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82 Cards in this Set

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Lupus nephritis class I
Normal
Lupus nephritis class II
Mesangial proliferation
Lupus nephritis class III
Focal segmental glomerulonephritis
Lupus nephritis class IV
Diffuse proliferative glomerulonephritis
Lupus nephritis class V
Membranous nephritis
Lupus nephritis class VI
Glomerulosclerosis
does myositis occur in SLE?
suggests overlap syndrome such as mixed connective tissue disorder
With which diseases are anti--Sm antibodies associated?
SLE
With which diseases are anti--RNP antibodies associated?
SLE, mixed connective tissue disease
With which diseases are anti-cardiolipin antibodies associated?
anti-phospholipid antibody syndrome
What risks are associated with Antiphospholipid antibody syndrome?
arterial and venous thrombosis, recurrent fetal loss, Raynaud phenomenon, and thrombocytopenia. Neurologic involvement also may occur and may include chorea and transverse myelitis. Patients also are at risk for Libman-Sacks endocarditis
Labs indicative of antiphospholipid antibody syndrome
anticardiolipin antibodies, prolonged phospholipid-dependent coagulation tests (such as PTT, abnormal dilute Russell viper-venom time), and circulating lupus anticoagulant
What is Neonatal Lupus Erythematosus?
transplacental transfer of maternal antibodies result in hematologic, cutaneous, hepatic, and cardiac abnormalities
What antibodies are most commonly involved in causin NLE?
anti-Ro- or -La
describe cutaneous lesions in NLE
present after delivery or in first few weeks, occur on face, and resolve without scarring
what is the most common heme findin in NLE?
thrombocytopenia (usually no bleeding)
When does NLE resolve?
by 6 months
What sequela of NLE is permanent?
Congenital heart block is permanent because the fetal conducting system is damaged during its development; may be detected early in pregnancy as bradycardia in the fetus; requires placement of a permanent pacemaker. mortality still is significant
What are the indications for hydroxychloroquine in SLE?
. Hydroxychloroquine is used to reduce fatigue, mucocutaneous symptoms, and alopecia
What medications are commonly used in SLE?
Sunscreen, NSAIDS, Methotrexate, hydroxychloroquine, Glucocorticoids, IV IGCyclophosphamide for serious organ involvement; sometimes Azathioprine and cyclosporine
What is indication for IV IG?
chorea and thrombocytopenia.
What are the causes of death in SLE?
renal disease, infection, and CNS disease
What are the most common presenting symptoms of SLE?
fever, rash, mucositis and arthritis. malaise and weight loss.
When are most pediatric patients diagnosed with SLE?
adolescence
female to male ratio
3:1 prior to puberty 9:1 after puberty
What ethnicity is most susceptible to SLE?
Native Americans
In what Ethnicities is SLE most severe?
African americans and Hispanics
What diseases are associated with a butterfly rash?
SLE and dermatomyositi
describe butterfly rash
photosensitive, over cheeks and crosses nasal bride, spares nasolabial folds
how does malar rash differ from rosacea?
no pustules, papules nor telaniectasia
What causes palmar erythema?
vasculitic lesions on the palms
Where are discoid lesions found?
on the scalp or extremities
Where are maculopapular lesions found?
may occur anywhere
describe the alopecia in lupus
begins in front; rarely permanent
describe the mucosal lesions in SLE?
painless
What are the most common CNS symptoms of SLE?
psychiatric manifestations (including psychosis), seizures, an headaches
less common CNS symptoms of SLE?
chorea, neuropathies, and transverse myelitis
Which layer of the heart is involved in SLE?
Any layer, but most commonly the pericardium
symptoms of pericarditis
chest pain that is exacerbated when lying down or taking a deep breath; a friction rub may be heard on auscultation
how does SLE myocarditis manifest?
CHF or arrythmia
Libman-Sacks endocarditis leads to ....
sterile verrucous vegetations
What % of people with SLE have Libman-Sacks endocarditis?
up to 50%
For what is a patient with Libman-Sacks endocarditis at risk?
