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USMLE HY notes > Reumotology finals > Flashcards

Reumotology finals Flashcards

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USMLE® Step 1 flashcards
1
Q

Which Vasculitis are associated with P-ANCA and which with C-ANCA

and each of those Ab, agianst which part?

A

**only the small vessel vasculitis:
**C-ANCA = Wegener (granulomatosis with polyangitis)
P-ANCA= Shug straus (EGPA) , microscopic polyangitis (MPA)

C- cytoplasmin&raquo_space; PR 3
P- perinuclear&raquo_space; MPO

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2
Q

If theres a vasculitis with both P and C anca positive.
it can be?

A

Drug induce vasculitis

mainly secondary to Cocaine use

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3
Q

What is the main Tx in vasculitis?

A

Steroids + cyclophophamide

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4
Q

what can be the use in Azathioprine (imuran) in vasculitis

what we have to check first?

A

maintanance- in GPA/MPA/GCA

must check levels of TPMT - related to severe cytopenias

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5
Q

RTX can be use in which type of vasculitis?

A

Microscopic polyangitis
granulomatosis with polyangitis - wegener

late onset neutropenia + HBV reactivation

small veseels.

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6
Q

which vascilitus can be treated with tocilizumab?

and what is the MOA
and C/I

A

GCA- giant cell arthritis (Temporal)
* also for RA as 2nd line
MOA- anti IL-6

C/I- Hx of diverticulitis

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7
Q

which anti suppresive medication can cuase hyperlipidemia + GI perforation?

A

Tocilizumab

toci li ze m-abdomnial

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8
Q

Mepolizumab can be use in which type of vasculitis?

A

EGPA

also for asthma
*remember- EGPA present with weird asthma

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9
Q

when we should give folate after tx with MTX?

A

a day after treatment

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10
Q

Granulomatosis With Polyangiitis (GPA)

What is the clinical presentation (triad)

A

Sinusitis, Otitis media, hemoptysis (think URI, LRI)
+
nephritic syndrome- hematuria + RBC casts

Upper resp. involv - 95%
lower- 85-90%
kidney - 77%

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11
Q

What we will see in CXR of Granulomatosis With Polyangiitis?

A

bi-lateral nodular infiltrate - to cavitations

in biopsy- necrotic vasculitis w/ granulamotosis

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12
Q

In which types of Vasculitis we will see pauci-immune?

A

all ANCA diseases:
GPA
EGPA
MPA

except- goodpasture syndrome (not a vasculitis)

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13
Q

Granulomatosis With Polyangiitis how we diagnose?

A

Biopsy- mainly from the lung

DDx for GPA- use of cocaine

C-ANCA

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14
Q

Which type of def. and substacne exposure will increase the risk for GPA

A

a1-AT and selica exposure

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15
Q

Tx for GPA + MPA

A

induction steroids + cyclophospamide (PO) (3-6month)
RTX- good as cyclo, given to pt with relapse
maintance- RTX (most effective )/ AZA/ MTX/ MMF (less effective) - at least for 2 years

50-70% יחוו הישנות

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16
Q

what is the different between
GPA to MPA

A
  • MPA- w/o Upper respiratory tract involvment
  • P-ANCA (and no C)
  • No granulomas

could also be- mononeuritis multiplex (also in GPA)

almost the same disease

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17
Q

Clinical presentation of EGPA (Chaug strauss)

A
  • late onset of weird asthma - rsistant to therapy,
  • sinusitis
  • neuropathy- Mononeuritis multiplex- second most common finding
  • eosinophilis in labs, P-anca
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18
Q

Why should we perform echocardiography for every pt with EGPA?

A

will determine the prognosis and the aggresive to tx

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19
Q

What is the most significant cause of death in EGPA

A

cardiac disease

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20
Q

What is the prognosis of EGPA w/o Tx?

A

25% for 5 year survival

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21
Q

What is the Tx of EGPA?

