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MSK II Exam II > Connective Tissues CIS (McGowan) > Flashcards

Connective Tissues CIS (McGowan) Flashcards

1
Q

what does a + ANA test tell you?

A
  • loosely associated with underlying autoimmune disease
  • titer of <1:40 is normal/ negative
  • titer of 1:160 or above means there is probably an underlying autoimmune disease
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2
Q

what are the clinical aspects of localized scleroderma?

A
  • benign skin conditions
  • affect children
  • discreet areas of discolored skin induration
  • NO Raynauds
  • NOT systemic
  • Histologically indistinguishable from SSc
  • Patches= Morphea
  • Coalesced patches= generalized morephea
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3
Q

what are the clinical aspects of Sjogren’s syndrome?

A
  • F>M
  • Mid 50’s/ postmenopausal
  • Sicca sx (immune mediated dysfunction of lacrimal and salivary glands):
    • ​dry eyes, dry mouth (xerostomia), vagina dryness, tracheo-bronchial dryness
    • increased incidence of oral infection (candida)
    • dental caries
    • parotid or other major salivary gland enlargement
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4
Q

What is the term used to describe the foreign body sensation in the eye due to inadequate tear production in SJogren’s syndrome?

A

Keratoconjunctivitis sicca

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5
Q

Which disease is Sjogren’s syndrome most strongly associated with?

A

B cell non-hodgkin lymphoma

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6
Q

Which disease is Libman-Sacks endocarditis (a type of non-infective endocardidtis) associated with?

A
  • SLE
  • complication can be stroke
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7
Q

which autoimmune disease increases risk for esophageal adenocarcinoma?

A

Diffuse scleroderma

Diffuse scleroderma–> GERD–> Barretts esophagus–>esophageal adenocarcinoma

treat with omeprazole

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8
Q

Diffuse sceleroderma can increase risk of getting esophageal adenocarcinoma and ____

A

Gastric Antral Vascular Ectasia (GAVE)

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9
Q

which autoimmune disease increases risk for pulmonary artery hypertension? is also a cause of mortality in these patients

A

limited scleroderma

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10
Q

which autoimmune disease increases risk for interstitial lung disease? is a cause of mortality in thses patients

A

diffuse scleroderma

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11
Q

what are the clinical sx of polymyalgia rheumatica? (PMR)

A
  • W>M
  • >40-50
  • white>
  • frequently coexists with giant cell arteritis
  • constitutional sx:
    • fever, malaise, weight loss
    • normal WBC count
  • proximal severe, symmetrical morning and daylong stiffness, soreness, and pain in shoulder, neck, and pelvic girdles
  • associated with GCA
  • elevated ESR
  • muscle enzymes and EMG normal
  • no inflammation on muscle bx
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12
Q

which condition only occurs in smokers?

A

Thromboangiitis Obliterans AKA Buerger Disease

treatment= stop smoking

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13
Q

What is Primary Raynauds?

A
  • physiologic hyperresponse to:
    • emotions
    • cold temperatures
  • no underlying pathology
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14
Q

How is Secondary Raynauds different from Primary Raynauds?

A
  • Secondary Raynauds occurs as part of an underlying condition.
  • Primary Raynauds has no underlying pathology associated with it.
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15
Q

what condition is associated with Hep B infection?

A

Polyarteritis Nodosa

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16
Q

Name some conditions that increase your risk for DVT

A
  • Factor V Leiden
  • Antiphospholipid antibody syndrome
  • SLE
  • Becet’s
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17
Q

What are the clinical sx of Becet’s disease?

A
  • Oral ulcers
  • genital ulcers
  • uveitis
  • DVT
  • arterial clotting
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18
Q

Which drugs are associated with drug-induced lupus?

A
  • hydralazine
  • isoniazid
  • minocycline
  • TNF inhibitors
  • Quinidine
  • Chlorpromazine
  • Methyldopa
  • Procainamide
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19
Q

ds-DNA Ab is mostly seen in ___

A

SLE

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20
Q

Sm Ab is mostly seen in

A

SLE

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21
Q

Histone Ab are seen in

A

Drug-induced SLE

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22
Q

centromere Ab are seen in ___

A

limited scleroderma (CREST)

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23
Q

Scl 70 Ab (Topoisomerase I Ab) is seen in

A

diffuse scleroderma

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24
Q

clinical signs of SLE

A
  • multisystem
  • unknown eitiology
    • inflammatory autoimmune disorder
    • T and B cell hyperactivity
    • Autoantibodies to nuclear antigens
      • Immune complexes (type III hypersensitivity)
  • spontaneous remissions and relapses
  • F>M
  • african americans>
  • those without insurance
  • UV light
  • EBV
  • Cotton wool spots
  • malar rash (butterfly rash)
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25
Q

What is a VDRL and in what autoimmune disorders is it positive in?