SBE
Is Raynaud's phenomenon usually primary or secondary? How distinguish between the two?
usually primary. Dilated or corkscrew shaped nailbed capillaries or dropout areas (a large gap between capillaries) suggests vasculopathy (secondary)
How do you prophylax patients with frequent and prolonged Raynaud's?
nifedipine (vasodilator) in the winter
Why do SLE patients have a high risk of premature atherosclerosis?
effects of glucocorticoids; nephrotic syndrome; antiphospholipid antibody
What is the most common pulmonary manifestation of SLE in childhood?
pleuritic chest pain due to effusion
What % of adolescents with SLE have pulmonary disease?
about 60%, much is subclinical
What causes shrinking lung syndrome?
a dysfunction in the diaphragm that elevates this organ, resulting in decreased lung volume
GI symptoms in an SLE patient prompt suspicion of...
pancreatitis (primary or secondary to steroids), mesenteric vasculitis, peritonitis, hepatitis
What % of SLE pts have renal disease?
~75% of children, often within the first 2 years of the disease
musculoskeletal symptoms of SLE
arthralgias or arthritis, myalgias, proximal muscle weakness
describe SLE arthritis
non erosive and non deforming
what joints does SLE arthritis affect?
both small and large joints
what causes jaccoud arthropathy
a nonerosive but deforming arthritis that results from joint subluxation
common hematologic findings in SLE?
cytopenias (all cell lines) are common at the onset; anemia occurs in ~ 50% of patients
what kind of anemia is associated with SLE?
anemia of chronic disease (normocytic normochromic) or autoimmune hemolytic anemia
how is SLE diagnosed?
clinical manifestations, autoantibodies
Is ANA a good screening test for SLE?
sensitive but not specific; positive in 33% of general healthy population
What other diseases are associated with positive ANA?
Juvenile Idiopathic Arthritis, Dermatomyositis, Scleroderma, Thyroid disease, recent infectious illness
What auto-antibodies are more specific for SLE than ANA?
anti-dsDNA; extractable nuclear antigens anti-Sm (Smith) and anti-RNP (ribonucleoprotein), anti-Ro (also called SS-A), anti-La (also called SS-B)
I'M AN OPD RASH: I
immune manifestations: anti-ds DNA or anti-Sm, false positive RPR, antiphospholipid antibodies (anticardiolipin or lupus anticoagulant)
I'M AN OPD RASH: M
malar rash
I'M AN OPD RASH: A
anti nuclear antibody elevation
I'M AN OPD RASH: N
neurologic manifestations: seizure or psychosis
I'M AN OPD RASH: O
oral ulcers
I'M AN OPD RASH: P
PHOTOSENSITIVITY
I'M AN OPD RASH: D
DISCOID RASH
I'M AN OPD RASH: R
RENAL DISEASE
I'M AN OPD RASH: A
ARTHRITIS
I'M AN OPD RASH: S
SEROSITIS: PLEURITIS OR PERICARDITIS
I'M AN OPD RASH: H
HEMATOLOGIC: ANEMIA, LEUKOPENIA, LYMPHOPENIA, THROMBOCYTOPENIA
Which antibody is most known for its association with neonatal lupus erythematosis (NLE) and the development of congenital heart block?
anti-Ro antibodies
serologic markers of active SLE?
reduced levels of C3 or C4, elevated anti-ds DNA, ESR and CRP
% of anti-ds DNA in SLE
73%
% SLE positive for anti-Ro (anti-SSA)
40%
% anti-SSB (anti-La) in SLE
10-15%
% of anti-Sm antibodies in SLE
20-30%
% of anti-cardiolipin in SLE
37%
% anti-RNP antibodies in SLE
15%
anti-Ro associated with....
SLE (especially with cutaneous manifestations), Sjogren syndrome, NLE
Which diseases are associated with anti-La?
SLE, sjogren, NLE