Autoimmune Rheumatologic Disorders Flashcards

1
Q

Demographics for typical SLE pt

A
  • Women of childbearing age

- African, Caribbean, Hispanic descent

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2
Q

When might you be suspicious of SLE in a pt?

A

Young woman presenting with multiple clinical problems but otherwise no significant PMH

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3
Q

Hallmark of SLE labs are _______?

A

Auto-antibodies to nuclear proteins

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4
Q

Describe the pathophysiology of SLE

A

Auto-antibodies to nuclear proteins damage tissues through immune activation and deposition of immune complexes

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5
Q

Besides auto-antibodies, what else is present in SLE?

A
  • C3/C4 deficiencies

- Increased B-lymphocyte stimulator (BlyS)

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6
Q

Clinical presentation of newly diagnosed SLE pt

A

Young pt with multiple problems:

  • Glomerulonephritis → edema, HTN, hematuria, foamy urine, oliguria
  • Hematologic disorders → anemia, thrombocytopenia
  • Arthritis
  • Mucucutaneous lesions → butterfly rash, livid reticularis
  • Raynaud phenomenon
  • Serositis → pleuritic pain, pericarditis
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7
Q

Clinical presentation of pt with established SLE

A
  • Hospital admission due to flare
  • Hematologic changes (d/t SLE or meds)
  • Neuropsychiatric dz
  • Premature CVD
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8
Q

What do you need to diagnose SLE?

A

Clinical presentation PLUS supporting labs

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9
Q

What labs should you get if you are suspicious for SLE?

A
  • Elevated ANA
  • CBC w/ diff → anemia, thrombocytopenia
  • UA → proteinuria, urinary sediment
  • Anti-dsDNA
  • C3/C4 complement deficiency
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10
Q

Dx lupus nephritis

A
  • Proteinuria → >0.5g/day or 3+
  • Urinary sediment → >5 RBC or WBC/HFP
  • Kidney bx
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11
Q

What should you do to treat SLE flare?

A
  • Don’t need to get ANA levels if established dx
  • R/o lupus nephritis via Cr, UA, urine sediment
  • Anti-dsDNA, ESR/CRP, C3/C4
  • Consult rheumatology
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12
Q

The mainstay of SLE treatment is _______

A

Glucocorticoids

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13
Q

Treatment of SLE includes

A
  • Glucocorticoids!
  • Hydroxychloroquine → reduces flares & improves survival
  • Belimumab → binds to and inhibits BlyS
  • IVIG if heart block
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14
Q

Indications for belimumab in SLE

A
  • Higher disease activity
  • Steroid use
  • Elevated anti-dsDNA
  • Low complement
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15
Q

Clinical manifestations of Sjogren’s syndrome

A

Xerophthalmia

Xerostomia

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16
Q

Pts with Sjogren’s also tend to develop ______

A

Malignant lymphoma

17
Q

Labs for Sjogren’s will show _______

A
  • Autoantibodies to Ro/SSA and La/SSB

- Antimuscarinic antibodies

18
Q

Why are xerophthalmia and xerostomia concerning?

A
  • High risk for corneal ulcer or perforation (consult ophthalmology)
  • Susceptible to dental caries
19
Q

What test can you use to help diagnose Sjogren’s, specifically xerophthalmia?

A

Schirmer’s test

20
Q

What can you do if you’re suspicious for Sjogren’s but all the tests are inconclusive?

A

Labial biopsy

21
Q

Tx Sjogren’s

A

Symptomatic relief

  • Artificial tears, drink more water
  • Propionic gels for vagina
  • Corneal ulcer → eye patch, boric acid ointment
  • Monoclonal antibody to CD20, add CHOP if lymphoma
  • Hydroxychloroquine for arthrlagias
  • Sodium bicarb for renal tubular acidosis
  • Glucocorticoids only indicated for systemic vasculitis
22
Q

What meds do Sjogren’s pts want to avoid?

A

Avoid diuretics, anti-HTN, anticholinergics, antidepressants

23
Q

What happens in scleroderma?

A

Fibrosis of skin & internal organs

24
Q

Name the 2 types of scleroderma.

Which is more common?

A
  • CREST syndrome (limited disease) → 80% pts

- Diffuse disease

25
Q

Presentation of limited scleroderma disease

A

CREST - limited to face, neck, distal extremities

  • Calcinosis cutis (calcification of skin)
  • Raynaud phenomenon
  • Esophageal dysmotility
  • Sclerodactyly (sausage fingers)
  • Telangiectasia
26
Q

Presentation of diffuse scleroderma dz

A
  • Widespread fibrosis in skin and internal organs
  • Shorter period b/w Raynaud’s & onset of other sx’s (weeks to months)
  • Polyarthralgia, weight loss, malaise
  • Early “edematous” phase w/ hyperpigmentation, carpal tunnel, muscle weakness, fatigue, decreased joint motility; Clinically silent organ involvement
27
Q

Most rapidly evolving pulmonary & renal damage in scleroderma pts occurs when?

A

Initial 4 years

28
Q

______ and _______ are present in almost all scleroderma pts

A

Raynaud phenomenon

Antinuclear antibodies

29
Q

_______ antibodies are found in 50% CREST pts

A

Anticentromere

30
Q

Tx severe Raynaud syndrome in scleroderma pt

A

CCB (e.g. nifedipine) or losartan

31
Q

What kind of counseling can you give scleroderma pt with esophageal involvement?

A
  • Take meds in liquid or crushed form
  • Avoid late-night meals and eating in supine/reclined position
  • PPIs
  • Prokinetics for hypomotility (erythromycin, metoclopramide, cisapride)
  • Delayed gastric emptying → small/frequent meals, remain upright 2 meals s/p meals