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CMS II Final Comprehensive Review - Rheumatology
Winter Semester 2011
137
Medical
Graduate
03/26/2011

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Cards

Term
Where does RA attack?
Definition
targets the synovium and works its way into the joint space
Term
What are the type of crystals found in gout?
Definition
Monosodium urate, needle-like negatively (yellow) birefringent
Term
What type of crystals are found in pseudogout?
Definition
Calcium pyrophosphate (short, rhomboid positively blue birefringent)
Term
What is Anti-CCP a predictor of?
Definition
Excellent predictor of RA
Term
definite drugs causing drug/induced lupus
Definition
hydralazine, minocin, procainamide
Term
what are some anti-TNF inhibitors that can cause drug-induced lupus
Definition
remicade, enbrel, humira
Term
in SLE what are NSAIDs used for?
Definition
joint pain
Term
in SLE what is hydroxyclorquine used for?
Definition
joint pain/skin rask/fatigue & helps decrease flares
Term
in SLE what are steroids used for?
Definition
significant arthritis or serositis/major organ involvement
Term
in SLE what is MTX used for?
Definition
significant arthritis
Term
in SLE what is Leflunomide used for?
Definition
significant arthritis, rash & perhaps major organ involvement
Term
in SLE what is cyclophosphamide used for?
Definition
serious renal disease/vasculitis
Term
in SLE what is azathioprine used for?
Definition
serious major organ involvement
Term
what are the triggers for scleroderma?
Definition
bleomycin, vinyl chloride, contaminated tryptophan, trichorethylene, silicone, epoxy resins
Term
what's the tx for Raynaud's?
Definition
CCB's (most effective in controlling vasospasm)
viagra
Term
how do we tx musculoskeletal issues in PSS?
Definition
NSAID, plaquenil, steroids
Term
what do we tx malignant HTN in PSS with?
Definition
ace inhibitors
Term
what's the tx for PSS
Definition
CCBs=raynaud's
Ace inhibitors=renal crisis
Bosarten=pulm HTN
NSAIDs/plaquenil=synovitis
Term
what tends to worsen PSS?
Definition
steroids b/c it can precipitate HTN/renal crisis so use low doses 5-10 mg if possible
Term
what's the tx for the glands in sjogren's?
Definition
hydration, drops, lozenges
salagen/evoxac
tear duct ablation
Term
what's the tx for extra-glandular issues in sjogren's
Definition
plaquenil (prevent breakdown of ach)
steroids
NSAID's
Term
what are some of the issues with using steroids in sjogren's?
Definition
weight gain, avascular necrosis
Term
what's a new drug that will quell inflammatory eye disease associated w/ sjogren's?
Definition
restasis (cyclosporine)
Term
what's the first line tx for PM/DM (Polymyositis/Dermatomyositis)?
Definition
steroids
Term
if steroids don't work in PM/DM (Polymyositis/Dermatomyositis) what do we move to?
Definition
MTX
Term
what are the options for tx of PM/DM
Definition
steroids, MTX, azathioprine, leflunomide, cyclophosphamide, IVIG
Term
how do we tx wegener's?
Definition
steroids & cyclophosphamide followed by MTX
Term
what's the tx for PAN?
Definition
high dose IV steroids
IV cyclophosphamide
Term
what are the drugs that will prevent the endothelium from getting inflammed/irritated
Definition
bosentan, iloprost
Term
what are the DMARD's?
Definition
hydroxychloroquine, azathiprine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, mycophenolate, clofazimine
Term
45 y/o pt has been taking hydralazine for a little over a week and is now complaining of muscle aches. You warn him that his medication as well as minocin & procainamide are drugs that have a definite ability to cause this. type of rash due to molecular mimicry. Your suspicion's are confirmed after finding anti-histone antibodies and - anti-DNA.