A

mild disease- steroids, consider Mepolizumab
severe- like GPA and MPA

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22
Q

Which type of infection is associate with Polyarthritis nodusa (PAN)

A

HBV+

*check also for HBC

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23
Q

Which leukemia is a/w PAN?

A

hairy cell leukemia

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24
Q

Why kidney biopsy is not a good way to Dgx PAN?

A

beacuse this disease damage medium size blood vessels.
which mean &raquo_space; the injury will be ischemic and will not see GN.

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25
Q

what is common sign in blood vessels in PAN?

and which Blood vessels are the most common?

A

Rosary sign- beads on string

look for this anurysms at the kidney, liver, mesenteric arteris.

on biopsy- transmural inflammation

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26
Q

Whats the Tx for PAN

A

Steroids + cyclophospamid

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27
Q

Clinical findings of GCA (Temporal)

A

headace
pain while eating
high ESR
pt > 50 can present with polymealgia rheumatica

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28
Q

major severe complication of GCA

A

Blindless&raquo_space; due to obstruction of the ophtalmic artery ischemic optic neuropathy

1/3 of pt can present with disease involving the aorta -mainly thoracic leads to dicecction and anyrusms

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29
Q

how to Dgx PMR (polymaglia rheumatica)

A

Clinical:
high ESR
classic complains
rapid repsonse to low dose presnisone

with no symptoms related to GCA- no need to check

Aspirin- PPX for ischemic injury to coronary arteris
Tocilizumab- also effective in indution and remmision

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30
Q

Tx for GCA
* for “regular” disease
* for involvment of the eye

A

for regular disease-High dose Prednisone (40-60) for 1 month&raquo_space; given low dose for around 2 yrs
for eye involvment- steroid palse 1 gr/day solmedrol X 3 days

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31
Q

Tx for PMR?

פולימיאלגיה ראומטיקה

A

prednisone- 10-20mg

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32
Q

IgA vasculitis (Henoch s.purpra)

Clinical menefistaion

A

Always after URI
Palpable purpura on butt and legs
GI- abdminal pain, malena
Kidenys- GN RBC + protein in urine

mainly a kids disease

Cardio involvment- not in kids!!

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33
Q

How we Dgx Hanoch schonlein?

A

skin biopsy- IgA-C3 complexes

high blood IgA- without any diagnostic of prognosis meanning

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34
Q

Tx for IgA vasculitis (hanoch)

A

Follow up.

steroids for relief of symptoms

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35
Q

Which vasculitis can present with RPGN

A

all small vessels vasculitis

GPA
MPA
EGPA
IgA vasculitis (hanoch)

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36
Q

Which artery is the most common injured in Takayasu?

A

Subclavian- 93% of cases

arm claudication, raynauds phenomenan

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37
Q

How to Dgx Takayasu?
and TX

A

Arteriography or CT/MRI

Tx- steroids + surgery approche , if can’t&raquo_space; MTX

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38
Q

Cryoglobulinemic Vasculitis

what is the MOA
which type of nephropathy is common?

A
  • MOA- Ab the sink in cold
  • kidney- GN&raquo_space; MPGN
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39
Q

Cryoglobulinemic Vasculitis

what is Type 1 vs Type 2

A

Type 1- monoclonal Ab, a/w monoclonal disease (MGUS, MM, WM)

Type2- can present with HCV / HIV immune complex (type III)

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40
Q

in Cryoglobulinemic Vasculitis
what will be the levels of
RF
C4
C3
ESR / CRP

and what we shold always look for?