A
  • VDRL is a test for syphillis
  • biologic false + VDRL tests seen in SLE and Antiphospholipid antibody syndrome
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26
Q

what is the best management/treatment plan for SLE?

A
  • hydroxychloroquine
    • is one of the mainstay first treatments for SLE
  • anti-coagulant to treat DVT
    • start with LMW heparin/unfractionated heparin first then
    • bridge to warfarin/coumadin
      • starting warfarin on its own is PROthrombotic
  • Can ALSO:
    • avoid sun exposure
    • use NSAIDS
    • use corticosteroids
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27
Q

Calcium channel blockers, such as Amlodipine, are used in the treatment of ___

A

Raynaud’s

28
Q

IVIG and high dose ASA is used to treat _____

A

Kawasaki Disease

29
Q

post prandial intermittent abdominal pain

A

intestinal angina (associated with SLE)

not a cause of mortality

30
Q

seizure disorder is associated with which autoimmune disease?

A

SLE

31
Q

Mortality in SLE patients is due to:

A
  • early years
    • infections
  • later years
    • accelerated atherosclerosis
    • thomboembolic events
32
Q

what are some common cardiac manifestations of SLE?

A
  • libman sacks endocarditis
  • pleuritis
  • pericarditis
    • pain worse with positional changes
    • diffuse elevated ST wave changes on EKG
  • myocarditis
33
Q

Which things are positive on SLE Serology?

A
  • ANA
    • non-specific for autoimmune disease
  • anti-ds DNA
    • correlates with disease activity
  • Sm (Smith)
    • does NOT correlate with disease activity
  • decreased C3 and C4
    • ​increased comsupmtion of compliment suggests disease activity
    • returns normal when in remission
34
Q

Elevated Creatine Kinase (CK) is diagnostic in which inflammatory myopathies?

A
  • dermatomyositis
  • polymyositis
35
Q

Deposits of ___ are diagnostic in Heoch-Schonlein Purpura

A

IgA

36
Q

List preventative measures to be taken for a patient with SLE

A
  • avoid smoking
  • minimize risk factors for atherosclerosis
  • influenza vaccination
  • pneumococcal vaccination
  • preventative cancer screening
  • check for avascular necrosis of the bone
37
Q

what are the clinical features of Kawasaki disease?

A
  • medium vessel
  • <5 yo, Asian
  • Mucocutaneous lumph node syndrome
  • Strawberry tongue
  • DDx: toxic shock syndrome and Scarlet Fever
  • death from coronary involvement (aneurysm or MI- can occur years later)
  • Tx: IVIG w/in 10 days of sxs and high dose ASA
    • yes ASA in a ped patient
38
Q

what are the clinical sx of dermatomyositis?

A
  • bimodal age: 7-15 yo and 30-60 yo
  • weakness w/o sensory sx
    • proximal muscles early–> distal muscles late in disease course
  • characteristic skin lesions of DM
    • heliotrope rash
      • ​periorbital edema, purplish suffusion over eyelids
    • “Shawl sign”
      • erythema over neck/shoulders, upper chest and back
  • increased risk of occult malignancy​ (correlates to age and risk factors for each individual patient)
  • Dx: biopsy-perimysial and perivascular inflammation
    • ​perifascicular atrophy
    • elevated creatine kinase and aldolase
    • anti Jo-1
    • anti Mi2
    • anti-MDA5
    • anti-P155/P140
39
Q

which connective tissue diseases include proximal muscle weakness?

A
  • dermatomyositis
  • polymyositis
  • polymyalgia rheumatica
40
Q

What does CREST stand for and what is another name for it?

A
  • C=calcinosis cutis (white nodules on fingwers)
  • R=Raynauds (secondary)
    • susceptible to digital ischemia (fingers turn blue)
    • usually first sx
  • E=esophageal dysmotility
  • S=sclerodactyly
  • T=telangiectasias
  • also called “limited scleroderma”
41
Q

what type of hypersensitivity reaction is SLE?

A

Type III

42
Q

Patients are at risk for MALT lymphoma with which disease?

A

Sjogren’s syndrome

43
Q

how do you diagnose Sjogren’s syndrome?

A

lip biopsy

44
Q

how do you diagnose temporal arteritis/giant cell arteritis?

A

temporal artery biopsy

45
Q

which disease is associated with temporal arteritis/giant cell arteritis?

A

polymyalgia rheumatica

46
Q

most likely cause of shortness of air in patients with diffuse scleroderma?

A
  • interstitial lung disease
    • restrictive PFT
    • mediastinal LAD and honeycombing on CXR
    • dry crackels
47
Q

what pulmonary artery pressure is indicative of pulmonary HTN?

