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Med 1 > Connective Tissue/Jt Disease > Flashcards

Connective Tissue/Jt Disease Flashcards

1
Q

Types of SLE (4)

A

spontaneous
Discoid lupus - skin lesionsw/out systemic
drug induced
ANA negative lupus(arthritis, raynuds, Ro antiSS +, risk neonatal lupus in infants)

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2
Q

SLE epidemiology

A

Women of childbrearing age
African Americans

appears late childhoors

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3
Q

Clinical Features(11)

need 4/11

A

Serositis-pleursy, pericarditis
Oral Ulcers -nasopharyngeal/painless
Arthritis
Photosensitivity

Blood disorders -leukopenia, thrombopenia
Renal involvement- proteinuria(nephrotic), casts
ANA
Immunity phenom- anti smith, anti dsDNA, anti phos
Neurologic - seizure/psychosis

Malar rash
Doscoid Rash

fatigue, malaise, fever weight loss

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4
Q

Libman sacks endocarditis

A

SLE associated complication

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5
Q

Antihistone ABs present in

A

Drug induced SLE

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6
Q

ANA elv in?(7)

A

SLE, RA, Scleroderma, Sjogrens, mixed connective, polyomyositis, drug induced lupes

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7
Q

Anti Ro(SS-A) and La(SS-B)

Found in(5)

A

Sjogrens
Sub cutaneous SLE
Neonatal lupes (w/ congenital heartblock)

Complement deficiency (C2+C4)
ANA negative lupus
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8
Q

Reumatoid factor (2)

A

70% of RA

healthy 3%

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9
Q

C- anca

A

Wegeners

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10
Q

P-anca

A

polyarthritis nodosa

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11
Q

ESR elv(4)

A

infection
malignancy
rheumatologic
miscellaneous (pregnancy, necrosis)

r/o inflam and monitor course of inflamm

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12
Q

C reactive protein (6)

A 
inflame states/infection
MI
vaculitis
trauma
malignancy
pancreatitis

used for infection, more sense and specific than ESR.

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13
Q

HLA DR2 and DR3(3)

A

SLE both

DR3 - sjogrens
DR4 - RA

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14
Q

HLA B27 (3)

A

ankylosinf spondylitis
Reiters
psoriatic arthrits

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15
Q

Tx for SLE(4)

A

steriods for flares
NSAIDs for less severe

long term - hydroxychloroquine for constitutional symptoms, eye exam for retinal toxicity

cyclophosphamide - glomerulonephritis

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16
Q

Scleroderma

A

high quantity of collagen deposited -> complications and fibrosis

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17
Q

What is NOT part of drug SLE

A

Renal and CNS involvement

also usually not butterfly, alopecia and ulcers

Anti hitone antibodies

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18
Q

Drugs -> lupus (6)

A 
Hydralazine*
Procainamide *
Isoniazaid*
Chlopromazine*
mthyldopa
quinidine

See anti histone antibodies

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19
Q

Anticentomere

A

CREST 80%

sclreoderma 30%

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20
Q

antiscleroderma 70

A

Scleroderma 20%

Crest 10%

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21
Q

most common finding in SLE

A 
Lupus glomerulonephritis - 5 types
minimal lesion,
 mesangial lupus, 
focal proliferation,
 diffuse proliferative (40% and bad), 
membranous
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22
Q

Scleroderma types (2)

A

women 35-50

Diffuse - ANA and antitopoisomerase(anti-Scl 70), widespread skin, rapid onset, visceral involvement early, POOR prognosis–peropheral edema, polyarthritis, fatigue, weak, Renal failure?, interstial lung disease

Limited - anticentromere w/ CREST, limited to disatl extremities and face, delayedonst, pulm HTN and ischemic vascular disease, better prognisis

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23
Q

CREST syndrome

A

Limited scleroderma anticentormere positive

Calcinosis of digits
Raynauds
Esophageal dysmotility
Scerodactyl of finfgers
Telangiectasis - digits and nails
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24
Q

Ddx of Reynauds(7)

A 
primary
Scleroderma
SLE
Mixed connective disease
vasculitis - bergers
Medication - beta blockers, nicotine
thromoboangitis obliterans
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25
Q

Features of sclerodema general (6)

A

Raynauds

Cutaneous fibrosis - tight skin of face, extremities (sclerodactyl)

GI- both diffuse, limited - dysphagia, reflux

Pulm involvment- cause death

Cardiac - pericardial effusion, -> CHF, arrythmia

Renal Crisis - rare today

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26
Q

antiphospholipid antibody Syndrome

Find?