A
  • RF- eleveted
  • C4- low
  • C3- normal (rare)
  • ESR/CRP- eleveted

always look for HCV infection

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41
Q

Behçet Syndrome

which type of HLA is found?

what is the systems involve / organs in this syndrome

A

HLA-B51

Systems- for diagnosis you need mouth ulcers + 2 of the 4 below
* mouth ulcers (not painful)- 98%
* reccurent genital ulcers- 80% most specific
* Skin lesions- acne like lesions, erythema nodosum, superfical thrombophlebitis,
* pathrgi reaction- hyperactivity reaction of skin to trauma- like needle
* eye lesions- pan-uveitis

and many more- CNS, GI, Thrombosis, anurysms of lung arteris

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42
Q

Which medication can be use in every one of the menefistations of Behçet Syndrome

A

Azathioporins

GI + EYE- combine with steroids
CNS- add Anti-TNF

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43
Q

When we will see deposition of Calcium oxalate in joints?

A

CKD

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44
Q

Which crystals are seen in Gout?

A

Monosodium Urate

uric acid > 6.85

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45
Q

Triggers for Gout attecks

A

everything that elevate uric acid levels:
1. red meat and seafood
2. alcohol
3. diuretics
4. Myeloproliferative disoder = high turnover (PV, ET, CML)
5. CKD (low GFR)

also seen in obesity and males

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46
Q

most common presentation of gout attack

A

mainly 1 joint- most times is the 1st MTP (Podagra) = toe

pass after 1-2 wks

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47
Q

Which physical finding can be seen in chronic hyperurecemia (a lot of gout attack)

A

Formation of tophi

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48
Q

Cheracteristics of the crystals in gout

A

Negetive bi-refrigiants
needle shape
Yellow in parallel
blue in perpandicular

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49
Q

Which medications can be use for gout PPX

A

Xhantine oxidase inhibitors
alloperinol- 1st line, can cause Steven jhonson, TEN
fubuxostat

must take with NSAIDS/ cholchicine in start- until tophi disapper or uric acid in target (the later )

Not during acute episode

the main use: Gout, PPX TLS

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50
Q

which 3 menefistations are a/w CPPD?

A
  1. a-symptomatic
  2. psuedo-gout
  3. psauso- OA
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51
Q

common finding in X-ray of CPPD joint

A

Chonsrocalcinosis
linear calcification of the joint

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52
Q

What defines the crystals in CPPD?

A

Rhomboids
positive Bi-regrignes
Blue in parallel

gout- needle shape, negitive, yellow in parallel

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53
Q

ESRD + vitamin C supplaments are major risk for which type of crystal deposition

A

Ca-Oxalata

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54
Q

Main cuase of death in SLE?

A

Cardio-vascular

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55
Q

Which Ab we might see in SLE?

A
  • ANA-95%
  • Anti-dsDNA
  • anti-smith

drug induce SLE:
* Anti-histone

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56
Q

Which Ab are in correlation of neonatal SLE?

and which other disease is a/w this menefistation

A

Anti SSA- anti ro
Anti SSB- anti la

more common in womens with Sjoren Syndrome (this is the Ab a/w the disease)

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57
Q

Main menefistation of SLE:

A
  • Musculoskeletal- Arthralgia/ polyarthritis
  • cutaneous- photosensetivity, malar rash, discoid
  • Hematologic- anemia of chronic disease. could be penia’s
  • Cardio- Pericarditis, libman sack
  • Renal- nephrotic syndtome
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58
Q

The difference between Sica syndrome to sjoren

A

Sica syndrome- dryness of eye and mouth. not present with anti-ro or anti-la (SSA + SSB)

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59
Q

most common presentation of SLE in lung

A

pleuralitis

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60
Q

what are the 4 main kideny menefistation of SLE

A
  • Minimal mesangial LN (class I)
  • Mesangial proliferative LN (class II)
  • Focal LN (class III)
  • Diffuse LN (class IV)
  • Lupus membranous nephropathy (class V)
  • Advanced sclerosing LN (class VI)
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61
Q

What is the indication of Hydroxychloroquine in SLE?

A

Recommended to all SLE pt

prolong life + reduce tissue damage

rq eye test once a year

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62
Q

Tx in non life thrething SLE?

