Term
|
Definition
targets the synovium and works its way into the joint space |
|
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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
|
|
Term
in SLE what are NSAIDs used for? |
|
Definition
|
|
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
|
|
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
|
|
Term
|
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
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|
Term
what's the first line tx for PM/DM (Polymyositis/Dermatomyositis)? |
|
Definition
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|
Term
if steroids don't work in PM/DM (Polymyositis/Dermatomyositis) what do we move to? |
|
Definition
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|
Term
what are the options for tx of PM/DM |
|
Definition
steroids, MTX, azathioprine, leflunomide, cyclophosphamide, IVIG |
|
|
Term
|
Definition
steroids & cyclophosphamide followed by MTX |
|
|
Term
|
Definition
high dose IV steroids IV cyclophosphamide |
|
|
Term
what are the drugs that will prevent the endothelium from getting inflammed/irritated |
|
Definition
|
|
Term
|
Definition
hydroxychloroquine, azathiprine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, mycophenolate, clofazimine |
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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
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|
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
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|
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
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|
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
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|
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
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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 |
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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
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|
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
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|
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
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|
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
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|
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
|
|
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
|
|
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
|
|
Term
which autoantibodies are present in CREST? |
|
Definition
|
|
Term
which autoantibodies are present in PSS (systemic)? |
|
Definition
|
|
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
|
|
Term
which disease correlates directly with C-ANCA |
|
Definition
|
|
Term
which diseases have DECREASED ace levels? |
|
Definition
|
|
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
|
|
Term
which antibodies are 98% pos for systemic lupus |
|
Definition
|
|
Term
which enzymes (3) will be elevated in a myositis? |
|
Definition
|
|
Term
which autoantibodies are specific for RA? |
|
Definition
|
|
Term
what is present in purpura, glomerulonephritis, lung dz, arthritis |
|
Definition
|
|
Term
which autoantibody crosses the placenta easier? |
|
Definition
|
|
Term
what does the C-anca target? |
|
Definition
|
|
Term
what do the P-anca's target? |
|
Definition
|
|
Term
which autoantibodies are present in sjogren's? |
|
Definition
|
|
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
|
|
Term
how do we dx discoid lupus? Are they ANA - or +? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
who may have a milder case of SLE? |
|
Definition
|
|
Term
what are the 2 ages of onset for SLE? |
|
Definition
|
|
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
|
|
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
|
|
Term
which antibodies correlate w/ both SLE & PSS? |
|
Definition
|
|
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
|
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
|
|
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
|
|
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
|
|
Term
what do you do when the APL becomes negative? |
|
Definition
|
|
Term
what is the limited cutaneous sclerosis? |
|
Definition
|
|
Term
a chronic widespread small vessel vasculopathy & fibrosis that usually involves the skin, lungs, & GI tract as well as the renal system |
|
Definition
|
|
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
|
|
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
|
|
Term
thickened skin extending from the hairline onto the forehead in morphea & doesn't involve the brain |
|
Definition
|
|
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
|
|
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
|
|
Term
pt presents with this type of scleroderma that involves polyarthralgias, friction rubs |
|
Definition
|
|
Term
What's the major cause of death is PSS |
|
Definition
|
|
Term
what's more likely to happen in pt's with PSS who have friction rubs? |
|
Definition
|
|
Term
what is malignant HTN and rising creatinine in PSS signify |
|
Definition
|
|
Term
what's the tx for the HTN associated with PSS? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
What is the name of the sex-linked disorder of HGPRT enzyme deficiency causing accumulation of uric acid in all tissues? |
|
Definition
|
|
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
|
|
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 |
|
|