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CPR2 Exam 1 > Connective Tissue Disorders And Vasculitis - Dr. McGowen > Flashcards

Connective Tissue Disorders And Vasculitis - Dr. McGowen Flashcards

1
Q

SLE prevalence and what is it

A

multisystem disorder
= F
= black and latino
= autoimmune to nuclear Ags (TYPE 3 hypersensitivity)

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2
Q

SLE SX you can see

A
  1. fever
  2. malar rash, photosensitivity
  3. arthritis inflammatory
  4. Pericarditis (pain laying supine, ST elevation diffuse, crushing sharp CP)
  5. Libman sacks endocarditits
  6. thrombosis
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3
Q

SLE DX how

A
  1. dsDNA ABs
  2. Sm (Smith) ABs
  3. low C3 / C4
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4
Q

SLE TX

A
  1. avoid sun
  2. NSAIDs for pain
  3. Glucocorticoids
  4. Hydroxychloroquine
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5
Q

SLE management for risks and prevention ** 6

A
  1. SLE increases atheroscleorosis : minimize risk
  2. no smoking, obesity, htn, hypercholestemia, exercise
  3. flu vaccine
  4. pneumonococcal vaccine every 5 years
  5. higher malignancy risk so screen
  6. glucocorticoid use = minitor for avascular necrosis in bones
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6
Q

Early stage SLE reason for death

A
  1. infections (opportunistic infections from immunosuppressive TX)
  2. Kidney + CNS disease *
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7
Q

later stage SLE reason for death

A
  1. Accelerated atherosclerosis from chronic inflammation (%x MI risk)
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8
Q

Anti-Phospholipid AB syndrome (APS) : secondary vs primary

A

primary : APS ABs only

secondary : APS and SLE (1/3 patients with SLE have APS

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9
Q

Anti-Phospholipid AB syndrome (APS) :

AB serology

A
  1. Anti- Cardiolipin (aCL)
  2. Lupus anticoagulant (LA)
  3. Beta 2 glycoprotein 1 (anti-B2 GPI)
    = all 3 testes 2 times at least 12 weeks after
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10
Q

Anti-Phospholipid AB syndrome (APS) :

what are risks and what happens

A
  1. venous and arterial thrombosis
  2. miscarriage
    = prolong PTT clotting time not reversed by plasma (plt free)
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11
Q

aCL features

A

+ in APS

= can cause false + syphilis test in non-treponemal test

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12
Q

APS tx

A

systemic anticoagulation

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13
Q

cotton wool spots are seen in

A

SLE
APS
DM
systemis HTN

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14
Q

Lupus - Like syndrome / Drug Induced Lupus (DIL)

happens how and drugs

A

= drug induce demethylation of DNA

  1. procainamide
  2. Hyrdalazine
  3. Isoniazid
  4. Sulfasalazine
  5. Hydrochlorothiazide
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15
Q

Lupus - Like syndrome / Drug Induced Lupus (DIL) DX and TX

A
    • ANTI- histone ABs

2. stop medication

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16
Q

Neonatal lupus happens how

A

cutaneous lesions look like SLE, only this is NOT SLE
= mother can have Sjögrens, SLE, or normal
= mother has Anti- Ro (SSA) ABs**

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17
Q

Neonatal lupus risks

A
  • ***congenital heart block = life threatening

- hemolytic anemia, thrombocytopenia, rashes, arthritis

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18
Q

Neonatal lupus DX

A

+ Anti-Ro mother

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19
Q

Neonatal lupus TX

A
  1. Delivery when fetus is in distress
  2. Dexamethasone when fetus has 1st or 2nd degree heart block can prevent progression
  3. Hydroxychloroquine : prevents in future child only
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20
Q

Discoid Lupus is what

A

part of or not part of SLE

=skin well-defined inflammation plaques —-> atrophic, disfiguring scars (usually on head)

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21
Q

Discoid Lupus DX and Tx

A
  1. Biopsy

2. photo-protection + topical anti-inflammatory / anti-malaria drugs

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22
Q

Discoid Lupus can look like what 3 things

A
  1. ring worm (tinea)
  2. Psoriasis
  3. Morphea (local scleroderma)
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23
Q

Scleroderma (SSc) 3 types

A
  1. local
  2. Limited (CREST)
  3. Diffuse
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24
Q

hallmark of SSc

A

thickening and hardening of skin
= multisystemic
= Raynaud Phenomenon (pigment changes seen first)

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25
Q

Localized scleroderma is what

A

localized patches of skin on different parts of body (joints, muscles can be involved)