A
  • low dose sterodis
  • AniFrolumab
  • Belimumab

Faru and Bella - SLE is a women disease

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63
Q

Tx in SLE with organ damage?

A
  • Steroids
  • induction- Cyclophophamid / MMF
  • maintancne- MMF / AZA

pregnency only AZA

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64
Q

when we will give ACEi / ARBS in SLE

A

lupus nephritis with protinura > 500

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65
Q

main risk in neonatal lupus

A

Complete Heart block

will need ICD

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66
Q

Pregnancy with Anti-phospholipid syndrome Tx

A

Enoxaparin - LMWH
+
Aspirin

קלקסן

67
Q

Ab positive in Drug induce lupus

and which drugs

A

**Anti-histone
**
Drugs:
SHIPPP
Sulfa
hydralazyne
isoniazid
Procinamide
Phyntoin
PTU

anti- TNF

ANA can be also positive

68
Q

Drugs associate with drug induce vasculitis

A

PTU
Hydralazine

69
Q

Antiphospholipid syndrome (APS)

Which type of thrombus?
what are the Ab?

A

Vein + artery

Ab:
1. Anti-cardiolipin
2. lupus anticoagulant
3. anti b2-glycoprotein

70
Q

What is catasthropic Antiphospholipid syndrome (APS)

A

Thromboembolic disease which involve 3 or more organs at the same time

71
Q

What are the criteria for Dgx of Antiphospholipid syndrome (APS)

A

lab-at leat one of the Ab to be positive in 2 test with gap of at least 12 wks
Clinical criteria- one or more thromboembolic event
or
Pregnancy realted- IUD of normal fetus > 10wks, 3 or more abortion < 10wks, preterm labor < 34

one lab criteria and one clinical critera

72
Q

Tx for Antiphospholipid syndrome (APS)

in pregnency and not

A

No pregnency- warfarin (comidin) for life. INR 2-3 after venous event, 3-4 after arterial event. or 2-3 + aspirin
Pregnant womens- Aspirin + LMWH (ppx dose), if event on Tx- LMWH thrapoetic dose / hydroxycholoquine / IVIG 5 days

73
Q

Why we should be careful in given DOAC to pt with Antiphospholipid syndrome (APS)?

A

Found to increase the risk of Aterioemblic events

74
Q

what are the Ab associated with both types of Scleroderma?

A

Diffuse- SCL-70 (anti-toposiomerase) + Anti-RNA polymerase III
Limited- Anti centromere

75
Q

What is the main clinical features of RA?

A
  • symmetrical polyarthritis
  • Morning stifness > 1 hr
  • Can present as a systemic disease - fever and ect.

Osteoarthritis is different in all of that

76
Q

Which joint are typically effect in RA?

A

MCP + PIP

DIP is spare

in OA- DIP will be involve

77
Q

Most common cause of death in RA

A

CV disease

78
Q

all of the following are related to which type of autoimmune disease:

  1. Baker cyst
  2. Serositis- pleurolitis, pericarditis
  3. Subcutneous nodules
  4. eye involvment
A

RA

79
Q

What is felthy syndrome?

A

Traid of RA + splenomegaly + neutropaenia

80
Q

Eleveted fever in RA pt should raise a suspect for?

A

rheumatoid vasculitis
or
infection (septic arthritis)

81
Q

Which Ab can present in RA?
which one is more specific

A

RF- in 75%
Anti-centrulline peptide (ACPA = Anti-CCP)- 75% pt but specifity of 95%

82
Q

which Ab that present in RA is in correlation to worsen prognosis in extra-joints menefistatoin?

A

Anti-CCP

83
Q

Which serious toxocity can cause hydroxycholoquine?

A

Retinal demege- irreversible (rq eye exmination pt > 40 yrs)

84
Q

Which serious toxicity can cause sulfasalazine ?

