Flashcards in 32. Systemic lupus erythematosus Deck (53)
classic linical presentation of SLE
rash , joint, pain and fever
SLE is MC ( sex, age origin)
female of reprodructive age and African descent
Lupus endocarditis - name
Lidman - Sacks endocarditits
Lidman - Sacks endocarditits of lupus
nonbacterial, wart - lile ( verrucous ) vegetations on both sides of valve ( mitral ) or aortic valve
Lupus nephritis is a result of
type III hypersensitivity
Lupus nephritis - MC
nephritic- diffuse proliferative glomerulonephritis nephrotic - membranous glomerulonephritis
MC and severe type is diffuse proliferative
Common causes of death in SLE
1. cardiovasuclar disease
3. Renal disease
SLE symptoms / findings / treatmnent - MNEMONIC RASH OR PAIN
RASH OR PAIN
Rash, Arhtitis, Soft tissues/ Serosistis, Hematologic disorders, Oral and nasopharyngeal ulcers , Renal disease/ Raynaud phenomenon, Photosensitivity, Antinuclear antibodies
Immunologic disorder ( anti -ds DNA, anti - SM, antiphopholipids) , Neurologic disorders
SLE - hematologic disorders - example
it can affect any layer of the heart ( endo, myi, pericarditis
SLE - rash
malar or discoid
SLE - lab findings
1. antinuclear antibodies ( ANA)
2. anti- dsDNA antibodies
3. anti - Smith antibodies
4. decreased C3 C4 CH50
5. Anti- histone antibodies
SLE - decreased C 3 C4 CH50 due to
immune complex formation
SLE - sensitive, not specidic test
SLE - specific, poor prognosis test
anti- dsDNA ( renal disease)
SLE - specific , not prognostic test
SLE - sensitive for drug induced lupus
Anti- Smith antibodies - against ....
antiphospholipid syndrome is a ... or .... disorder
1ry or 2ry autoimmune disorder
antiphospholipid syndrome as a 2ry autoimmune disorder is MC in
antiphospholipid syndrome - autoantibodies against
antiphospholipid syndrome - autoantibodies against
diagnosis of antiphospholipid syndrome is based on
clinical criteria and lab findings
clinical criteria of antiphospholipid syndrome
1. history of thrombosis ( arterial or venous )
2. recurrent spontaneous abortion
antiphospholipid syndrome arterial or venous thrombosis includes
2. hepatic vein thrombosis
3. placental thrombodis
antiphosphoolipid syndrome - lab findings
1. lupus anticoagulants
3. anti - β2 - glycoprotein antibodies
lupus anticoagulant is an
immunoglobulin that binds to phospholipids and proteins associated with cell membrane
antiphospholipid syndrome - lupus anticoagulants and anticardiolipin antibodies can cause .... ( lab)
false positive VDRL/RPR
falsely elevated PTT
antiphospholipid syndrome - treatment
Drug induced lupus - drugs
Lupus endocarditis - valves
Mixed onnective tissues disease - clinical presentation
Feature of SLE, systemic sclerosis, and /or polyomyositis
Mixed connective tissues disease - Labs
anti - U1 RNP antibodies ( speckled ANA)
SLE - lab findings ( and their characteristics )
1. antinuclear antibodies ( ANA) - sensitive, not specific
2. anti-ds DNA antibodies - specific , poor prognosis ( renal disease)
3. anti- Smith antibodies - specific , not prognostic ( directed against snRNPs)
4. decreased C3, C4, CH50- due to immune complex formation
5. Anti- histone antibodies - sensitive dor drug -induced lupus )
• What demographic is most commonly affected by lupus?
Females of reproductive age and African descent
• A patient with lupus dies of complications of renal disease. Autopsy of her heart valves may show what pathology?
Nonbacterial verrucous endocarditis (i.e., Libman-Sacks endocarditis) with vegetations on both sides of the valves
• A man with glomerulonephritis, Raynaud phenomenon, and photosensitivity is VDRL/RPR positive. What antibodies are specific for this disease?
Anti-dsDNA and anti-Smith antibodies (the patient likely has systemic lupus erythematosus)
• A 19-year-old woman with a malar rash notes that her hands turn white and then blue when she is cold. What condition does she likely have?
Raynaud phenomenon, which is associated with systemic lupus erythematosus
• A 27-year-old woman with a history of lupus develops hematuria. What histologic finding(s) do you expect on renal biopsy?
Evidence of diffuse proliferative glomerulonephritis (a common cause of nephritic syndrome in lupus patients)
• A lupus patient suffers from nephrotic syndrome. What type of glomerulonephropathy do you expect her to have?
• Patients with systemic lupus erythematosus may be diagnosed falsely with which disease based on serologic testing? Why does this happen?
Syphilis (due to a positive VDRL/RPR test); antiphospholipid antibodies cross-react with the cardiolipin antigen used in the test
• A patient presents with a malar rash, mouth ulcers, and arthritis. She is about to go on vacation to Hawaii. What do you tell her?
She has systemic lupus erythematosus and therefore could be photosensitive—she should protect against sun exposure
• Which serologic findings (antibodies) can be positive in patients with lupus?
Antinuclear, anti-double-stranded DNA, and anti-Smith antibodies (antihistone antibodies in cases of drug-induced lupus)
• A man on procainamide develops arthritis, fatigue, and pericarditis. His serum might reveal the presence of which antibody?
Antihistone antibodies, specific for drug-induced lupus
• What are possible mucocutaneous manifestations of systemic lupus erythematosus?
Malar or discoid rashes, mucositis (oropharyngeal ulcers), photosensitivity
• Which antibody is specific for lupus but is not prognostic?
Which antibody is specific for lupus and predicts a poor prognosis?
Anti-double-stranded DNA antibodies
• Which antibody is sensitive but not specific for lupus and is used as a primary screening test?
• A young woman has leukopenia and oropharyngeal ulcers. She also has diffuse joint pains and a facial rash. What treatments can be offered?
Treat with NSAIDs, steroids, immunosuppressants, and/or hydroxychloroquine (this is likely systemic lupus erythematosus)
• Renal biopsy of a lupus patient shows immune complex deposition. Which lab findings would support the formation of these complexes?
Decreased C3, C4, and CH50 levels
• A young woman presents with a history of seizures and psychotic episodes. Is lupus on your differential diagnosis?
Yes, as systemic lupus erythematosus may present with neurologic abnormalities such as seizures and psychosis