospholipid antibody and ANA
- Anti Sm
- dsDNA, C3, and C4