Part 15 - Allergology, Immunology, Rheumatology Flashcards
In SLE, what complement deficiencies are involved?
C1q,r,s
C2
C4
People with ______ karyotype have a significantly increased risk for SLE.
XXY (Klinefelter’s)
In SLE, at what skin layer (on skin biopsies) is deposition of immunoglobulin found?
Dermal-epidermal junction (DEJ)
At what class of disease is treatment for lupus nephritis NOT recommended?
Class I or II
What is the name of the criteria for classification of SLE?
Systemic Lupus International Collaborating Clinic (SLICC)
What is the most common pattern of vasculitis in SLE, that may also indicate active disease?
Leukocytoclastic vasculitis
In SLE, ANA is positive in how many percent of patients during the course of disease?
> 98%
What is the best screening test for SLE?
ANA
What autoantibodies are specific for SLE?
Anti-dsDNA
Anti-Sm
What autoantibody is SLE-specific and may correlate with disease activity?
Anti-dsDNA
Which autoantibody is more frequently seen in drug-induced lupus than in SLE?
Antihistone
Which autoantibody, when produces a positive test in serum correlates with depression or psychosis due to CNS lupus?
Antiribosomal P
In SLE, what is the gene defect found on the X chromosome?
Mutation in TREX1
What are the types/classes of lupus nephritis?
Class I: minimal mesangial Class II: mesangial proliferative Class III: focal Class IV: diffuse Class V: membranous Class VI: advanced sclerotic
What is “Rhupus”?
Rheumatoid-like arthritis with erosions and fulfill criteria for both RA and SLE
In SLE, what diagnosis will you consider if arthralgia is persistent in a single joint?
Ischemic necrosis of bone
What is the most common chronic dermatitis in lupus?
Discoid lupus erythematosus
What is the treatment for discoid lupus erythematosus?
Topical or locally injected glucocorticoids and systemic antimalarials
What is the most serious manifestation of SLE?
Nephritis
What is the most common manifestation of diffuse CNS lupus?
Cognitive dysfunction
In SLE, at what dose of Prednisone does glucocorticoid-induced psychosis occur?
> or = to 40 mg
Endocarditis of SLE is called?
Libman-Sacks endocarditis
What is the most common pulmonary manifestation of SLE?
Pleuritis with or without pleural effusion
Life threatening pulmonary manifestations of SLE
Interstitial fibrosis
Shrinking lung syndrome
Intraalveolar hemorrhage
What is the most frequent cardiac manifestation of SLE?
Pericarditis
Endocardial involvement in SLE may lead to insufficiencies commonly on what valves?
Mitral or aortic
What is the most frequent hematologic manifestation of SLE?
Anemia
usually normochromic normocytic
What are serious ocular manifestations of SLE that may cause blindness?
Retinal vasculitis
Optic neuritis
Three antiphospholipid antibodies
Anticardiolipin
anti-B2 glycoprotein
Lupus anticoagulant
What titer level of IgG anticardiolipin is considered high?
> 40 IU
What is the criteria for diagnosing APS?
Presence of 1 or more clotting episodes and/or repeated fetal losses
PLUS
at least 2 positive tests for antiphospholipid antibodies at least 12 weeks apart
What autoantibody indicates increased risk for neonatal lupus, sicca syndrome, and subacute cutaneous lupus?
Anti-Ro (SS-A)
What treatment has been shown to prevent SLE flares in patients with rising anti-DNA plus falling complement?
30 mg Prednisone daily for 2 weeks
Antimalarials used for treatment of SLE
Hydroxychloroquine
Chloroquine
Quinacrine
Mainstay of treatment for any inflammatory life-threatening manifestations of SLE
Systemic glucocorticoids
(0.5-1 mg/kg per day PO
OR
500-1000 mg of methylprednisolone succinate IV daily for 3 days followed by 0.5-1 mg/kg of daily prednisone or equivalent
Cytotoxic/immunosuppressive agents added to glucocorticoids for treatment of severe SLE
Cyclophosphamide or mycophenolate mofetil
Alkylating agent used to treat serious SLE
Cyclophosphamide
Lymphocyte specific inhibitor of inosine monophosphatase / purine synthesis used to treat serious SLE
Mycophenolate mofetil
Common manifestation of mycophenolate mofetil toxicity
Diarrhea
Common manifestations of cyclophosphamide toxicity
Amenorrhea
Leukopenia
Nausea
Common effect of high dose cyclophosphamide (not low dose)
Ovarian failure
Given to reduce incidence of ovarian failure due to high-dose cyclophosphamide therapy for SLE
Gonadotropin-releasing hormone agonist (ex. Leuprolide 3.75 mg IM) prior to each cyclophosphamide dose
Can be used if necessary to control active SLE in pregnant patients (if conventional therapy do not work)
Azathioprine
A folinic acid antagonist that may be used for treatment of arthritis and dermatitis due to SLE (but not in nephritis or life-threatening disease)
Methotrexate
Anti-BLys agent used for treatment of seropositive patients with SLE who have failed standard treatments
Belimumab
Widely used scoring to measure SLE disease activity
Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)
What score in SLEDAI reflects clinically active disease in SLE?
> 3
Induction therapy of choice for crescentic lupus nepritis
High-dose cyclophosphamide
Placental enzyme that deactivates glucocorticoids
11-B-dehydrogenase 2
Pregnancy category of methotrexate
Category X
Pregnancy category of azathioprine, hydroxychloroquine, mycophenolate mofetil, and cyclophosphamide
Category D
Pregnancy category of cyclosporine, tacrolimus, rituximab
Category C
Pregnancy category of glucocorticoids
Category A
Active SLE in pregnant women should be controlled with?
Hydroxychloroquine > prednisone/prednisolone (lowest possible dose) > azathioprine