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26
Q

Localized scleroderma SX details

A

MORPHEA (patches)
= discreet area of discolored skin
= red/purple oval –> thickens –> central hypopigmentation + erythematous border around
= asymptomatic , itching, pain

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27
Q

Localized scleroderma TX

A

resistant to tx, immunosuppressives, PT of joints

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28
Q

Limited (cutaneous) Scleroderma is what and SX

A

= hardening and thickening inflammatory condition involving internal organs also
1. C : Calcinous cutis (calcification)
2. R : Raynoud’s (digital ischemia)
3. E : Esophageal dysmotilty
4. S : Sclerodactyly
5. T : Telangiectasia
= progressive Pulmonary Artery HTN** SOB

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29
Q

Limited (cutaneous) Scleroderma DX

A
    • Anti - centromere *
  2. Right heart catheterization for PAH (gold standard)*
  3. esophagus barium swallow , hand xray
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30
Q

Limited (cutaneous) Scleroderma TX

A

manage each organ system

  1. warm clothing
  2. CCB (raynauds)
  3. ACE inhibitors (control htn)
  4. glucocorticoids (slow SSc**) can cause renal crisis
  5. Phosphodiesterase Type 5 inhibitor : Pulm htn**
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31
Q

Diffuse (Cutaneous) SSc is what and sx 4

A

= inflammatory condition thickening of tissues includes internal organs

  1. extr. + trunk
  2. Interstital Lung Disease **, dry velcro-like crackles, SOB
  3. Renal crisis
  4. Raynauds phenomena later stages
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32
Q

Diffuse (Cutaneous) SSc DX

A
    • Anti- scl 70 (Anti-DNA topoisomerase 1)
  2. Anti-RNA polymerase 3
  3. ILD : interstitial lung d, CXRay, CT
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33
Q

ILD

A 
interstitial lung disease 
= dry velcro like crackles
= SOB
= CT : ground glass or honeycomb looking 
= Pulmonary Function test
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34
Q

Diffuse (Cutaneous) SSc TX

A
  1. ILD : glucocorticoids, O2 + Pulmonary rehab = renal crisis can happen
  2. manage each organ system like in limited SSc
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35
Q

Systemic Sclerosis : SKIN

A

= hyper or hypo pigment

= fibrosis of skin glands (dry itchy)

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36
Q

Systemic Sclerosis : Pulmonary

A
  1. ILD : DIFFUSE SSc, Dx with PFT or CT, Auscultation velcro crackles
  2. PAH : pul artery htn, LIMITED SSc, dx right heart catheterization
  3. high risk bronchoalveolar carcinoma
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37
Q

Systemic Sclerosis : Renal

A

chronic kidney disease , renal crisis (Diffuse, when using glucocorticoids)

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38
Q

Systemic Sclerosis :Endocrine

A

hypothyroid from thyroid fibrosis

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39
Q

Systemic Sclerosis : MSK

A

Carpal tunnel : fibrosis on tendon sheaths

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40
Q

Systemic Sclerosis : Cardiac

A

Myocardial fibrosis, Cardiomyopathy, arrhythmia, pericarditis, pericardial effusion

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41
Q

Systemic Sclerosis : GI

A

Xerostomia, Esophagus = GERD and dysphagia

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42
Q

Sjögren Syndrome prevalence and what

A

autoimmune to exocrine glands

postmenopause, F

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43
Q

Sjögren Syndrome SX

A
  1. sicca sx : dry eyes, mouth , oral infaction, cavitites, parotid and other salivary gland enlargement
  2. Keratoconjunctivits sicca : X tears
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44
Q

Sjögren Syndrome risk

A

B-cell non-Hodgkin lymphoma (MALToma)

45
Q

Sjögren Syndrome DX

A
    • Anti SSA/Ro
  2. Anti SSB/La
  3. Polyclonal Hypergammaglobuulinemia
  4. Labial salivary gland (LIP) biopsy, if unilateral gland enlargement bx it
46
Q

Sjögren Syndrome TX

A

tx problems, artificial tears, infection, drink water more

47
Q

Inflammatory myopathies 3 types

A
  1. Dermatomyositis (DM)
  2. Polymyositis (PM)
  3. Inclusion Body Myositis (IMB)
48
Q

Inflammatory myopathies most common sx

A

bilateral proximal muscle weakness

= hard time standing from chair or bathtub or going up stairs

49
Q

Inflammatory myopathies dx typical

A

high CK and adolase

ESR and CRP is normal

50
Q

Dermatomyositis what happens, SX

A
  1. weakness with no sensory sx (early proximal, later distal)
  2. skin lesions : Gottron’s Patches / papules, Heliotrope rash, V-neck erythmia
51
Q