A

Hypercoagulable state -
idiopathic, SLE to other collagen disease

recurrent venous thrombosis- PE risk
Arterial thrombosis, fetal loss recurrent, thrombocytopenia,

Prolonged PTT of PT not correctable w/ plasma addition

Tx - Warfarin

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27
Q

Antitopoisomerase

A

Specific for Diffuse Scleroderma

antiscleroderma 70

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28
Q

Tx of scleroderm

A

no cure
Tx symptoms - NSAIDs (mkstl pain), H2/PPIs

Tx pulm and renal complications

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29
Q

Sjogrens Syndrome

2 types

A

Autoimmune in women

Multiorgan disease -> skin, lungs, thyroid, vessels, liver

Primary - dry mouth/eyes w/ lymphocytic infiltration of minor salivary glands

Secondary - Dry eyes/mouth + RA/scleroderma/SLE.polysyositis

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30
Q

Sjogrens has increase risk of death 2/2

A

nonhodgkins lymphoma

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31
Q

Dx of Sjogrens

A

Dry eyes (burning, red, blurred) out, arthralgia, extra glandular arthritis, nephritis, vasculitis

ANA, Anti Ro and La

Normocytic, normochromic anemia

Schirmer test - tilter paper -> lacrimal output

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32
Q

Pregnant people w/ antibodies to ro (SS-A) have increased risk of

A

child w/ neonatal SLE

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33
Q

Tx sjogrens

A

Pilocarpine /ceimeline

Artifical tears
NSAIDs, steroids

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34
Q

Mixed connective tissue disease

A

overlap syndrome

Features of SLE, RA, scleroderma, polymyosistis

Fins: pulm invovelment, esophageal dysmotility, polyarthritis, sclerodactyl cutaneous manifestations, myopathy, Raynauds

ANTi U1-RNP

High ANA + RF?

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35
Q

Arthritis is LESS likely RA if (3)

A

joints not symmetric

DIP is involved( can be every other jt)

Constitutional symptoms absent (especially in morning)

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36
Q

most common jts for RA

A

**Hands - PIP and MCP and wrist

See ulnar deviation

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37
Q

Boulonniere deformaties

Swan neck contractures

A
  • PIP flexed and DIP extended

- MCP flexed and PIP extended and DIP dlexed

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38
Q

Extra-articular manifestations of RA 2 main, a lot of others

A

malaise, weight loss, fever

  • rheumatoid nodules (elbow, sacrum, occiput)
  • Pleural effusion- has low glucose

Cardiac - nodules, pericarditis (40%), effusion

eyes- scleritis, scleromalacia,

Nervous - infarction of nerve trunk, systemic vasculutus- can’t move arm

Feltys syndrome

Blood - anemia, thrombocytosis

Cascultis

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39
Q

feltys triad

A

RA, neutropenia, splenomegaly

anemia, thrombocytopenia, LAD

Late in disease process

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40
Q

Osteophytes found in

A

osteoarthritis

changes in jts w/ RA usually more extensive w synovial involvement

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41
Q

Dx of RA

2 factors

A

high titres fo RF -> more severe disease but also nonspecific

Anticitullinated peptide/protein antibodies (ACPA) more sensitive

Elv ESR, C reactive protein
Anemia

sympotms >6wks

Radiograph: periarticular erosion, osteopenia, narrow jt space seen late

42
Q

Tx of RA

A

NSAIDs - pain control
Corticosteroids low dose if not manages

DMARDs early in Dx, have slow onset of action: Methotrexate*(SFx GI, oral ulcers, alopecia, hepatocellular injury),
Leflunamide
Hydroxychloraquine - (eye exam for retinal risk)
Sulfasalzine
Antitumor necrosis factor - etanercept, infliximab

43
Q

Variants of RA (2)

