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Clin med 1 - DERM/RHUM - exam 1 > Rhuem > Flashcards

Rhuem Flashcards

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1
Q

Define RA and explain pathophysiology

A

INFLAMMATORY SYNOVITIS

autoantibodies attack host
Neutrophils and T cells in the synovial fluid cells are activated

Cytokines, Interleukin-1, TNF-alpha, Chondrocytes all attack cartilage.

Synovium then digests cartilage, inflammatory molecules released containing interleukin-1 and TNF alpha

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2
Q

shared epitope in RA (HLA___?)

A

HLA BRB1

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3
Q

epidemiology of RA

A

FM 3:1
>15 y/old
more common in smokers

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4
Q

what areas does RA spare?

A

Lower back

DIP

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5
Q

WHat is a Pannus?

A

abnormal tissue growth cause by thickening or inflammation of the synovium.

Cytokines cause new blood vessels to develop in the synovium causing growth of extra tissues  eventually forms pannus

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6
Q

what is a characteristic of RA seen on Physical exam and where would these be found?

A

Nodules

seen on
hands
olecranon
Calcaneal tuberosity

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7
Q

2 deformities seen in RA

A

Boutonniere deformity: flexion of the PIP and hyperextension of the DIP joint.

Swan neck deformity: hyperextension of the PIP and flexion of the DIP joint.

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8
Q

What would you see on a blood test in pts w/ RA

A

Hypochromatic-microcytic anemia with low serum Fe

or normal iron-binding capacity almost universal in patients with active RA

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9
Q

what would you find on Physical exam in a pt with RA

A

Warm tender erythematous joints, hands and wrists

DIP are notably excluded

Ulnar Deviation of the digits

Boutonniere’s deformity

Rheumatoid Nodules

Cysts in popliteal space (Baker’s cyst)

Decrease ROM in joints

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10
Q

What antibodies are usually positive in pts w/ RA

A

Anti-CCP
ANA (20%)
RF (although does not rule out RA if negative)

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11
Q

what are 2 subtypes of RA

A

ACPA + is a more aggressive clinical phenotype (67%)
responds better to tx

ACPA - respond less to common treatments

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12
Q

how many points id dx for RA

A

6 points

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13
Q

first line tx for RA

A

DMARD
methotrexate

Leflunomide ( alt to methotrexate high GI side affects)

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14
Q

what do you prescribe pts w/ RA while waiting for DMARDs to take effect

A

corticosteroids (prednisone) for flares

NSAIDs
COX-2

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15
Q

complications seen w/ RA

A

feltys synd
baker cyst
carpal tunnel
altanto-axial subluxation

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16
Q

what is felty’s synd

A

Triad of RA + Neutropenia + Splenomegaly

complication of RA - tx RA and this should resolve

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17
Q

what is Polymyalgia Rheumatica (PMR)

A

inflammatory disorder of pain associated with the proximal muscles

seen in elderly, more common in white women

HIGH correlation w/ GCA

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18
Q

key feature of PMR

A

key feature is pain and not weakness, strength is IN TACT

Symptoms worse in AM

Does not cause swollen joints

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19
Q

Patient describes general aches and pains, stiffness, especially upper arms, thighs, hip girdle and shoulders. “trouble getting dressed”
Dx????

A

PMR

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20
Q

Gold standard Dx of PMR

A

ESR.

abnormally high.

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21
Q

Tx PMR

A

prednisone -> QUICK response

If no improvement in 2-3 weeks, unlikely PMR

NSAIDS don’t work

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22
Q

Who should pts w/ PMR always be referred to?

A

ophthalmology for blindness

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23
Q

What is reactive arthritis?

A

Auto-immune condition that can occur post infection (typically Genitourinary or Gastro-intestinal infections)

Reaction” to an infection which took place outside of the joints

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24
Q

what are the common pathogens responsible for reactive arthritis?

A 
Chlamydia
Campylobacter
Salmonella
Shigella
Yersinia
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25
Q

what haplotype is Reactive Arthritis strongly linked to?

A

HLA-B27

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26
Q

classic triad seen in reactive arthritis

A

non-infectious urethritis, arthritis
conjunctivitis

CAN’T SEE, CAN’T PEE, CAN’T CLIMB A TREE

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27
Q

Si/Sx of reactive arthritis

A

Joint inflammation characterized by redness, swelling, pain and warmth in the joints.

Lower limbs and Sacro-iliac joints most effected

Inflammation of the urinary tract and the conjunctiva.

Other symptoms: fever, weight loss, lower back pain, heel pain.

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28
Q

how to tx reactive arthritis

A

Tx offending agent (antis)
prednisone for joints

DMARDS: When NSAIDs and Steroids are ineffective Sulfasalazine (good when triggered by GI)

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29
Q

what is JIA?

