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CPR 2 Exam 1 > McGowan Vasculitidies > Flashcards

McGowan Vasculitidies Flashcards

1
Q

Etiology of SLE?

A
  • Multisystem inflammatory AI disorder
  • Type III hypersensitivity
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2
Q

What can be seen on PE for a patient with SLE?

A
  • Constitutional sx such as fever, fatigue, malaise, weight loss
  • Cutaneous rashes and photosensivitity
  • Arthritis
  • Hematologic issues
  • Pericarditis
  • Libman-Sacks endocarditis
  • Risk MI
  • Nephritis
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3
Q

SLE diagnosis?

A
  • Serolgy:
    • dsDNA
      • does correlate with disease activity
    • Smith ab
      • doesnt correlate with disease activity
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4
Q

SLE treatement?

A
  • Avoid sun exposure
  • NSAIDs for pain (caution with renal dz)
  • Glucocorticosteroids (systemic or topical)
  • Hydroxychloroquine
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5
Q

Management and prevention of SLE?

A
  • Minimize risk factors for atherosclerosis
    • avoid or quit smoking
  • Flu & pneumococcal vaccine
  • Cancer screenings
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6
Q

What is the prognosis/COD in the early years after a diagnosis with SLE?

A
  • Infections especially in those treated with immunosuppressives
  • Active SLE COD is kidney or CNS disesase
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7
Q

What is the typical COD in later years after a diagnosis of SLE?

A
  • Accelerated atherosclerosis linked to chronic inflammation
  • MI is 5x more likely
  • Thromboembolic events
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8
Q

What are the three primary autoantibodies seen in Antiphospholipid Ab syndrome?

A
  • Anti-Cardiolipid antibodies aCL
  • Lupus anticoagulant
  • Beta 2 glycoprotein I
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9
Q

A 27 year old female patient comes in to the ED. She is having a miscarriage, this is her 3rd one over the past 3 years. Her PMH is insignificant except for a DVT after a 15 hour plane ride. She takes no medications. What are you suspecting?

A

Antiphospholipid Ab Syndrome

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10
Q

What Ab causes false positives for syphilis?

A

aCL, seen in APS

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11
Q

What Ab prolongs PTT and is not corrected by adding normal platelet free plasma, and what is it seen in?

A

Lupus Anticoagulant (LA) seen in APS

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12
Q

How do you treat APS?

A

Indefinite systemic anticoagulation

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13
Q

What two disorders can you see cotton wool spots in?

A
  • SLE
  • APS
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14
Q

How do you diagnose Drug induced lupus (lupus like syndrome)?

A
  • +ANA
  • + Anti histone abs
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15
Q

Hx/PE of Dil?

A
  • No renal or neuro sx but looks similar to SLE
  • Medications such as:
    • Procainamide
    • Hydralazine
    • Isoniazid
    • Methyldopa
    • Lithium
    • Phenytoin
    • Nitrofurantoin
    • Sulfasalazine
    • HCTZ
    • Simvastatin

*List of meds is purple

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16
Q

Neonatal lupus affects children born of mothers with ___.

A

Neonatal lupus affects children born of mothers with Anti Ro (SSA) abs.

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17
Q

How will a baby with neonatal lupus present?

A
  • Congenital heart block
  • Transient rashes, thrombocytopenia, hemolytic anemia, arthritis
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18
Q

How do you diagnose neonatal lupus

A
  • + anti ro in mom
  • Serial echocardiograms and obstetric sonograms
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19
Q

Treatment and management of neontal lupus?

A
  • Delivery if fetus is in distress
  • Dexamethasone tx of mom with in utero heart block sometimes prevents progression to complete heart block
  • Good outcome if managed
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20
Q

Discoid lupus etiology and presentation?

A
  • Independent or manifestations of SLE most common on the head
  • Well defined inflammatory plaques that evolve into atrophic disfiguring scars
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21
Q

Diagnosis and treatment of Discoid lupus?

A
  • Biopsy
  • Photo protection and topical anti inflammatory agents or systemic antimalarial drugs
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22
Q

Hallmark of SSc? (scleroderma)

A
  • Thickening of the skin
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23
Q

What is Localized Scleroderma? How does it present and how do you diagnose and treat?

A
  • Not systemic but can involve joints or muscles in the area
  • Discrete patches of discolored skin induration, usually asx (Morphea)
  • NO raynauds
  • diagnose with Hx and biopsy (indistinguishable from other SSc)
  • PT, Steroids, phototherapy
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24
Q

How does Limited Scleroderma present? (CREST syndrome)

A
  • Calcinosis
  • Raynauds
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
  • Progressive Pulmonary artery HTN presenting as SOB
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25
Q

How do you diagnose CREST? (LCSSc)

A
  • Dx made in advanced cases
  • + anti centromere ab
  • +ANA
  • PE, XR, UA, BX
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26
Q

How do you treat LCSSc?