Definition
drug induced lupus
Term
40 y/o african america female presents with generalized weakness, a rash over her face sparing the nasolabial folds, mouth sores. On PE you find evidence of a some sort of pericardial inflammation, pancytopenia. After further questioning she complains she has become more sensitive to the sun meaning she is getting burns after only spending a short time in the sun. She has a + ANA as well as a + anti-smith antibodies.
Definition
SLE
Term
A pt that has a prior hx of SLE would like to know if there kidneys are affected or have a chance of getting affected what would you order?
Definition
anti-ds DNA
Term
pt who has has a prior hx of SLE is now presenting w/ a fishlike "rash" on her inner thighs. After further questioning you find that she has had >3 miscarriages in the bast 5 years. On w/u you find she is + anti-cardiolipin. What syndrome do we suspect?
Definition
APL syndrome
Term
pt presents w/ a c/o of tightening of her hands/fingers that has worsened. She says she is becoming more and more intolerant to cold saying her hands turn the colors of the American flag! She came in today b/c she has noticed some little broken blood vessels & she is having trouble swallowing food. Upon w/u you find she has a + centromere antibodies. What do you suspect?
Definition
CREST
Term
pt presents w/ diffuse skin thickening & fibrosis. She has wrinkling around her lips. On exam you find that below the wrists there is no issue and u find no telangiectasias or subQ Ca. After doing a w/u she is + for anti SCL 70 antibodies. What do you suspect?
Definition
progressive systemic sclerosis
Term
pt presents w/ a 4 year hx of cold intolerance. She says when she gets cold her hands turn white & then blue & after a couple minutes finally regain there red color. She's worried she has something wrong with her? What do you think?
Definition
Raynaud's disease not phenomenon
Term
65 y/o women presents w/ a c/o of dry eyes/mouth as well as swelling of her cheeks. She has also been experiencing some limited joint pain. After doing a w/u you find she is + for ANA, Ro and La antibodies. After taking a bx of the lacrimal gland tissue you find there is lymphocytic infiltration. You find a + Shirmer's test as well as a + lissamine green staining test.
Definition
sjogren's syndrome
Term
pt presents w/ a 1 months hx of weakness climbing stairs and lifting her children. She complains she is having difficulty flexing her neck muscles. On a w/u you find she has a + anti Jo 1 and Mi 2 antibodies. As a generalized consensus what do you suspect?
Definition
polymyositis
Term
40 y/o pt presents w/ a 1 months hx of weakness involving her proximal muscles like climbing the stairs and lifting objects. You notice she has a rash and some edema right below her eyes. On PE you find she has some papules on her MCP's, PIP's, and DIP's that completely spare the joints in b/w. What do you suspect?
Definition
dermatomyositis
Term
In working a pt up with a polymyositis what enzymes do you expect to be elevated?
Definition
CPK, serum aldolase, SGOT
Term
35 y/o WM presents w/ a recurrent hx of epistaxis and a recent URI. You notice his nose has started to change shape and looks like a saddle. His eyes are red and appear to have uveitis. He is complaining he has begun coughing up blood and had blood in his urine. You find there are some palpable purpura. On w/u find he is + for C-ANCA & decide to put him on high dose steroids as well as cyclophosphamide.
Definition
wegener's granulomatosis
Term
30 y/o male presents w/ a 5 yr hx of repeated abdominal pain as well as testicular pain and has noticed a 17 kg weight loss. He also c/o of pain/paresthesia radiating to his ankle that you believe to be the sural nerve. You find he has a prior hx of hep B. On PE his lungs are CTA. You notice he has developed some splinter hemorrhages as well as purpura. What do you suspect?
Definition
polyarteritis nodosa
Term
what are the diseases the present with a saddle nose deformity?
Definition
relapsing polychondritis
wegener's
church strauss
Term
what autoantibodies are associated w/ drug induced lupus
Definition
anti-histone
Term
which autoantibodies are present in the myositis (PM, DM)?
