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SBL Test 2 > McGowan Extra > Flashcards

McGowan Extra Flashcards

1
Q

SLE Serology

A
    • ANA
  2. anti-dsDNA
  3. anti-Sm (Smith)
  4. Decreased C3/C4
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2
Q

c-ANCA =

A

PR3-ANCA

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3
Q

p-ANCA =

A

MPO-ANCA

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4
Q

Who is SLE most common in?

A
  1. F AA and Hispanics
  2. Low SES
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5
Q

Libman Sacks Endocarditis (what is it?) is often seen in _____

A
  1. Non-infectious vegetative thrombi that can embolize
  2. SLE
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6
Q

What should you give a pregnant F in need of anticoagulation therapy and why?

A

LMW Heparin, because Warfarin/Coumadin is CI.

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7
Q

Drug-Induced Lupus-Like Syndrome

  1. Serology
  2. How is it different from lupus?
A
  1. No renal or neuro symptoms
  2. + ANA
  3. + Anti-histone Ab (95%)
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8
Q

What drugs can cause SLE flares?

A

Sulfa drugs

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9
Q

Neonatal lupus affects children born to mothers with what ab?

A

1. Anti-Ro (SSA)

2. Anti-La (SSB)

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10
Q

Treatment of SLE

A
  • 1. Avoid sun and wear sunscreen
  • 2. NSAIDS
  • 3. Corticosteroids
  • 4. Hydroxycholoroquine
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11
Q

Immediate Type 1 Hypersensitivity Reactions

A

A = allergEE

  1. Allergens/ allergies produce IgE Abs => release of vasoactive mediators from mast cells
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12
Q

Type 2 HS Reactions

A

I = Ig/Ab-mediated

IgG/IgM Ab bind to target cell/tissue => phagocytosis or lysis by (+) compliment or (+) Fc receptors

  1. AIHA
  2. Goodpasture
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13
Q

Type 3 HS Reaction

A

Immune-complex mediated

  1. Deposition of complexes => (+) compliment

SLE

Serum sickness

Arthus reaction

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14
Q

Type 4 HS REaction

A

Delayed; T-cell mediated => release of cytokines => T cell mediated cytotoxicity

1. Contact dermatitis

2. MS

3. T1DB

4. TB

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15
Q

Scleroderma MC affects who?

A

30-60YO W

More severe in AA

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16
Q

Diffuse Scleroderma (dcSSc)

  1. Phases
  2. Involvement
  3. Organ involved?
  4. Prognosis
A
  1. 1st has a inflammatory edematous phase => fibrotic (stiffness)
  2. Systemic, diffuse involvement: early and progressive involvment of internal organs
    1. Lungs: Interstitial lung disease
    2. Kidney: Renal crisis (hemolytic anemia + malignant HTN, progressive renal insufficiency)
    3. Cardiac problems (50%)
    4. GI: malnutrition (fat/protein/B12/D deficiency), Barrets ESO, GAVE (gastric antral vascular ectasias = watermelon stomach)
    5. MSK: carpal tunnel
    6. Thyroid: Hypothyroidism d/t fibrosis,
  3. Worse prognosis; high dose corticosteroids can cause renal crisis
17
Q

Limited Scleroderma (dcSSc)

Involvement

Organ involved?

Prognosis

A
  1. CREST Syndrome: Calcinosis = calcium deposits in skin; 2’ raynauds; Esophageal dysmotility (GERD), scerodactyly; telangiectasias
    1. Vascular manifestations are more pronounded
      1. Digital ischemia
      2. Pulmonary HTN
    2. Renal crisis = NOT common
  2. Fingers + toes + face + neck + distal extrememties
  3. Better prognosis
18
Q

What is the primary cause of mortality and morbidity in scleroderma?

Tests?

A

Pulmonary issues

  • Diffuse = Interstitial lung disease; Pulmonary function tests (PFT)
  • Limited = Pulmonary HTN (>40mmHg on echo and a right heart catheter to confirm)
19
Q

Scleroderma has increases incidence of what cancers?

A
  1. Bronchoalveolar carcinoma (d/t pulmonary problems)
  2. Esopheageal adenocarcinoma (d/t Barretts ESO)
20
Q

Diffuse Cutaneous Scleroderma Serology

A

1. Anti-scl 70/anti-topoisomerase I

2. Anti-RNA polymerase III

21
Q

Limited Cutaneous Scleroderma Serology

22
Q

Sjrogen Serology

A
  • 1. ANA
  • 2. RF
  • 3. High ESR
  • 4. Hypergammaglobinemia
  • 5. Anti-Ro (SSA)/Anti-La (SSB)
23
Q

What is the treament for Sjrogens?