A

Feltys syndrome - anemia, neutropenia, splenomegaly, RA

Juvenile RA - befroe age 18, estra articular manifestations predom (STILLS disease_ or arthritis predom

44
Q

Increase uric acid production in (3)

Decrease excretion

A

hypoxanthine-guanine phosphoribosyltranserase deficiency - Lesch Nyhan syndrome
Phophoribysl pyrophosate synthetase overactivity
increases cel turnover

Renal disease
NSAIDs, Diuretics
Acidosis

45
Q

Asymptomatic hyperuricema Tx

A

should NOT be - 95% % remain asymptomatic

46
Q

Acute gouty attack precipitates (5)

A 
dehydration
decrease in temp
stress - emotional.physical
alcoholic use
starvation
47
Q

Synovial fluid anaysis in presumed gout attack

A

gram stain and culture to r.o septic arthritis

48
Q

Dx of gout

Role of serum Uric Acid

A

Needle shaped and negative biofringence rate crystals

Uric acid levels NOT helpful in Dx and can be normal

Radiographs -> punched out erosions

49
Q

Complications of gout (2)

A

nephrolithiasis - < 1%/yr

Degenrative arthritis in <15% pts

50
Q

Meds that increase uric acid levels

other causes?

A

thizide and loop diuretics

obesity
Alcohol
Dietary purine - red meat/seafood

51
Q

Acute gout Tx? (3)

A

Bed rest
NSAIDS - indomethacin
- not ASA( aggravates) or tylenol (no anti inflame)

Colchicine - alt for NSAIDs
- N/V, abdominal cramps, diarrhea, NOT for use w/ renal insufficiency and cytosine

Corticosteriods (oral prednisone 7-10 days), maybe intraarticular injection

52
Q

Prophylactic therapy for gout

A

wait till 2-3 acute gouty attacks ~/yr

allopurinol - xanthine oxidase inhibitor, decreases production, NEVER acutely. SFx rash/SJ syndrome

probenacid/ sulfinpyrazone - increase renal excretion (need working kidneys)

53
Q

Conditions -> increase Ca crystal deposition

A

hemochromatosis, hyperparathyroidism, hypothyroidsim, Bartter’s syndrome

Age and w/OA of joints

54
Q

Presentation of pseudo gout

Dx?

A

Commonlu affects knees and wrists
usually monoarticular, but can be poly

weakly positively birefringent rod/rhomboid crystals

Radiograph - chondrocalcinosis

55
Q

Tx of pseudogout

A

Tx underlying disorder,
NSAIDs, cochicine, intra-articular steriodinjections

Total joint replacement if appropriate

56
Q

Polymyosistis diagnostic criteria(4)

A

2/4 probable w/ increasing likelihood

Symmetric proximal muscle weakness
elvation in serum creatine phosphokinase
EMG finding of myopathy
Biopsy evident myositis

Rash = dermatomyositis

Usually females

57
Q

Inclusion body myositis -

A

oddball

M>F, no autoantibodies, distal muscles, and low CK

58
Q

Causes of dermatomyositis and polymyositis

A

genetically susceptible + env trigger -> immune activation

Dermato - hummoral immune mech

Poly - cell mediated

59
Q

dermatomyositis features

increased risk of?

A

symmetrical proximal muscle weakness over weeks-months (dysphagia 30%)

Rash - heliotrope (eyes, bridge of nose, cheeks)
Gottrons papules - papular erythematous scaly lesions over knuckles
V sign - rah on face, neck anterior chest
Shawl sign - rash on shoulders, upper back, elbows, knees

periungunal erythema

Risk of

  • vasculitis - GI, kidneys, lungs, eyes
  • CA, dermatomyocitis remits once CA removed
60
Q

Elv CK,**
LDH, Aldolase, AST and ALT elv

anti Jo 1 Antibodies(antisynthetase) or anti Mi2 antibodies

A

Dermatomyocitis polymyocitis

61
Q

Tx of dermatomyositis and polymyositis

A

Corticosteriods

immunosuppression if not responsive - methotrexate, cyclophosphamide, chlorambucil

PT

62
Q

Polymyalgia Rheumatica epidemiology

association w/ what disease?