Definition
anti-Jo-1, anti-Mi-2, anti-SRP
Term
which autoantibodies are present in lupus nephritis
Definition
anti-ds DNA
Term
which autoantibodies are present in CREST?
Definition
anti-centromere
Term
which autoantibodies are present in PSS (systemic)?
Definition
anti-SCL 70
Term
which diseases are + for Ro and La autoantibodies (SSA & SSB)
Definition
sjogren's
neonatal lupus
subacute cutaneous lupus
Term
what are the autoantibodies that are present in vasculitis (general)
Definition
ANCA
Term
which disease correlates directly with C-ANCA
Definition
wegener's granulomatosis
Term
which diseases have DECREASED ace levels?
Definition
scleroderma
Term
which diseases have an increased ACE level?
Definition
sarcoidosis, leprosy, interstitial lung disease (as far as this lecture we only covered sarcoidosis)
Term
which autoantibodies correlate with fetal wastage, arterial/venous thrombosis, thromboyctopenia?
Definition
APL
Term
which antibodies are 98% pos for systemic lupus
Definition
anti-smith
Term
which enzymes (3) will be elevated in a myositis?
Definition
CPK, SFOG, aldolase
Term
which autoantibodies are specific for RA?
Definition
anti-CCP
Term
what is present in purpura, glomerulonephritis, lung dz, arthritis
Definition
cryoglobulins
Term
which autoantibody crosses the placenta easier?
Definition
anti-ro
Term
what does the C-anca target?
Definition
proteinase 3
Term
what do the P-anca's target?
Definition
myeloperoxidase
Term
which autoantibodies are present in sjogren's?
Definition
ANA, anti-SSA or SSB
Term
what are some of the triggering events for SLE?
Definition
infxn, stress, hormonal (estrogen/progesterone worsen it), sunlight, drugs
Term
this type of lupus is localized to skin mostly & tends to scar (above the waist)
Definition
discoid lupus
Term
how do we dx discoid lupus? Are they ANA - or +?
Definition
skin bx
ANA neg!
Term
what signifies a true lupus induced butterfly rash?
Definition
spares the nasolabial folds
Term
this type of lupus produced annular, circular, papulosquamous lesions that are very photosensitive & involve sun exposed areas; constitutional sx's may also be present
Definition
subacute cutaneous lupus
Term
what are the antibodies associated w/ subacute cutaneous lupus erythematosus?
Definition
Ro (SSA) and La (SSB)-->ribonucleoproteins
Term
what's the first line tx for both discoid and subacute cutaneous?
Definition
topical steroids
Term
how does neonatal lupus occur?
Definition
vertical transmission of Ro and LA from mother to fetus
Term
this is a transient lupus that lasts up to 8 months due to vertical transmission of Ro and La antibodies across the placenta
Definition
neonatal lupus
Term
what are the most severe effects of neonatal lupus?
Definition
cardiac (congenital heart block w/ bradycardia) or myocarditis
Term
what are some possible causes of drug induced lupus
Definition
quinidine, phenytoin, sulfonomides, D-penicilliamine, isoniazid
Term
this is a self-limited lupus that is mainly a serositis due to molecular mimicry inflamming the internal organs when taking certain drugs
Definition
drug induced lupus
Term
who may have a milder case of SLE?
Definition
elderly
Term
what are the 2 ages of onset for SLE?
Definition
15 and 40
Term
what are some of the medications that can trigger SLE?
Definition
sulfa antibiotics, hormones, BCP's
Term
What diseases pathogenesis is described as exessive T helper cells w/ low T suppressor cell activity which results in B cell proliferation & autoantibody production?
Definition
SLE pathogenesis
Term
what are some of the clinical features of SLE?
Definition
systemic (fatigue, fevers)
butterfly rash
synovitis
oral ulcerations
pleural/pericardial inflammation
renal disease
neurologic
hematologic (hemolytic anemia, leukppenia, thrombocytopenia)
Term
which antibodes are + in SLE?