A

Granulocytopenia
G6PD hemolytic

85
Q

MTX affect on lung, liver, BM?

A

lung- intestitial pneumonitis
Liver hepatotoxicity
BM- myelodepresson (penia)

86
Q

Which medication can reverse the affect of MTX?

A

Levcovorin

87
Q

Side effects of Cyclophosphamid?

A
  • Cystitis hemmorhagic
  • SIADH- hyponathremia
  • risk for bladder cancer
  • infartility
88
Q

Which immunosupressive medication should be screen first to PDD (TB)?

A

Infliximab (TNF-a inhibitors)

89
Q

What is the only bilogic medication that not rq TBB test prior to administation

A

RTX

90
Q

Which 4 medication rq serologic testing for viral hepatitis

A
  • Anti=TNF
  • RTX
  • MTX
  • ARAVA (leflunomide)
91
Q

What is the most common medication for the use of RA (DMARDS)

A

MTX

92
Q

C/I for MTX?

A

pregnanct
Cr > 2
ILD

93
Q

Side effect of MTX

A
  • Somatomatitis / mucositis
  • BM depression- pancytopenia
  • pneumonitis ~ PF

ARAVA (Leflunomide) similar to MTX

94
Q

C/I for anti-TNF (infliximab)

A

HBV
CKD III/IV

might cause a reactivation of TB

95
Q

in which case of RA we will use in RTX?

A

Refactory RA

96
Q

What is the approch of Tx with RA?

A
  1. mild-severe disease- MTX
    if after 3-6 month theres no desire outcome - move to combination therapy
  2. Oral triple therapy- MTX + sulfasalizne + Hysroxycholoquine
  3. MTX + Leflunomife
  4. MTX + anti-TNF

Tx of ILD- high dose steroids + more immunosuppresent therapy

97
Q

Axial spondyloarthitis with finding in imaging will be called:

A

Ankylosing spondylitis (AS)

98
Q

Which HLA is prevelance in Ankylosing spondiloarthritis?

A

HLA-B27
90% in AS

50% in psoriatic arthritis

99
Q

What are the 4 seronegetive spondyloarthritis?

A
  1. Ankylosing spondylitis
  2. Psoritatic arthritis
  3. IBD
  4. Reactive arthritis

they can also menifest toghter for exp- AS with IBD/ psoriasis

100
Q

What is the most common symptom of Ankylosing spondylitis

A

lower back pain - 75%
inflammtory pain- night pain, pain in rest, improve in movment

pain in lumber region/ butt/ sacroiliac

101
Q

Which cheracterisitc are present in all of the seronegetvie spondyloarthritis

A
  1. oligoarthritis - 2-4 joints
  2. a-symmetrical (mainly in lowe extremity)
  3. Dactilytis- sausage fingers
  4. enthersitis- ligament inflame in the insertion to the bone
  5. involve the axial spine

in RA: symmetrical, polyarthritis, mainly hands

  1. Ankylosing spondylitis
  2. Psoritatic arthritis
  3. IBD
  4. Reactive arthritis
102
Q

Which Eye menefistation is the most common on AS (AxSPA)

A

Ant. uveitis ~40%, mainlt unilateral

103
Q

Which extra skeletal menefistation can be present in Ankylosing spondylitis (AxSPA)

A

uveitis
Aortitis&raquo_space; AR
Restrictive lung disease

104
Q

How to Diagnose AxSPA

ankylosing spondlyartritis

A

Pt with back pain >3 month ang age < 45

criteria:
Sacroilitis on imaging + 1 < SpA features
or
HLA-B27 + 2 < SpA features

the features:
* inflammatory back pain
* Arthritis
* Enthensitis- heel pain
* ant. uvietits
* dactylitis
* psoriasis
* IBD realted
* HLA-b27
* elevete CRP
* family Hx of SpA
* good respond to NSAIDS

105
Q

What test is the imaging when AxSpA is suspected?