Dermatomyositis risk

A

occult
malignancy
= when dx do age appropriate screening

52
Q

Dermatomyositis DX

A
  1. high CK (creatine Kinase)
  2. high aldolase
  3. Anti Jo-1
  4. Biopsy : perimysial and perivascular inflammation = perifascicular atrophy
53
Q

Dermatomyositis tx

A

glucocorticoids

54
Q

Gottron’s patches and papules

A

raised violet lesions bumps over dorsal hand of the finger joints

55
Q

Heliotrope rash

A

purple red edema over upper eyelids

56
Q

V-neck erythema

A

Poikiloderma : “Shawl sign” red over neck and shoulders upper chest and back

57
Q

Polymyositis what happens sx

A

no skin changes,

proximal muscle weakness

58
Q

Polymyositis dx

A
  1. high CK and adolase
  2. Anti Jo-1
  3. Biopsy : endomysial inflammation + non-necrotic muscle fibers, no other disease sxs (IBM or Muscular dystrophy)
59
Q

Polymyositis TX

A

glucocorticoids

60
Q

Inclusion Body myositis (IBM) what happens sx and prevalence

A 
more in M (only one)
and white (others were black)

weakness in finger flexion and quadriceps

61
Q

Inclusion Body myositis (IBM) DX

A
  1. mild elevated CK
  2. Anti -cN1A (dont have to know)
  3. Biopsy : endomysial inflammation + rimmed vacuoles*, invasion of non-necrotic muscle fibers
62
Q

Inclusion Body myositis (IBM) TX

A

resistant to tx

support with pain killers and Physical therapy

63
Q

IGA vasculitis other name and DX

A

Henoch- Schönlein purpura (HSP)

= IgA deposits on biopsy

64
Q

Henoch- Schönlein purpura (HSP) TETRAD

A

Henoch- Schönlein purpura (HSP)

  1. Palpable purpura (not thrombocytopenia)
  2. Arthritis, arthralgia
  3. ABD pain
  4. Glomerulonephrits, renal disease
65
Q

Henoch- Schönlein purpura (HSP) TX and prevalence

A

kids more common, in small vessels

= support + glucocorticoids

66
Q

Anti-Glomerular Basement Membrane other name and what happens

A

GOODPASTURE SYNDROME

  1. Hematuria (glomerular capillaries)
  2. Hemoptosis (pulmonary capillaries can cause hemorrhage)
67
Q

GOODPASTURE SYNDROME DX

A

renal bx : Anti-BM ABs

in small vessels

68
Q

GOODPASTURE SYNDROME TX

A
  1. plasmapheresis (remove ABs)

2. Glucocorticoids

69
Q

Granulomatosis with polyangitis (GPA) other name and prevalence

A

WEGENER’S GRANULOMATOSIS

= MALE*, over 40yo

70
Q

WEGENER’S GRANULOMATOSIS what happens

A
  1. Granulomatous inflammation
  2. necrotizing vasculitits
  3. segmental glomerularnephritis
  4. 90% NASAL involvment (saddle nose bleeding crusting)
  5. lung cavitary lesions = alveolar hemorrhage
  6. venous thrombotic events
71
Q

WEGENER’S GRANULOMATOSIS dx

A
  1. ANCA +

2. biopsy is granulomas in small vessels

72
Q

WEGENER’S GRANULOMATOSIS TX

A

stop smoking, glucocorticoids high dose

73
Q

Eosinophilic Granulomatosis with Polyangitis (EGPA) other name and TX

A

Churg- Strauss Syndrome

= no smoking, glucocorticoids

74
Q

Churg- Strauss Syndrome is what

A

resp tract

= asthma + eosinophilia —-> vasculitis with granulomas

75
Q

Churg- Strauss Syndrome 3 phases

A
  1. prodromal : arthma, allergy, months to years
  2. eosinophilia - tissue infiltration : high E in blood, go to lungs and GI and others
  3. Vasculitis : systemic necrotizing heart, lungs, nerves, skin + Palpable purpura
76
Q

Churg- Strauss Syndrome DX

A
  1. high E count
  2. ANCA+ usually (MPO-ANCA+ = pANCA+)
  3. bx lung : granulomas and vascular changes, E in tissue
77
Q

Churg- Strauss Syndrome vessels

A

small and medium

78
Q

Behcet Syndrome prevalence and DX

A
  1. Turkey, Asia, Mid East

2. HLA -B51

79
Q

Behcet Syndrome what is it and triad

A 
=Large vessel aneurysms
=DVT
1. recurrent mouth ulcers
2. genital ulcers 
3. eye inflammation uveitis
= can look like MS (neuro) or Chrons (GI)
= pustules at site of needle injection
80
Q