Definition
ANA
anti-ds DNS
smith
ACL abs
Term
what does MD SOAP BRAIN stand for in SLE?
Definition
M - malar rash,
D - discoid rash,
S - serositis,
O - oral ulcers,
A - arthritis,
P - photosensitivity,
B - blood (anemia, thrombocytopenia/leukopenia), R - renal,
A - +ANA,
I - immunologic antibodies,
N - neurologic features
Term
which antibodies affect which organs?
Definition
kidney: anti ds DNA
heart: ro/la
anti-histone: skin
Term
in the fractionated ANA which antibodies appear speckled?
Definition
anti-Sm & RNP
anti ro and la
anti-jo-1 and Mi-2
anti-scl-70
Term
which antibodies correlate w/ PSS (CREST)?
Definition
anti-centromere
Term
which antibodies correlate w/ both SLE & PSS?
Definition
anti-nucleolar
Term
what's the order of tx for SLE?
Definition
steroids, MXT, leflunomide
Term
what do APL antibodies attack?
Definition
negatively charged phospholipid/plasma proteins
Term
what is APL syndrome?
Definition
hypercoaguable state resulting in fetal wastage, arterial/venous thrombosis, thrombocytopenia, livedo reticularis
Term
pt presents w/ a hx of 4 previous miscarriages, fishlike purpleish hue rash b/w forearms & thighs as well as a hx of thrombocytopenia what do you suspect?
Definition
APL syndrome
Term
how do you make a dx of APL syndrome?
Definition
any of the 3 labs (LAC, ACL, beta glyp !)
Term
what's the recommended tx for APL w/o thrombosis?
Definition
baby asa
Term
what's the recommended tx for APL w/thrombosis?
Definition
coumadin w/ high INR b/w 3-4
Term
what's the recommended tx for APL w/thrombosis?
Definition
coumadin w/ high INR b/w 3-4
Term
when should you never anticoagulate?
Definition
plts <50K
Term
what do you do when the APL becomes negative?
Definition
continue anticoagulation
Term
what is the limited cutaneous sclerosis?
Definition
CREST
Term
a chronic widespread small vessel vasculopathy & fibrosis that usually involves the skin, lungs, & GI tract as well as the renal system
Definition
scleroderma
Term
what are the 3 variants of scleroderma?
Definition
progressive systemic sclerosis (PSS)
CREST (localized)
morphea (localized)
Term
what are the environmental causes of scleroderma?
Definition
bleomycin, vinyl chloride, contaminated tryptophan, trichloreythelene, silicone, epoxy resins
Term
What does CREST stand for
Definition
C-calcinosis
R-raynaud's phenomenon
E-esophageal dysmotility
S-sclerodactyl
T-telangiectasias
Term
how far does sclerodactyl in CREST go?
Definition
to wrists (doens't go past)
Term
what syndromre is strongly associated with SLE?
Definition
APL syndrome
Term
what are the cutaneous changes in CREST?
Definition
early diffuse finger swelling, hands below wrist, telangectasias, subQ Ca
Term
what are some of the triggers for morphea?
Definition
RT in cancer pts
infxn (EBV)
trauma
Term
what's the difference between morphea and scleroderma?
Definition
lacks features such as sclerodactyl, raynaud's phenomenon, internal organ development
Term
What does en coup de sabre occur in?
Definition
morphea
Term
thickened skin extending from the hairline onto the forehead in morphea & doesn't involve the brain
Definition
en coup de sabre
Term
if there is diffuse/deep morphea how do we tx it?
Definition
systemic IV steroids, penicillamine, phototherapy
Term
Pt presents w/ diffuse skin sclerosis, major organ involvment and + Anti SCL 70 antibody involving skin above wrists and there are no telangectasias, subQ Ca
Definition
progressive systemic sclerosis
Term
what's the initial sx in 90% of pt's w/ scleroderma
Definition
raynaud's phenonmenon
Term
What does BAD CT correlate with and what does it stand for?