A
  • No therapy alters disease course
  • Education
  • Ca channel blockers for raynauds
  • ACE inhibitors for htn and limiting renal disease
  • PPI reffulx
  • Glucocorticoids (high doses can lead to development of renal crisis)
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27
Q

Hx and PE of Diffuse Scleroderma?

A
  • Interstitial lung disease
  • Renal crisis
  • skin induration and hyper/hypo pigmentation leading to loss of body hair and impaired sweating
  • Fibrotic joints
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28
Q

When comparing CREST(LCSSc), Localized scleroderma (SSc) , and diffuse scleroderma, which syndrome has Raynauds occuring early and which one has internal organ involvent?

A
  • Raynaud’s occurs early in CREST, later in diffuse and, not at all in Localized
  • SSc does not involve internal organs, LCSSc involves some, DCSSc involves internal organs
29
Q

Your patient has ___ and you hear dry course velcro like crackles on lung auscultation.

A

Your patient has Diffuse Scleroderma and you hear dry course velcro like crackles on lung auscultation.

30
Q

How do you diagnose DCSSc?

A
  • Anti Scl 70 aka Anti Topoisomerase I
  • Anti RNA pol III
  • ANA
  • CXR or CT of chest
  • PFTs
31
Q

What is the primary cause of morbidity and mortality in systemic sclerosis?

A
  • Pulmonary involvement
    • PAH and ILD
32
Q

Describe the effects of systemic sclerosis on the renal system.

A
  • CKD
    • proteinuria
    • elevation creatine
    • Htn
  • Renal crisis is abrupt onset of:
    • malignant hypertension
    • hemolytic anemia
    • Progressive renal insufficiency
    • more common in diffuse SSc
33
Q

Describe Pe findings for a patient with Sjogren syndrome

A
  • Sicca symptoms
    • dry eyes mouth vagina tracheo bronchial
  • Keratoconjunctivitis sicca: foreign body sensation due to inadequate tears
  • Parotid gland enlargement
  • associated with malt lymphoma
34
Q

How do you diagnose Sjogren?

A
  • Polyclonal hypergammaglobulinemia
  • + anti SSA/Ro
  • + Anti SSB/La
  • Labial salivary gland lip biopsy
35
Q

How do patients with Dermatomyositis present>? (DM)

A
  • Weakness without sensory sxs
  • Ages 7-15 and 30-60
  • Skin lesions
    • gottrons patches
    • Helitrope lash
    • V neck erythema
      • Poikiloderma “Shawl Sign”
36
Q

What should you look for when you diagnose a patient with Dermatomyositis?

A

Increased risk of occult malignancy

37
Q

How do you diagnose DM?

A
  • Elevated CK and adolase
  • Serology Anti Jo-1
  • Bx shows perimysial and perivascular inflammation perifascicular atrophy
38
Q

How do you treat Dm?

A

Glucocorticoids

39
Q

How does Polymyositis present?

A
  • NO skin changes as sseen in DM
  • Subacute proximal muscle weakness over weeks to months (same as DM)
  • 30-50+ yo
40
Q

Labs for PM?

A

Similar to DM (bx different)

  • Elevated CK and adolase
  • Anti Jo-1
  • Endomysial inflammation with invasion of non necrotic muscle fibers without features suggestive of another dx
41
Q

Inclusion body myositis presnetation and treatment?

A
  • Males>Females
  • Weakness in finger flexion or quads
  • Refractory to tx so its supportive
42
Q

How do you diagnose IBM?

A
  • Mild elevation or normal CK
  • anti cN1A autoabs
  • Bx shows Endomysial inflammation rimmed vacuoles invasion of non necrotic muscle fibers
43
Q

What are the vasculitides that impact small vessels?

A
  • IgAV aka Henoch Schonlein Purpura (HSP)
  • Anti-GMP (Goodpasture)
  • Granulomatosis with Polyangiitis (GPA)
44
Q

What are the vasculitides that impact medium vessels

A
  • Thromboangiitis Obliterans (Beurger Disease)
  • Kawasaki
  • Polyarteritis Nodosa (PN)
45
Q

What are the vasculitides that impact Large vessels

A
  • Takayasu Arteritis
  • Giant cell arteritis (temporal arteritis)
46
Q

Who does HSP impact and what is the tetrad?

A
  • Kids
  • Palpable purpura without thrombocytopenia
  • Arthritis
  • Ab pain
  • Renal disease
47
Q

IGA vasculitis (HSP) biopsy results and tx?

A
  • Bx shows IgA deposits
  • Tx is supportive and glucocorticoids
48
Q

Goodpasture syndrome presentation dx and treatment?