A

Inflammation of the CT seen in children

6 wks of pain and swelling with exclusions of other causes

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30
Q

what are the 5 types of JIA

A 
polyarticular arthritis 
oligoarticular arthritis 
systemic onset
enthesis (related arthritis)
psoriatic arthritis
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31
Q

what type is JIA manifests w/ CONSTANT pain and resembles the adult form of RA?

A

Polyarticular arthritis

arthritis in 5 or more joints with major symptoms of pain in the knees, ankles, wrists, fingers, elbows and shoulders.

children of ANY age

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32
Q

JIA that manifests w/ Arthritis in 1 or more joints,

sacroilitis, DIP joint synovitis, dactylitis and nail pitting + psoriatic rash or family hx of psoriatic rash

A

psoriatic arthritis

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33
Q

child presents w/
Repeated fevers to 103F w/ fluctuating salmon colored rash. Inflammation of the internal organs, enlarged lymph nodes and spleen?

A

Systemic Onset arthritis

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34
Q

with Systemic Onset arthritis when is malignancy suspected?

A

with anemia and splenomegaly

Complications involve pericarditis and pericardial effusions

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35
Q

young boy (8 yrs old) presents w/ Pain and inflammation where the ligaments and tendons attach to bones along w/ back pain. Dx?

A

Enthesis (Related Arthritis)

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36
Q

what type of JIA has a high risk of iritis?

A

Oligoarticular Arthritis

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37
Q

young girl presents w/ asymmetrical morning stiffness in LARGE joints knees, ankles, elbows, wrists. Does not complain of much pain, mostly stiffness. Dx?

A

Oligoarticular Arthritis

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38
Q

Pt presents w/ proximal muscle weakness, upper and lower limbs, bilaterally. she complains of difficulty raising arms, lifting object over head, combing hair. Trouble climbing stairs, getting up from sitting. Dx?

A

Polymyositis

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39
Q

Polymyositis vs PMR

A

polymyositis - Proximal muscle weakness, upper and lower limbs, bilaterally

PMR - pain w/o WEAKNESS

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40
Q

what lab values would you expect to be elevated in polymyositis

A

MUSCLE ENZYMES

CPK
LDH

CHECK LIVER ENZYMES

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41
Q

define heliotrope rash

A

purple discoloration on upper eyelids, flat red rash on cheeks and upper trunk

Dermatomyositis

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42
Q

2 identifying fts of Dermatomyositis

A

heliotrope rash

gorttons papules

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43
Q

young girl complains of muscle weakness. A muscle bx is done and reveals perivascular and perimysial inflammation. dx?

A

dermatomyositis

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44
Q

Tx for dermatomyositis and polymyositis

A

prednisone (corticosteroids)

extra skeletal manifestations 
Azathioprine
Cyclophosmaide
Chlorambucil 
Cyclosporine

INF inhibitors –infliximab
Anti-CD20 (Rituximab)

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45
Q

60 y/o man complains of frequent FALLING along w/ weakness of DISTAL muscles and facial muscles. Dx?

A

inclusion body myositis

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46
Q

how to differentiate inclusion body myositis from polymyo and dermatomyo

A

inclusion body Can have asymmetrical weakness making it different than (PM, DM)

DM - rash and seen in children

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47
Q

what dz is associated w/ Raynaud’s phenomenon, RA, SLE, Sjogren’s, CV dz, Cancer
drugs?

A

polymyositis

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48
Q

Name large vessel vasculitis conditions?

A

Giant Cell / PMR

Takayasu Arthritis

49
Q

Name si/sx assoc w/ large vessel?

A 
Limb claudication
Asymmetric blood pressures
Absence of pulses 
Bruits 
Aortic dilation
50
Q

name medium vessel vasc conditions?

A

Polyarteritis Nodosa

Kawasaki Disease

51
Q

name skin manifestations seen in medium vessel vasc?

A

palpable purpura
livedo reticularis
digital gangrene

52
Q

name small vessel vasc conditions?

A

Microscopic Polyangiitis (MPA)

Granulomatosis with Polyangiitis GPA (Wegener’s)

IgA Vasculitis (Henoch-Schönlein)

53
Q

what is standard approach to screening for large-vessel vasculitis

A

CT and MRI

54
Q

what is a concerning sx where you MUST r/o infective endocarditis?

A

splinter hemorrhages

55
Q

is low serum complement (C4) usually seen in vasculitis?

A

NO - seen in SLE

56
Q

65 y/o pt comes in complaining of new onset headaches, jaw claudication (difficulty chewing food) and unexplained fever. Dx?

