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MKSAP 16

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action of BAFF

mediates the maturation and survival of


immature B cells to antibody-secreting
plasmablasts, and its levels are elevated
in patients with SLE and Sjgren
syndrome

acute vs chronic
inflamm or
noninflamm
oligoarthritis

Acute inflammatory oligoarthritis by


gonorrhea or rheumatic fever. Chronic
inflammatory oligoarthritis by
spondyloarthritis or a connective tissue
disease. Chronic noninflammatory
oligoarthritis is usually caused by
osteoarthritis.

advantage of
febuxostat over
allopurinol

approved for patients with mild to


moderate kidney failure

adverse effects of
abatacept

increased risk of lymphoma and lung


cancer as well as flares of COPD

adverse effects of
allopurinol

rash, bone marrow failure, hepatic


failure, and Stevens-Johnson syndrome

adverse effects of
uricase and
rasburicase

highly immunogenic and is not an


option for chronic use.

adverse events
related to
cyclophosphamide

leukopenia, anemia, increased rate of


bacterial and fungal infections,
hemorrhagic cystitis and bladder
cancer, lymphoma, and other (longterm) malignancies

agents often used


in treatment of RA
in pregnant

Hydroxychloroquine and sulfasalazine

ANCA-associated
vasculitides

granulomatosis with polyangiitis (also


known as Wegener granulomatosis),
microscopic polyangiitis, and ChurgStrauss syndrome

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12.

arthrocentesis
counts suggestive
of septic arthritis

greater than 50,000/L

corticosteroidsparing or
maintenance agent
in lupus,
vasculitis, and
polymyositis

azothioprine

cyclophosphamide
and pregnancy

never used unless life of mother is at


stake

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different
manifestations of
GCdepending on
blood vessel
involved

Cranial vessel involvement includes


jaw claudication (due to ischemia of
the masseter or temporalis muscles),
scalp tenderness, and temporal or
occipital headache. Inflammation of
the ophthalmic artery may cause
ischemia of the optic nerve, resulting
in visual loss. Involvement of the
subclavian vessels may result in upper
extremity limb claudication or
subclavian steal syndrome. Aortic
valve regurgitation may occur with
GCA involvement of the proximal
ascending aorta.

effect of
sulfasalazine in
male fertility

cause reversible oligospermia

effective as initial
monotherapy for
patients with
rheumatoid
arthritis of any
duration or degree
of activity

methotrexate or leflunomide

enzyme tht
metabolizes
azathioprine

thiopurine methyltransferase (TPMT)

extra-articular
sequelae of RA

Felty syndrom; rheumatoid vasculitis;


rheumatoid nodules; scleritis with
scleral ulceration; and interstitial lung
disease

Felty syndrome

pancytopenia, splenomegaly, and leg


ulcers

glomerulonephritis
associated with
small vessel
vasculitis

"pauci-immune" (lacking immune


complexes) glomerulonephritis

how does
methotrexate work
in rheuma
conditions?

mechanism of its benefit in rheumatic


diseases may relate not to folic acid
antagonism but to its ability to raise
extracellular adenosine levels

how is diagnosis of
Wegener,ls
granulomatosis
established?

lung or kidney biopsy

how is Takayasu
diagnosis
established?

demonstration of characteristic
narrowing of the aorta or its major
branch vessels

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34.

human genomederived monoclonal


reagent with
specificity for the
p40 subunit shared
by IL-12 and IL-23

ustekinumab

hydroxychloroquine
pand pregnancy

pregnancy risk category C


medication, expert consensus states
that it is relatively safe in pregnancy
and should not be discontinued

indications for
colchicine

gout, pseudogout, and familial


Mediterranean fever

initial
monotherwpy for
RA with moderate
or high disease
activity

TNF- inhibitors (etanercept,


infliximab, adalimumab, golimumab,
and certolizumab pegol)

initial presentation
of Kawasaki

fever with nonexudative


conjunctivitis, pleomorphic
erythematous rash, and oral
mucositis

laboratory
abnormalities are
useful in
demonstrating the
presence of an
inflammatory
process

elevated C-reactive protein (CRP)


level, anemia of chronic disease,
thrombocytosis, and
hypoalbuminemia

large vessel
vasculitis

Giant cell, Takayasu and polymyalgia


rheumatica

leflunomide and
pregnancy

extremelynlong half life, needs


cholestyramine regimen (three times
daily for 8 days) to remove

MOA
hydroxychloroquine
in rheumatologic
disease

inhibition of antigen processing and


costimulatory activation

MOA leflunomide

blocks pyrimidine biosynthesis and


targets lymphocytes, which lack
pyrimidine salvage pathways

MOA mycophenolate
mofetil

inhibits inosine monophosphate


dehydrogenase (an enzyme in the
purine synthetic pathway).
Mycophenolic acid preferentially
inhibits T and B lymphocytes; likely
as effective as cyclophosphamide

38.