A

MRI

106
Q

What is the 1st line medication in AxSpA

A

NSAIDS - high dose long term

sendongf line - Anti- TNF (could cause secondary psoriasis due to tx)

107
Q

Which bacteria can cause Reactive Arthritis?

A

Enteric or urogenital:
Entreric- Shigella, Salmonela, Yersina, Combylobacter jejuni

Urogenital- Clamydia throchomanis

mainly 1-4 weeks prior the symptoms

108
Q

What is Rieter syndrome?

A

When reactive Arthritis present with Ant. uveitis + and dysurea

Can’t See Can’t Pee, Can’t Climb a Tree

109
Q

Clinical features of Reactive arthritis?

A

after infection
can present with:
* oligo-a-symmetrial arthritis
* dactylitis
* enthritis
* oral ulcers

Keratoderma blennhorragica- vasicular rash with crust in hands and feets

110
Q

Tx for Reactive Arthritis?

A

High dose NSAIDS

(arthritis last for 3-5 months)

111
Q

Which seronegetive disease present with only the involvment of DIP (in hands)

A

Psoriasis arthritis

112
Q

How many cases of psoriasis arthritis can present as symmetrical arthritis like RA

A

40%

113
Q

a pt with psoriasis anf chenge in the nails will most likely will also have

A

Arthritis.
(psoriasitc arthritis)
90% of pt.

114
Q

In which disease we will see “pencil in a cup”

A

Psoriasis arthritis

115
Q

Which viral disease can cuse psoriasis arthritis?

A

HIV

116
Q

what are the 3 best choice of Tx for psoriatic arthritis

A
  • Anti-TNF- improve both skin and joint disease (check TB)
  • Anti-IL17 (Sekucicnumab)- could make IBD worsen
  • Anti-IL23
117
Q

Which type of IBD is relates to AxSpA?

A
  • Type I- joints < 5, comes with wosresning of the IBD
  • type II- joints > 5, w/o correlation to the severity of the IBD disease/ flares
118
Q

what is the Tx for IBD associated arthritis?

A

Anti-TNF
except ETNACEPT- only for RA or SpA

119
Q

Which type of histological feature we will see in sarcoidosis?

A

Non- ceasting granulomas

TB- ceating granulo,as

120
Q

How many pt will recover for sarcoidosis?

A

more then 50%

after 2-5 years

121
Q

Which electrolyte disbalance sen in sarcoidosis?

A

Hypercalcemia- due to realese of a-1 hydroxylase from macrophages in alveoli&raquo_space; more vitamin D active

122
Q

What we will see in CXR of sarcoidosis?

and what is the most common fiding (in presentation)

A
  1. Hilar lymphadenopathy
  2. interstitial infiltrates

might progess to pulmonary fibrosis

most common findings- pt with cough and dyspnea and on CXR with lymphadenopathy

123
Q

What are the 4 stages of sarcoidosis?

A

Stage 1- hilar LAD
stage 2- Adenopathy + infiltrates
Stage 3- infiltrates alone
stage- PF

124
Q

What is the most sensitive test for interstitial disease?

in lung

A

DCLO

125
Q

What is the extra-lung menefistation of sarcoidosis

A
  1. skin- Erythema nodosum- on shins
  2. eyes- Ant. uveitis (could also be post)
  3. Heart- heart block, cardiomyopathy
    4.** CNS-** bells palsy, motor deficit
  4. liver involve in biopst ~50%
  5. **kidney- **CKD (hypercalcemia)
126
Q

how to Dgx sarcoidosis?