A

GCA (PMR)

vision loss is also common
tenderness of temporal artery

57
Q

what is gold standard for dx GCA

A

Temporal artery biopsy

58
Q

what sx is most highly predictive of a positive temporal artery for diagnosis?

A

jaw claudication

59
Q

Tx of GCA in pts w/o visual loss

A

prednisone

Pt will respond QUICKLY

60
Q

Tx of GCA in pts w/ visual loss

A

methylprednisolone

61
Q

Pts lab results show Normochromic normocytic anemia and elevated ESR / CRP. Dx?

A

Takayasu Arteritis

pt will appear unwell

62
Q

what is Subclavian steal syndrome

A

Subclavian artery involvement common -> stenotic lesion proximal to origin of vertebral artery can lead to subclavian steal syndrome

Reduced blood pressure in one or both arms

Asymmetrically diminished arterial pulses in the arms and legs

63
Q

epidemiology of Takayasu Arteritis

A

WOMEN 7:1

ASAIN

64
Q

Tx for Takayasu Arteritis

A

acute - prednisone

chronic - angioplasty surgical bypass

65
Q

what dz spares the lungs, but kidney often affected (casing renin mediated HTN) and NOT assoc w/ ANCA.

A

Polyarteritis nodosa (PAN)

66
Q

pt comes in presenting of nonspecific complaints: fever, malaise, weight loss, anorexia, and abdominal pain (Constitutional sx), HTN, elevated ESR. dx?

A

Polyarteritis Nodosa- PAN

67
Q

causes of Polyarteritis Nodosa- PAN?

A

idiotpathic
HBV
HBC

68
Q

Tx of PAN

A

mild disease: Prednisone

mod-severe disease:
prednisone w/ Cyclophosphamide

severe disease: methylprednisolone

69
Q

key finding in Kawasaki

A

Child w/ fever lasting >5 days with treatment

70
Q

Si/Sx in Kawasaki

A

Bilateral bulbar conjunctival injection

Oral mucus membrane changes (injected or fissured lips, injected pharynx, strawberry tongue)

Peripheral extremity changes (erythema of pals and soles of feet, edema of hands or feet, periungual desquamation

Polymorphous rash

Cervical lymphadenopathy

71
Q

Tx Kawasaki

A

IVIG

Aspiring - r/o viral infection Reyes

72
Q

what is Behçet Disease

A

triad of of aphthous oral ulcers, genital lesions, and recurrent eye inflammation

73
Q

presence of HLA ___ is a risk factor for Behçet

A

HLA B51

74
Q

leading cause of blindness in Japan?

A

Behçet

75
Q

Tx of Behçet

A

steroids
colchicine / thalinomide

severe CNS or ocular sx- Cyclophosphamide

76
Q

define allodynia

A

pain due to a stimulus which does not normally provoke pain

77
Q

2 most common sx related to FM

A

pain

FATIGUE (80%)

78
Q

1st and 2nd line tx for FM

A

1st NSAIDS/ tramadol

2nd TCA (r/o SUICIDE) 
Amitriptyline, nortriptyline

3rd SSRIs/SNRIs
Duloxetine, Venlafaxine
Fluoxetine, Citalopram

79
Q

3rd line tx for FM used instead of Amitriptyline

A

Cyclobenzaprine

80
Q

define raynauds

A

abrupt onset of well-demarcated pallor of the digits which progresses to cyanosis with pain and often numbness—followed by reactive hyperemia on rewarming.

81
Q

primary vs secondary raynauds

A

Primary Raynaud’s is not associated with an underlying cause

Secondary Raynaud’s is associated with or caused by some other systemic illness or disease process
Common in pts w/ CT diseases

82
Q

describe the link b/w scleroderma and raynauds

A

90% of patients with scleroderma experience Raynaud’s phenomenon, and it may be a presenting manifestation in many of them.

83
Q

physical emac findings in primary vs secondary raynauds

A

In primary Raynaud’s phenomenon, the physical exam is normal between attacks.

in secondary Raynaud’s phenomenon, pits or ulcerations on the fingertips may be present in patient’s with scleroderma, CREST syndrome or thromboangiitis obliterans.

84
Q

The dihydropyridine Ca++ channel blockers (e.g., Amlodipine and Nifedipine) are the most effective pharmacotherapeutic agents for ?????.

A

Raynauds

85
Q

who does sceroderma most commonly present in?

A

women in 30s

AAs

86
Q

Pathophysiologic Triad in scleroderma

A

Vasculopathy, including Raynaud’s Phenomenon—the earliest vascular complication of SSc

Inflammation and Autoimmunity—Cellular Immunity: Evidence of inflammation and autoimmunity is an invariable feature that SSc shares with other rheumatic diseases such as SLE and RA.

Fibrosis: Fibrosis of the skin and multiple internal organs is the distinguishing feature of SSc.