MOA of probenecid

inhibits the renal tubule transporter


URAT1 to block retention of urate

39.

MOA rituximab

chimeric monoclonal antibody that


depletes CD20+ B-cell lymphocytes,
lasts 6-12 months

40.

MOA Tocilizumab

chimeric (mouse-human)
monoclonal antibody with specificity
for the IL-6 receptor; attenuates B
and T lymphocyte activation,
leukocyte migration, osteoclast
activation, and hepatic synthesis of
acute phase reactants

monoclonal antibody
with specificity for
B-cell-activating
factor (BAFF), also
referred to as Blymphocyte
stimulator (BLyS)

belimumab

the most common


ocular condition in
rheumatoid arthritis

keratoconjunctivitis sicca

most important
toxicity from
hydroxychloroquine

visual loss resulting from retinal


pigment deposition

NOTE that selective


COX-2 inhibitors are
not safer for the
kidney

than nonselective COX inhibitors


and may exacerbate kidney disease
and hypertension

NSAID that reduces


cardiovascular risk

than nonselective COX inhibitors


and may exacerbate kidney disease
and hypertension

pegylated
recombinant
mammalian uricase
that has received
FDA approval for
treatment-failure
gout

pegloticase

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42.

maintains the
gastric lining and
kidney blood flow

prostaglandin E2

medium size
vasulitis

PAN, Kawasaki

methotrexate
toxicities

macrocytic anemia and drug-induced


hepatitis (contraindicated in regular
ETOH consumption), highly
teratogenic

44.

MOA abatacept

fusion protein of CTLA4, a molecule


expressed on activated human T cells
and the Fc portion of human IgG.
This agent binds to antigenpresenting cells and blocks
costimulatory signals to T
lymphocytes

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50.

primary
presenting features
of
cryoglobulinemic
vasculitis

Cutaneous purpura, mononeuritis


multiplex, and an immune complex
glomerulonephritis

side effects of
febuxostats

liver function abnormalities, which are


often transient

side effects of
leflunomide

hematopoietic abnormalities,
infection, and interstitial lung disease,
highly teratogenic

side effects of
probenecid

increase the risk of kidney stones

side effects of
rituxkmab

severe infusion reactions can occur,


and rare cases of progressive
multifocal leukoencephalopathy
associated with JC virus reactivation

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side effects of
tocilizumab

leukopenia, thrombocytopenia, and


elevated serum aminotransferase
levels. Increases in serum lipid levels

systemic vasculitis
in the spectrum of
hypereosinophilic
disorders that
most often occurs
in the setting of
antecedent asthma,
allergic rhinitis, or
sinusitis

Churg-Strauss syndrome

treatment
forpatients with
patients with
hepatitis Bassociated
polyarteritis
nodosa

corticosteroids should be given


concomitantly with antiviral therapy
(such as entecavir)

treatment of GA

1 mg/kg/d of prednisone; tapering of


prednisone is initiated after 4 to 6
weeks, typically performed in 10%
decrements every 2 weeks

treatment of
hepatitis Cassociated
cryoglobulinemia

antiviral therapy with interferon alfa


and ribavirin. A short course of
corticosteroids may be required to
suppress the acute phase of
inflammation and vasculitis; patients
with severe manifestations, including
kidney failure, digital gangrene, and
severe neurologic disease, a 2- to 3week course of plasma exchange

treatment of
Kawasaki disease?

High-dose salicylates and early


administration of intravenous immune
globulin, steroids if unresponsive

61.

62.

type 1
cryoglobulins

immunoglobulins that self-aggregate and are


associated with Waldenstrom
macroglobulinemia and multiple myeloma;
they may also be seen in patients with Sjgren
syndrome or B-cell lymphoma who develop
monoclonal paraproteins

type 2
cryoglobulins

rheumatoid factors that are monoclonal IgM


or IgA immunoglobulins with specificity for
the Fc portion of IgG; this subtype is most
often associated with vasculitis and most
commonly occurs in the setting of viral
infections with hepatitis C or HIV

usual setting
of PAN

50% of cases occur in the setting of recently


acquired hepatitis B virus infection

when is urate
lowering
therapy
indicated?

repeated gout attacks (typically two or more


per year) or develop tissue deposits of urate
(tophi

when to use
abatacept

moderate or high disease activity and poor


prognostic features who have had an
inadequate response to sequential
nonbiologic DMARDs or methotrexate in
combination with other DMARD