What should be avoided and what does NOT work?

A
  1. Symptomatic management (also scleroderma)
  2. Avoid atropinic drugs and decongestants
  3. Immunomodulatory drugs DON’T work.
24
Q

Inflammatory Myopathies include what diseases

A
  • 1. Dermatomyositis
  • 2. Polymyositis
  • 3. Inclusion body myositis
25
**_Common features of inflammatory myopathies_** 1. MC affects = 2. Symptoms = 3. Labs
1. **AA F** 2. **Bilateral proximal muscle weakness**: diff standing, rising from chair/bathtub, climbing stairs 3. Labs 1. **↑ CK** and **adolase** (except inclusion body myositis = NL/midly ↑ CK) 2. **NL ESR/CRP**
26
What inflammatory myopathy is **Poikiloderma** a finding?
Dermatomyositis = shawl sign
27
**Dermatomyositis** Serology
* **1. Anti Jo1** * **2. Anti Mi2** * **3. Anti MDA5** * **4. Anti P155/140**
28
When **Dermatomyositis** is diagnosed, start looking for occult malignancies. What is the MC cancer and what work-up should be conducted?
* **Ovarian cancer** * **Transvaginal US** + **CT abd/pelvis** + **CA-125.**
29
**Polymyositis** serology/labs
1. **Elevated CK** 2. **Anti-Jo1**
30
**Inclusion Body Myositis** serology and labs How does treatment differ from other 2 myopathies?
1. NL/mildly elevated CK 2. **Anti-cN1A ab** 3. **Supporative treatment;** do NOT give corticosteroids
31
**Takayasu Arteritis** 1. What is it/size of vessels affected? 2. Symptoms/signs by organ affected 3. Mc in? 4. Course? 5. Dx? 6. Tx?
* **Large vessels of aorta** (subclavian and innominate are MC) =\> long smooth tapered stenosis * CV: AAAA (aortic dilation, regurg, rupture + aneurism). * Pulseless disease (no UE pulses, but collaterals prevent limb loss) * Kidney: If Renal A is involved (dx on doppler renal US) =\> HTN =\> retinopathy (copper wiring infarctions) * Lungs: 50% of pulmonary involvement * **MC** = W under 40 ; MC in Asia * **Course** = chronic: relapsing and remitting * Dx = MRI or CT angio; histology (granuloma + giant cells, with fibrosis in chornic stages) * Tx = steroids
32
**Behcet Syndrome** 1. What is it/size of vessels affected? 2. Symptoms/signs by organ affected 3. Sign? 4. Dx? 5. Tx?
1. **Variable sizes** 1. Large vessels = aneurisms 2. Veins = DVTs 2. **Triad** 1. Recurrent mouth ulcers 2. Genital ulcers 3. Uveitis: eye inflammation 4. OTHER: ulcers in distal ileum/cecum (DDx = Crohns) 3. **Pathergy** = pustules form at sites of needle pricks 4. **D**x = Hx and PE; Serology = HLA-B51 5. **Tx**= low dose steroids
33
**Polyarteritis Nodosa (PAN)** ## Footnote What is it/size of vessels affected? Symptoms/signs by organ affected MC in? Dx? Tx?
1. Inflammation of medium vessels + segmental, due to HepB. 2. Symptoms 1. Skin: Livedo reticularis/subQ nodules 2. Nerves: Neuropathy (foot drops) 3. Cardiac: newly acquired HTN d/t renal HTN 4. LUNGS SPARED!!!!! 3. MC in **MEN**! 4. Dx = Hx and PE =\> confirm with angiogram or biopsy = 1. fibrinoid necrosis and NO granumoas 2. Angiogram = microaneurism 3. Serology: ANCA (+/-, MC -); look for HepBsAg/Ab, HbeAg) 5. Tx = corticosteroids
34
**GPA (Wegners Granulomatosis)** 1. Mc in who? 2. Positive for what marker? 3. Affects mainly what organs 4. What is seen on CXR
1. M \> 40YO 2. **c-ANCA (PR3-ANCA)** 3. Lungs and kidney (resp tract = involved 90% of time - saddle nose) 4. CXR = cavitary lesiosn

SBL Test 2 (11 decks)

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