A

elderly - rare M

autoimmune cause, self limited 1-2 yrs

10%-> temporal arteritis

63
Q

Features of Polymyalgia Rheumatica

A

Hip and shoulder pain - bilateral; pain w/ movement and normal strength

Morning stiffness common

Malaise, fever, depression

Jt swelling , 20% knees wriss, hands

64
Q

Dx of polymyalgia rheumatica

Tx?

A

clinical diagnosis
ESR elv >50-100

corticosteroids w/ response 1-7 days
- stop w/in 2 yrs for most,can be longer

65
Q

Fibromyalgia Epi and Key Dx points

A

Adult women w/ chronic non progressive waxing and waning pain

KEY - maple trigger points, tender to palpaion, symmetrical (18)’

Unknown etiology

66
Q

Symptoms of fibromyalgia

A

Stiffness**, body aches

changes w/ weather, sleep, stress, cold, worse in morning

Sleep dysruptions/unrefreshing

Anxiety/depression

67
Q

Dx of fibromyalgia

A

Pain (including axial) for min 3 months
11/18 tender pt sites

r/o: myofascial syndrome, rheumatoid, polymyalgia rheumatica, ankylosing spondylitis, spondyloarthropathy, chronic fatigue syndrome, lymes, hypothyroid, polymyosistis

68
Q

Tx of fibromyalgia

A

Reassure - > active and productive
Rx don’t work - SSRIs, TCAs?

cognitive behavioral therapy

69
Q

Seronegative spondyloarthropathies

Types(4) and associations

A

Associated w/ HLA B27 M>W

Ankylosing spondyltitis
reactive arthritis/reiters
Psoriatic arthrits
Arthropathy of IBD

70
Q

Common features of seronegative spondyloarthropathies (7)

A

negative RF
HLA B27 assocaition
Oligoarthritis -asymmetrical
enthesitis - inflam insertion of tendon onto bone
Inflam arthritis (axial/sacroiliac)
extra-articular features (eye -anterior uveitis, cardiac, renal)
Familial deposition(IBD, ankylosing spondylitis, psoriasis)

71
Q

characteristic of ankylosing spondylitis

A

low back pain worse in the morning and better w. use and warm showers

72
Q

Complications of ankylosing spondylitis(5)

A 
restrictive lung disease
cauda equina syndrome
spine Fx w/ cord injury
Osteoporosis
Spondylodiscitis
73
Q

Polyarticular Jt pain (8)

A 
RA
SLE
viral arthritis
Reiters Syndrome
Rheumatic Fever
Lymes
Gonococcal
Drug induced
74
Q

Monoarticular Jt pain (6)

A 
osteoarthritis
Gout
pseudogout
Truama
septic arthritis
Hemarthosis
75
Q

Tx of seronegative spondyloarthropathies (ankylosing spondylitis, psoriatic, IBD, Reactive)

A

NSAIDs
Anti TNF
PT
surgery?

76
Q

Reactive arthritis Characteristics and causes

A

clinicial diagnosis - suspected w/ moving arthritis

inflam oligoarthitis of usually lower its - preceeded by infection 1-4wks prior

Reiters syndrome is an example

Causes salmonella, shigella, camplobacter, chlamydia, yesernia

77
Q

GI infection of UTI infection followed by asymmetric arthritis 1-4wks later that moves

A

Reactive arthritis 2/2-> salmonella, shigella, camplobacter, chlamydia, yesernia

Fatigue, malaise, weight loss, fever

78
Q

Undifferentiated spondyloarthropathy

A

Features of reactive arthritis w/out evidence of UTI or GI infection

79
Q

Reiter syndrome

A

Can’t see(anterior uveitis)
Can’t pee(urethritis)
Can’t climb tree(arthritis)

may not always occur together,
Post infection

80
Q

Dx of active arthritis /reiters

A

synovial fluid does not grow anything, Antibody response

r/o infeciton and crystals

81
Q

Tx of reactive arthritis

A

NSAIDs, maybe sulfasalazine and azathioprine

Abs generally NOT used

82
Q

Psoriatic arthritis

A

10% of psoriasis Pts

gradual onset after skin condition

Aymmetric and polyarticular

Upper extremities and small its more common

NSAIDs

83
Q

Temporal arteritis/giant cell arteritis

affects what?

