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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
How do you diagnose CREST? (LCSSc)
* Dx made in advanced cases * **+ anti centromere ab** * +ANA * PE, XR, UA, BX
26
How do you treat LCSSc?
* 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)**
27
Hx and PE of Diffuse Scleroderma?
* Interstitial lung disease * Renal crisis * skin induration and hyper/hypo pigmentation leading to loss of body hair and impaired sweating * Fibrotic joints
28
When comparing CREST(LCSSc), Localized scleroderma (SSc) , and diffuse scleroderma, which syndrome has Raynauds occuring early and which one has internal organ involvent?
* 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
Your patient has ___ and you hear dry course velcro like crackles on lung auscultation.
Your patient has **_Diffuse Scleroderma_** and you hear dry course velcro like crackles on lung auscultation.
30
How do you diagnose DCSSc?
* **Anti Scl 70 aka Anti Topoisomerase I** * **Anti RNA pol III** * ANA * CXR or CT of chest * PFTs
31
What is the primary cause of morbidity and mortality in systemic sclerosis?
* Pulmonary involvement * PAH and ILD
32
Describe the effects of systemic sclerosis on the renal system.
* CKD * proteinuria * elevation creatine * Htn * Renal crisis is abrupt onset of: * malignant hypertension * hemolytic anemia * Progressive renal insufficiency * *more common in diffuse SSc*
33
Describe Pe findings for a patient with Sjogren syndrome
* 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
How do you diagnose Sjogren?
* Polyclonal hypergammaglobulinemia * **+ anti SSA/Ro** * **+ Anti SSB/La** * **Labial salivary gland lip biopsy**
35
How do patients with Dermatomyositis present\>? (DM)
* Weakness _without sensory sxs_ * Ages 7-15 and 30-60 * _Skin lesions_ * _gottrons patches_ * _Helitrope lash_ * V neck erythema * Poikiloderma “Shawl Sign”
36
What should you look for when you diagnose a patient with Dermatomyositis?
**Increased risk of occult malignancy**
37
How do you diagnose DM?
* Elevated CK and adolase * Serology Anti Jo-1 * Bx shows perimysial and perivascular inflammation perifascicular atrophy
38
How do you treat Dm?
Glucocorticoids
39
How does Polymyositis present?
* NO skin changes as sseen in DM * Subacute proximal muscle weakness over weeks to months (same as DM) * 30-50+ yo
40
Labs for PM?
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
Inclusion body myositis presnetation and treatment?
* Males\>Females * Weakness in finger flexion or quads * Refractory to tx so its supportive
42
How do you diagnose IBM?
* Mild elevation or normal CK * anti cN1A autoabs * Bx shows Endomysial inflammation **rimmed vacuoles** invasion of non necrotic muscle fibers
43
What are the vasculitides that impact small vessels?
* IgAV aka Henoch Schonlein Purpura (HSP) * Anti-GMP (Goodpasture) * Granulomatosis with Polyangiitis (GPA)
44
What are the vasculitides that impact medium vessels
* Thromboangiitis Obliterans (Beurger Disease) * Kawasaki * Polyarteritis Nodosa (PN)
45
What are the vasculitides that impact Large vessels
* Takayasu Arteritis * Giant cell arteritis (temporal arteritis)
46
Who does HSP impact and what is the tetrad?
* Kids * **Palpable purpura** without thrombocytopenia * Arthritis * Ab pain * Renal disease
47
IGA vasculitis (HSP) biopsy results and tx?
* Bx shows IgA deposits * Tx is supportive and glucocorticoids
48
Goodpasture syndrome presentation dx and treatment?
* Hematuria * Hemoptysis * Diagnose with renal bx showing deposition of anti basement membrane autoantibiodies * Tx with plasmapheresis and glucocorticoids
49
Hallmarks of Granulomatosis with polyangiitis (aka Wegener's granulomatosis)
* **Granulomatous inflammation** * Necrotizing vasculitis * Venous thrombolytic events * Segmental glomerulonephritis * URI/LRI * **\*90% cases have nasal involvement** * Saddle nose * Lungs show **cavitary lesions**
50
How do you diagnose and treat GPA?
* + ANCA * Bx shows vessel changes with granulomas * NO smoking and give high dose glucocorticoids
51
Who does GPA effect more?
Males
52
What are the Hx/PE findings for Eosionophilic granulomatosis with polyangiitis (EGPA) aka Churg Strauss syndrome?
* Respiratory tract * Asthma + Eosinophilia → vasculitis with granulomas
53
What are the phases to EGPA?
* Prodromal phase: * allergic disease * Eosinophilia tissue infiltration phase: * High eosinophils in circulating blood * Vasculitis phase: * Systemic necrotizing and palpable purpura
54
Bechet syndrome?
* Large vessel aneurysms * DVT * Triad of small vessels: * Recurrent mouth ulcers * Genital ulcers * Eye inflammation
55
What vasculitis do you see pathergy in? (pustules at site of sterile needle prick)
Bechet syndrome
56
How do you diagnose Bechet and treat?
* HLA B51 * Low dose glucocorticoid
57
Thromboangiitis Obliterans (Buerger disease) Hx/PE diagnostics and treatment?
* Young men \<45 * ONLY occurs in those who smoke * Loss of digits * Angiography shows corckscrew appearance * Tx is to stop smoking
58
Polyarteritis nodosa Hx and PE?
* Assoc with HBV * Livedo reticularis, ulcers, digital gangrene * 80% have vasculitis neuropathy and foot drop * Newly acquired HTN * **lungs spared**
59
How do you diagnose and treat polyarteritis nodosa?
* 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
Kawasaki Disease Hx and PE? (mucocutaneous lymph node syndrome)
* Worldwide but highest rate in Japan, \<5 yo (M\>F) * Fever lymphadenopathy rash strawberry tongue
61
What accounts for morbidity in Kawasaki?
Coronary aneurysm or MI
62
Tx for Kawasaki?
* IVIG w/n 10 days sx * High dose ASA
63
Takayasu Arteritis hallmarks?
* 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
Overlapping features between GCA and PMR?
* Frequently co exist * F\>M * \>40-50 * Constitutioinal sx * Elevated ESR and CRP * Tx with glucocorticoids
65
Giant cell arteritis (aka Temporal arteritis) Hx and PE.
* Cranial arteries tender * Dull headache * Jaw claudication (painful chewing) * **_Associated with Polymyalgia Rheumatica_** * Amaurosis fugax or diplopia
66
How do you diagnose and treat GCA?
* 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
Hx and PE findings with Polymyalgia Rheumatica (PMR)?
* Stiffness, soreness and muslce pain * Starts in morning and throughout the day * Feels weak subjectively but not actually, it is due to pain
68
Diagnosis and treatment of PMR?
* Everything normal except ESR and CRP elevated * no inflammation on muscle bx and muscle enzyme labs are normal * EMG normal * Tx with glucocorticoids
69
Differentiate primary from secondary Raynaud's phenomenon.
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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