A
  • Hematuria
  • Hemoptysis
  • Diagnose with renal bx showing deposition of anti basement membrane autoantibiodies
  • Tx with plasmapheresis and glucocorticoids
49
Q

Hallmarks of Granulomatosis with polyangiitis (aka Wegener’s granulomatosis)

A
  • Granulomatous inflammation
  • Necrotizing vasculitis
  • Venous thrombolytic events
  • Segmental glomerulonephritis
  • URI/LRI
  • *90% cases have nasal involvement
  • Saddle nose
  • Lungs show cavitary lesions
50
Q

How do you diagnose and treat GPA?

A
    • ANCA
  • Bx shows vessel changes with granulomas
  • NO smoking and give high dose glucocorticoids
51
Q

Who does GPA effect more?

A

Males

52
Q

What are the Hx/PE findings for Eosionophilic granulomatosis with polyangiitis (EGPA) aka Churg Strauss syndrome?

A
  • Respiratory tract
  • Asthma + Eosinophilia → vasculitis with granulomas
53
Q

What are the phases to EGPA?

A
  • Prodromal phase:
    • allergic disease
  • Eosinophilia tissue infiltration phase:
    • High eosinophils in circulating blood
  • Vasculitis phase:
    • Systemic necrotizing and palpable purpura
54
Q

Bechet syndrome?

A
  • Large vessel aneurysms
  • DVT
  • Triad of small vessels:
    • Recurrent mouth ulcers
    • Genital ulcers
    • Eye inflammation
55
Q

What vasculitis do you see pathergy in? (pustules at site of sterile needle prick)

A

Bechet syndrome

56
Q

How do you diagnose Bechet and treat?

A
  • HLA B51
  • Low dose glucocorticoid
57
Q

Thromboangiitis Obliterans (Buerger disease) Hx/PE diagnostics and treatment?

A
  • Young men <45
  • ONLY occurs in those who smoke
  • Loss of digits
  • Angiography shows corckscrew appearance
  • Tx is to stop smoking
58
Q

Polyarteritis nodosa Hx and PE?

A
  • Assoc with HBV
  • Livedo reticularis, ulcers, digital gangrene
  • 80% have vasculitis neuropathy and foot drop
  • Newly acquired HTN
  • lungs spared
59
Q

How do you diagnose and treat polyarteritis nodosa?

A
  • Biopsy shows fibrinoid necrosis and NO granulomas
  • Angiogram shows micro aneurysm
  • Serology ANCA and check HBV serology
  • Tx with glucocorticoids
  • Tx HBV if present
60
Q

Kawasaki Disease Hx and PE? (mucocutaneous lymph node syndrome)

A
  • Worldwide but highest rate in Japan, <5 yo (M>F)
  • Fever lymphadenopathy rash strawberry tongue
61
Q

What accounts for morbidity in Kawasaki?

A

Coronary aneurysm or MI

62
Q

Tx for Kawasaki?

A
  • IVIG w/n 10 days sx
  • High dose ASA
63
Q

Takayasu Arteritis hallmarks?

A
  • Aorta is involved and can result in aneurysm, rupture, dilations and regurgitation
  • Pulseless disease (obliterates UE peripheral pulses)
  • 50% have pulmonary involvement
  • Copper wiring retinopathy
  • Renal artery stenosis
64
Q

Overlapping features between GCA and PMR?

A
  • Frequently co exist
  • F>M
  • >40-50
  • Constitutioinal sx
  • Elevated ESR and CRP
  • Tx with glucocorticoids
65
Q

Giant cell arteritis (aka Temporal arteritis) Hx and PE.

A
  • Cranial arteries tender
  • Dull headache
  • Jaw claudication (painful chewing)
  • Associated with Polymyalgia Rheumatica
  • Amaurosis fugax or diplopia
66
Q

How do you diagnose and treat GCA?

A
  • Very high ESRS
  • HLA DR4
  • Temporal artery bx is gold standard
    • see multinucleated giant cells
  • Start glucocorticoids immediately as this could lead to blindness
67
Q

Hx and PE findings with Polymyalgia Rheumatica (PMR)?

A
  • Stiffness, soreness and muslce pain
  • Starts in morning and throughout the day
  • Feels weak subjectively but not actually, it is due to pain
68
Q

Diagnosis and treatment of PMR?

A
  • Everything normal except ESR and CRP elevated
  • no inflammation on muscle bx and muscle enzyme labs are normal
  • EMG normal
  • Tx with glucocorticoids
69
Q

Differentiate primary from secondary Raynaud’s phenomenon.

A

Primary:

  • Benign
  • Symmetric
  • Exaggerated physioilogic response to cold or emotion
  • 15-30 yo F>M
  • Normal Nailfold capillaroscopy

Secondary:

  • >30 yo
  • Unilateral
  • Secondary to something
  • More severe ischemia
  • Nailfold capillaroscopy is distoreted with widened and irregular loops dilated lumen and areas of vascular dropout

CPR 2 Exam 1 (21 decks)

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