A
  1. typical clinical presentation
  2. imaging
  3. biopsy of non-ceating granulomas
127
Q

which test can support sarcoidosis if biopsy is negetive

A
  1. high ACE levels- not senstive nor specific
  2. PET scan- if many organs are involve
  3. BAL- lymp > X2 (sensetive), ratio CD4:CD8 > 3.5 - higly specific

Sarcoidosis is a CD4 cells disease which secrete&raquo_space; **IL2 » TH1 prolifration +CD4 secrete ** INF-y&raquo_space; activate of macrophages

and granuloma formation

128
Q

Tx of choice in sarcoidosis?

in acute flate and also in chronic

A

Steroids

mild or a-symptomatic disease- just follow up
one organ invilve- topical steords
multi organ disease or /Brain, heart, eyes , hypercalcemia- systemic steorids

129
Q

Which steroid sparing can be use in sacroidosis?

in acute flare + chronic disease

A

for acute flare
skin- minucyclin or hydroxycholoqine
MTX- affective in ~ 2/3 of cases
AZA- as MTX but more toxic
for chronic disease
Infliximab- effective in lung dsease. risk for TB reactivation

130
Q

What is the CREST in limited scleroderma

A

CREST
Calcinosis
Rauneyds
Esophageal dysmotility
Sclerodactyly
Telangiecatia

Anti-centromere Ab

131
Q

Which feature can be distinguish between a primary renualds to secondary

A

on capiloscopia abnormality of the nail bad- in secondary

132
Q

How is the skin of a person with Scleroderma will look?

A
  • face mask, fish mouth
  • tight and thick skin
  • Telangiectasia- mainly on the dace
  • sclerodactulity- swollen fingers
133
Q

What will be the lung function test in a pt with Systemic scleroderma

A

low volumes + DLCO

Pulmonary fibrosis

134
Q

What is the test of choice for diagnosis of PF or ILD in scleroderma

A

Hgh resulotion CT
(HRCT)

135
Q

Renal crisis and scleroderma:
* which type is more commen?
* Risk factors?
* what is a protective factor?

A
  1. more common in the diffuse type
  2. risk factors- anti-RNA pol III (Ab in diffuse with SCL-70), use of steroids, afroamericans, penia (of blood)
  3. protective factors- Anti-centromere
136
Q

A pt with Scleroderma present to the ER with severe headace and visual disturbacne, BP 210/120. on CBC Thrombocytopenia with schistocytes with sings of AKI.

what is the Dgx?
what is the Tx?

A

Renal crisis
Malignant HTN (in 10% BP is no eleveted)
MAHA- with schystocyts
AKI
Thrombocytopenia

Tx
ACEi

137
Q

Which disease are in correlation with PBC (primary billiary chirossis)

A
  • Limited Scleroderma- 5-15%
  • Hasimoto
  • RA, SLE, Sjoren
138
Q

Tx in Renal crisis in scleroderma

A

Short T1/2 ACEi- Captopril
If HTN persist- CCB, ARBS
consider- Eculizumab (against Complement system)

2/3 of pt will need dialysis later
90% mortality w/o treatment

139
Q

Which Scleroderma Pt are at high risk for Renal crisis

4

A
  • Early disease
  • large skin involvment
  • Tendon friciton rab
  • Anti-RNA polymerase III

Avoid Steroids

140
Q

What is the Tx of choice in PAH in scelroderma

A

Endothilin 1 receptor antagonist (Bisentan) / PDE-5 inhibitor (dildenafil)

141
Q

Wha is the Tx of coice in Raynaud?

A

CCB- Dhydro / dilitazam
another option: ARBS

for refactory cases- PDE-5 inhibitors, a1 blockers, prostaglidins

ACEi- not effevtive in Renuyad

142
Q

How the following conditions in scleroderma treated:
1. GAVE- watermelon stomach
2. GERD
3. Bacterial overgrowth- low B12 with high Folate

A
  1. צריבה באנדוסקופיה
  2. PPI
  3. Broad spectrum Abx- like Flagyl
143
Q

Sjoren Syndrome clinical menefistation

A

Sicca- dry eyes
Dry mouth

Extranodular- only 30%, Arthritis/ arthralfia/ every organ.

hyperavtivity of B cells

144
Q

Sjoren syndrome is a/w high risk of ———— lymphoma

A

MALT

145
Q

Dgx of Sjoren syndrome

A

Anti-Ro (SSA
Anti LA (SSB)

if dgx is not clear&raquo_space; biopsy with lymphocytes

must rule HCV

146
Q

Which are the most common joints damage in Osteoarthritis?