Behcet Syndrome tx

A

low dose glucocorticoids

81
Q

Thromboangiitis Oblitirans other name and prevalence

A

BUERGER Disease

= MALES, under 45yo

82
Q

BUERGER Disease what happens

A

only in people who smoke

= loss of digits

83
Q

BUERGER Disease DX and Tx

A
  1. corkscrew appearence on agiography , recent tobacco use, under 45yo
  2. Stop smoking
84
Q

Polyarteritis nodosa prevalence and vessel type

A

medium BV

= HBV, MALE

85
Q

Polyarteritis nodosa SX

A
  1. fever, livedo reticularis, ulcers, digital gangrene
  2. 80% = vasculitis neuropathy (foot drop)
  3. renin made HTN
  4. caridiac problem can cause HTN
  5. NO LUNG PROBLEMS
86
Q

Polyarteritis nodosa DX

A
  1. bx: fibrinoid necrosis , no granulomas
  2. angiogram : micro-aneurysm
  3. HBV
87
Q

Polyarteritis nodosa tx

A

glucocorticoids, tx HBV

88
Q

Kawasaki Disease other name and prevalence

A

MUCOCUTANEOUS LN SYNDROME

- MALE, under 5yo, JAPAN

89
Q

MUCOCUTANEOUS LN SYNDROME tetrad

A
  1. fever
  2. lymphadenopathy
  3. rash
  4. strawberry tongue
    = red eyes, pealing skin around nails
90
Q

MUCOCUTANEOUS LN SYNDROME DX

A

clinical features

91
Q

MUCOCUTANEOUS LN SYNDROME risk

A

coronary artery aneurysm of MI

92
Q

MUCOCUTANEOUS LN SYNDROME TX

A

IVIG within 10days

high dose ASA (Asprin)

93
Q

Takayasu Arteritis vessel typer and prevalence

A

= large

= under 40yo, F, AORTA, asia

94
Q

Takayasu Arteritis what happens

A

AORTA or branches involved
= limb pulseless (only collaterals made to prevent ischemia)
= Renal artery stenosis, HTN
= Retinopahty : copper wire infarctions)
= Aortic dilation, regurg, aneurysm, rupture
= lung probs

95
Q

Takayasu Arteritis DX

A
  1. MRI, CT angiogram = long smooth tapered stenosis (atherosclerosis looks irregular lesions)
  2. BX : granuloma with giant cells
96
Q

Takayasu Arteritis tx

A

glucocorticoids

97
Q

Giant cell arteritis (GCA) associated with

A

Polymyalgia Rheumatica (PMR)

98
Q

Giant cell arteritis (GCA) other name , vessel type

A 
Temporal Arteritis (TA)
large BV
99
Q

Giant cell arteritis (GCA) what happens

A

cranial arteries involved (Temporal A)

  1. dull headache, tender temporal A upon palpation
  2. jaw pain while chewing
  3. PMR association
  4. amaurosis fugax or diplopia : vision problems
100
Q

Giant cell arteritis (GCA) DX

A
  1. HIGH ESR (over 50mm/h)
  2. HLA- DR4
  3. Temporal A BX **: segmented granulomatous vasculitis + multinucleated giant cells **
101
Q

Giant cell arteritis (GCA) TX

A

TREAT GLUCOCORTICOIDS BEFORE anything else including bx

they can go blind if not treated

102
Q

Polymyalgia Rheumatica (PMR) is what

A
  1. stiffness, soreness and muscle pain = causing untrue muscle weakness sx (only muscles are normal in strength and everything, just from pain)
  2. pain putting coat on, combing hair, shoulder, neck, pelvis
103
Q 
Polymyalgia Rheumatica (PMR) 
DX
A
  1. high ESR and CRP
  2. no inflammation in muscles,
  3. normal EMG (elecromyography)
104
Q 
Polymyalgia Rheumatica (PMR) 
 tx
A

glucocorticoids

105
Q

Raynauds Phenomenon what happens

A

episodic from cold / stress = primary , from other disease cause = secondary + more ischemia

  1. pallor (vasoconstriction) white
  2. Cyanosis (ischemia) blue
  3. erythema (reperfusion) red
106
Q

Raynauds Phenomenon age

A

primary 15-30

secondary over 30

107
Q

Raynauds Phenomenon dx

A

Naifold capillaroscopy : normal in primary

= distorted + widened and irregular loops , dilated lumen and vascular “dropout” areas in secondary

108
Q

Raynauds Phenomenon tx

A

wear gloves, stay warm, lotion, dont smoke, limit sympathetic drugs
CCB
secondary : tx underlying cause

CPR2 Exam 1 (19 decks)

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