A

>40mmHg

48
Q

CHF can be seen in _____

A

Polyarteritis Nodosa

49
Q

What are the clinical aspects of inclusion body myositis?

A
  • >40-50 yo
  • M>F
  • caucasian more common
  • finger flexion pr quadriceps weakness
  • Ck is mild elevation or normal
  • muscle biopsy:
    • ​endomysial inflammation
    • rimmed vacuoles
    • invasion of non-necrotic muscle fibers
    • anti-cN1A autoantibodies
50
Q

what is the treatment for inclusion body myositis?

A

refractory to treatment, so treatment is supportive (physical therapy)

51
Q

what are the clinical sx of Takyasu arteritis?

A
  • asians (indian in CIS)
  • affects large vessels (aorta and branches)
    • subclavian and innominate most common
  • Age <40, F>M
  • chronic relapsing and remitting course
  • long smooth tapered stenosis
  • “pulseless disease”
    • ​obliterate UE peripheral pulses
  • 50%pulmonary involvement
  • retinopathy, renal artery steonsis, aortic dilations, aortic regurgitation, aneurysm, aortic rupture
52
Q

how do you diagnose takyasu arteritis?

A
  • MRI or CT angiography
  • copper wiring on fundoscopy
53
Q

what histology is diagnostic of takyasu arteritis?

A

granuloma with some giant cells

54
Q

what is the treatment for takayasu arteritis?

A

glucocorticoids

55
Q

what are the clinical aspects of Behcet syndrome?

A
  • silk route (Turkey, Asia, mid East)
  • HLA-B51
  • Triad:
    • _​_recurrent mouth ulcers
    • genital ulcers
    • eye inflammation (uveitis)
  • large vessel anerysms
  • venous involvement–> DVT
  • pathergy= pustules at site of sterile needle pricks
56
Q

What are the clinical findings in diffuse scleroderma?

A
  • thickening and hardening of the skin
  • fibrosis of skin and visceral organs
  • systemic
  • diffuse involvement
  • including proximal extremities and trunk
  • early and progressive internal organ involvement
    • especially kidney, cardiac, and interstitial lung disease
  • worst prognosis
  • serology
    • ​+ Anti Sc1 70
      • ​aka anti topoisomeras 1
    • anti-RNA polymerase III
57
Q

positive serology for scleroderma

A

*

58
Q

________is an abrupt onset of malignant hypertention, hemolytic anemia, and progressive renal insufficiency and is most common in _____

A

renal crisis; diffuse scleroderma

M>F

59
Q

what is polyarteritis nodosa?

A
  • medium vessel vasculitis
  • associated with Hepatits B
  • M>F
  • constitutional: fever, malaise, weight loss
  • lungs are spared
    *
60
Q

how do you diagnose polyarteritis nodosa?

A
  • biopsy-infiltration and destruction of blood vessels by inflammatory cells–> fibrinoid necrosis, NO granulomas
  • angiogram-micro-aneurysm
  • ANCA negative (check HBsAg and HBeAg)
61
Q

patient presents with headache that will not go away with tylenol and her temporal arteries are tender to palpation (temporal/giant cell arteritis). What is the appropriate next step?

A
  • START CORTICOSTEROIDS BEFORE GETTING BIOPSY
  • no treatment puts them at risk for blindness
62
Q

what are the clinical manifestations of polymyositis?

A
  • 30-50 yo+
  • subacute (over weeks to months) proximal muscle weakness
  • like dermatomyositis except NO skin changes
  • elevated serum CK
  • anti-Jo 1
  • muscle biopsy
    • ​endomysial inflammation with invasion of non-necrotic muscle fibers without features suggestive of another dx (IBM or muscular dystropy)
63
Q

what are the clinical features of Eosinophillic Granulomatosis with Polyangiitis (Churg Strauss Syndrome)?

A
  • ANCA+ [<50% of patients]
    • MPO-ANCA
  • granulomas with eosinophilia
  • asthma+eosinophila–> vasculitis
64
Q

what organ systems are affected by polyarteritis nodosa?

A
  • cardiac
  • GI
  • skin
  • renal
  • vasculitis neuropathy (right foot drop)
65
Q

what is the most common cause of mortality in kawaskai disease?

A

coronary artery aneurysm

66
Q

what are the clinical manifestations of Granulomatosis With Polyangiitis: aka Wegners Granulomatosis?

A
  • C-ANCA/PR3-ANCA +
  • respiratory tract incolvement
    • ​upper and lower
    • saddle nose
  • cavitary lesions on CXR
  • Kidney involvement
  • granulomatous inflammation
  • necrotizing vasculitis
  • segmental glomerulonephritis

MSK II Exam II (4 decks)

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