Definition
Raynaud's
B: blood disorder (polycythemia)
A: arterial (blockage)
D: drugs (BB's)
C: CT disorders (SLE, SCL)
T: Traumatic (vibration injury)
Term
with which type of scleroderma is raynaud's always present?
Definition
CREST
Term
pt presents with this type of scleroderma that involves polyarthralgias, friction rubs
Definition
PSS
Term
What's the major cause of death is PSS
Definition
renal disease
Term
what's more likely to happen in pt's with PSS who have friction rubs?
Definition
renal problems
Term
what is malignant HTN and rising creatinine in PSS signify
Definition
renal crisis
Term
what's the tx for the HTN associated with PSS?
Definition
ace inhibitors
Term
although renal problems are a major issue associated with death in PSS what's usually the cause of death?
Definition
cardiac issues such as CHF and arrhythmias
Term
What's the different b/w pulmonary findings in PSS vs. CREST
Definition
PSS consists of pulmonary fibrosis as well as restrictive disease that is usually bibasilar where CREST has an incidence of pulm HTN late in the disease that is monitored by DLCO that may be responsive to CCB's or bosarten
Term
what can we tx the pulm HTN associated with CREST with?
Definition
CCB's
Bosarten
Term
what's the difference between GI sx's in PSS vs CREST?
Definition
PSS and CREST both involve esophagitis and distal motor dysfunction but in CREST lower GI involvement is rare where in PSS sm/lg bowel hypomotility is common. Primary biliary cirrhosis may occur in CREST
Term
in which type of scleroderma does pseudodiverticulum occur?
Definition
PSS
Term
What are characteristics of OA of the hands on PE?
Definition
Heberden's (DIP) and Bouchard's (PIP) nodules
Term
50 y/o Pt presents with pain in her knees, especially when she has stopped moving (after rest). What condition do you suspect?
Definition
OA
Term
Pt presents with painful, swollen big toe (metatarsal joint) that is erythematous. The joint is warm and is intolerable to any touch. The pt is 45 y/o and has never experienced anything like this previously.What condition do you suspect? How would you treat it?
Definition
Gout; Colchicine
Term
What is the name of the sex-linked disorder of HGPRT enzyme deficiency causing accumulation of uric acid in all tissues?
Definition
Lesch-Nyhan Syndrome
Term
How is uric acid build up's cause determined?
Definition
24 hr urine (looking for uric acid)
Term
What is the majority of hyperuricemia associated with?
Definition
90% associated with undersecretion of uric acid
Term
Which diseases is gout associated with?
Definition
DM, HTN, Hyperlipidemia
Term
Pt c/o of morning stiffness lasting > 1hr, especially in both their wrists. She also has nodules on her elbows. What condition do you suspect? What is the gold standard tx for this condition?
Definition
RA; Methotrexate(Rheumatrex/Trexall):gold standard/FIRST LINE
Term
What is the classic triad of symptoms of Sjogren's syndrome?
Definition
Xerostomia, Keratoconjunctivitis sicca, & Arthritis
Term
What is the diagnostic procedure for sjogren's syndrome?
Definition
Abnormal salivary gland or lip biopsy showing lymphocytic infiltration
Term
What are the auto anti-bodies associated with Sjogren's
Definition
Positive RF or ANA or SSA/SSB
Term
What is a heliotrope rash and what condition is it associated with?
Definition
(purplish, scaly rash over eyelids) associated with Polymyositis
Term
How is the definitive diagnosis of polymyositis made?
Definition
Muscle biopsy (DEFINITIVE)
Perivascular infiltrate of inflammatory cells (lymphs)
Rule out on muscle biopsy: inclusion body myositis (vacuoles)
Mitochondrial (ragged red fibers)
Term
What is the best means of diagnosis of osteoporosis?
Definition
Bone Mineral Density (BMD) & Vertebral Fracture Assessment (VFA) – gold standard
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