Whos affected

A

Vasculitis of unknown cause >50 yrs W>M

temporal arteries frequently but also aorta or carotids

-> increase risk of aneurism and dissection

84
Q

Temporal arteritis featured(5)

Associated w/

A 
constitutional - malaise, fatogue, fever
HA - severe
Visual impairment * only 25-50%
Jaw apin w/ chewing
Tenderness of temporal artery, no pulse

40% have polymyalgia rheumatica

85
Q

Vasculitis types

A

Large vessel - takayasu;s arteritis, temporal arteritis

Medium - PAN, kawasaki, Wegeners, Churg straus, mucrscopic polyangitis

Small - henrocj stolen purpura, hypersensitivity vasculitis, Behcet’s Syndrome

86
Q

Dx of temporal arthritis (5)

A 
ESR elv 
biopsy key (neg does not r/o)

Age >50
New HA
tender/palpable artery
jaw claudication

87
Q

Tx of temporal artheritis

A

High dose steroids early*
- do NOT wait for biopsy for fear of blindness

Tx 4wks minimum but maybe up to 2-3 yrs

Follow ESR

88
Q

Takyasu’s Arteritis

demographic

Affects what artery

Dx?

A

young asian women

vasculitis of aortic arch and branches -> stenosis

Dx w/ areriogram

89
Q

Young women w/ decreased peripheral pulses, discrepent PB of arm vs leg and arterial bruits

A

Takyasu arteritis

90
Q

Features of Takyasu arteritis

A

Constitutional - fever, night swears, malaise, arthalgias

Pain/tender vessels

*Absent pulses in carotid, radial ulnar artris, aortic regurg

Ischmia signs

-> limb ischemia, aoritc regurg, stroke, secondary HTN (renal stenosis)

91
Q

Tx of takasu

A

Steriods
Treat HTN 2/2 renal stenis

Surgery to recannulate stenosis

92
Q

Churg Strauss

Associated w/ ?

A

vasculitis of many organ systems

fever, fatigue, weight loss
Prominent respiratory symptoms: asthma/dyspnea

Skin lesions - sub Q nodules and palpable purpura

P-ANCA positive

poor prognosis - Steroids Tx

93
Q

Wegener’s Granulomatosis

Affects?

Features

A

Vasculitis of kidneys and lower respiratory tract

Up respiratory symptoms, purulent/bloody d/c
Oral ulcers
Pulm symptoms - cough hemoptysis, dyspnea
Renal involvement - glomerulonephritis (death)
Eye - conjunctivitis/scleritis
Arthralgia/myalgia
Tracheal stenosis

94
Q

Dx of wegenrs

A

CXR abnormal - nodules or infiltrates

Elv ESR, Anemia, hematuria C-ANCA

biopsy confirms

95
Q

Prognosis and Tx of wegeners

A

POOR, die w/in yr

Tx - cyclophosphamide and corticosteroids -> remission

Renal transplant?

96
Q

Polyarteritis nodosa

Associated w/ (3)

A

vasculitis of nervous system and GI tract

Hep B, HIV, drug Rxns

PMN invasion of all layers and fibrinoid necrosis _ intimal proliferation -> luminal decrease

97
Q

Symptoms of Polyarteritis nodusa

A

NO pulm involvement

Fever/weakenss, weight loss, myalgia, arthralgia

ab pain w/ bowel angina

HTN, mononeuritic multiples, livedo reticularis

Maybe P-Anca +

98
Q

Polyarteritis nodusa Dx

A

P anca + can be present

ESR elveatied
Fecal occult blood

Tx w/ steriods, cyclophosphamide

POOR prognosis

99
Q

Burgers Disease - Thromboangiitis obliterans

A

Young men who smoke

Acute inflame -> ischemia nd gangrene

Clausdicaion, cold cyanotic, painful extremities, ulceration

STOP smoking

100
Q

Hypersensitivity Vasculitis

A

Small vessel vasculitis in rsponse to: Drug, (penicllin, sulf), infection or other

Skin involved -> palpable purport, macule, vesicles; painful lesions

constitutional sympoms

Dx w/ biopsy

Prognosis good w/ withdrawal of agent

Med 1 (11 decks)

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