87
Q

what is the hallmark of scleroderma

A

thickening and hardening of the skin (scleroderma), but most patients also manifest characteristic abnormalities in the lungs, g.i. tract, kidneys and heart.

88
Q

characteristically develop extensive skin induration and early progressive internal organ involvement.

A

with diffuse cutaneous systemic sclerosis (dcSSc)

89
Q

commonly present with long-standing Raynaud’s phenomenon, modest skin changes and indolent internal organ dz.

A

limited cutaneous systemic sclerosis (lcSSc)

90
Q

where on the face is spared during SLE butterfly rash

A

nasolabial folds

91
Q

who antibodies are positive in SLE

A

ANA test is positive in virtually all patients;

Anti Sm Abs are highly specific for SLE.

92
Q

tx for sle

A

Prednisone
hydroquinone

Immunosuppressive Agents, alkylating agents:
Cyclophosphamide; purine synthesis inhibitors:
Azathioprine and MMF; folate antagonist: methotrexate

93
Q

what age group is Sjogren’s Syndrome ABSENT in

A

children

94
Q

what diagnostic test is used for sjogens ?

A

Schirmer test, performed without anesthetic eye drops.

95
Q

Pharmacological Tx of sjogens -

A

Topical cyclosporine
although there is a concern for herpesvirus infections.

Oral pilocarpine and cevimeline may stimulate functional exocrine glands and improve sx.

Nystatin utilized for thrush complication.

96
Q

what age group and gender is sjorgens seen in?

A

women 30-50s

97
Q

composition of crystals in gout vs psueodgout

A

gout: monosodium urate
pseudo: Ca++ pyrophosphate dihydrate (CPPD)

98
Q

arthrocenteiss shows:
Needle shaped
Negative birefringence under polarized light microscopy

dx?

A

Gout

99
Q

after a 24 hour urine test of a pt suspected of Gout. Results show elevated level.

is this person an overproducer or underexcreter?

A

overproducer

90% of pts are underexcreters and will have a NORMAL level

100
Q

what are examples of Xanthine oxidase inhibitor

A

Allopurinol - 1st line, not in mod-severe renal failure

Febuxostat

101
Q

Uricosuric: underexcretor prophylaxis

A

Probenecid - 1st line in pts w/ CKD (cannot tolerate allopurinol)

Sulfinpyrazone

102
Q

Tx for acute gout attack

A

NSAID - indomethacin
Colchicine
corticosteroids (r/o infection)

103
Q

pt comes in w/ valgus (angulation toward midline) deformity of the knee. what is this HIGHLY suggestive of

A

CPPD

pseudogout

104
Q

arthrocentesis shows
positive birefringence, rhomboid crystals

dx?

A

pseudogout

105
Q

what radiographic finding is DIAGNOSTIC of pseudogout?

A

Radiographic findings of joint cartilage calcified and calcium deposits in joint spaces are DIAGNOSTIC!

106
Q

acute tx of pseudogout

A

NSAID
colchicine
steroids

107
Q

chronic tx of pseudo

A

colchicine

NSAID

108
Q

90% of pts w/ gout are ________?

A

underexcretors

109
Q

most common joint affected in gout vs pseudo

A

gout 1st MTP (podarga)

pseudo knee

110
Q

Most common cause of Necrotizing glomerulonephritis

A

MPA

111
Q

what vasc syndrome presents w/ splinter hemh

A

MPA

112
Q 
pt presents w/ Purpura
 Pulmonary hemorrhage
 Interstitial lung fibrosis 
Ulcers 
Splinter hemorrhages

dx?

A

MPA

113
Q

Pt presents w/ Classic triad of upper and lower respiratory tract disease with glomerulonephritis
dx?

A

GPA

114
Q

crusting, ulceration, bleeding, and/or perforation of nasal septum common

A

GPA

115
Q

what is the most common vasculopathy in children

A

IgA

116
Q

describe classic tetrad and what sx it relates to?

A

IgA Vascu

Purpura: ALL

Arthralgia/arthritis: Joint symptoms second most common manifestation, occur in over 50% of pts (usually affects LOWER extremities )

Abdominal pain: Colicky pain occurs in approx. 50% of patients and GI bleeding in approx. 20-30% (intussusception most common complication)

Renal disease: Frequency of renal involvement ranged from 21-54% (more common in older children)

117
Q

Pt presents w/
Proptosis:
Ptosis:
Ophthalmoplegia:

Scleritis

dx?

A

GPA

118
Q

Tx of GPA

A

Cyclophosphamide PO plus Prednisone

Rutuximab PO plus Prednisone

Continue Cyclophosphamide with prednisone

Clin med 1 - DERM/RHUM - exam 1 (2 decks)

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