A

Knee > hip > hands- DIP, PIP, 1st MCP (base of thumb) > cervical spine > lumbuscaral

in RA- no DIP involvment

147
Q

Haberden’s nodes and bouchards nodes are clinical finding which we can see in ————-

A

osteoarthritis
DIP, PIP

148
Q

היצרות הסדק המפרקי, סקלרוזיס וציסטות של עצם סאב-כונדרלית בהדמייה עם אוסאופיטים בקצוות העצם מכוון אותנו לאיזו מחלה?

A

OS

149
Q

What finding will rule out Osteoarthritis in pancutre of the synovial fluid?

A

WBC > 10000
think of a different Dgx

150
Q

Tx for OA

A

life changing, less stress on the joints
NSAIDS
steroids injections

151
Q

What is IgG-4 related disease, what is the pathophysiology and which type of pt is most common in?

A

גבריפ בגיל העמידה

Tendency to create tumor like lesions with atophic clinical presenation. every part in the body can be involve.

also called type 1 autoimmune pancreatitis

152
Q

What we will find in biopsy of IgG4 related disease

A

lympocytes and plasma cells which are positive for IgG4 + esoinophilia

153
Q

Tx for IgG related disease

A

1st line- Steroids
refoctory / steroids not good enough- RTX

154
Q

what are the 2 clinical signs patognemonic for Dermatomyositis?

A

Heliotrope rash- purple color and a bit adema in upper eyelid

Gottorn sign- red rash on hands joints and chunkles

Shawl and V sign- בחשיפה לשמש תיהיה פריחה אדומה סגלגלה, נראה כמו שאל / סימן וי של שיזוף

155
Q

Which test rq for defenitive Dgx of Inflammatroy myopathies

Derma to + polymyositis

A

**Muscle biopsy
**
Dermatomyositic- inf. in perimysium. CD4 +
Polymyositis- inf. in endomysium. CD8+

156
Q

What is the main different between polymyalgia reumatica to inflammatory myopathis?

A

in polymyalgia reumathica theres no muscle weakness- they will complain on stiff joints

157
Q

How can er describe the pattern of muscle involvment in poly + Dermatomyositis

A

proximal + symmetrical muscle weakness

קושי לעלות במדרגות, להרים רגליים, להסתרק, לעמוד מתנוחת ישיבה

158
Q

Which type of cancer is associated with Polymyositis and dermatomyositis

A

Adenocarcinoma

mainly dermatomyositis

159
Q

Tx for Inflammatory myopathies

A
  1. Steroids
  2. MTX/ AZA
  3. IVIG- in severe cases / refactory cases

MMF instead of MTX if- CKD,liver induff. or ILD)

160
Q

Cheracteristics of Inclusion body myopathy

  • muscles invovle
  • symmetric vs a-symmetric
  • tx
  • age
A
  • muscles invovle- proximal and distal + muscle of the face and swallowing but not the eyes
  • a-symmetric
  • tx- steroids + AZA/ MTX
  • age- males > 50
161
Q

Which type of Vasculiitis can be seen in FMF?

A

Polyarthritis nodusa and HSP

161
Q

Which type of Amyloidosis can be seen in FMF?

A

AA- mainly kidney involvment

Protenuria between FMF episodes

162
Q

Tx for FMF?

A

daily Cholcince PO (1.2-1.8 mg)

can raise risk for trisomy 21
but overall - can take in pregnancy
2nd line- IL-1 inhibitors (Canakinumab)

USMLE HY notes (34 decks)

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