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2 - CPR (cardio) > Connective Tissue Disorders - Scleroderma/Sjogren > Flashcards

Connective Tissue Disorders - Scleroderma/Sjogren Flashcards

1
Q

What are the 3 types of Scleroderma?

A
  1. Localized
  2. Limited (CREST)
  3. Diffuse
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2
Q

What are the 3 types of Scleroderma?

A
  1. Localized
  2. Limited (CREST)
  3. Diffuse
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3
Q

What is the hallmark of Scleroderma?

A

Thickening and hardening of skin

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4
Q

What is the hallmark of Scleroderma?

A

Thickening and hardening of skin

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5
Q

Are there approved therapies for Scleroderma?

A

NO - treat symptoms

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6
Q

What do most patients have with Scleroderma?

A

Secondary Raynaud’s Phenomenon

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7
Q

Localized Scleroderma - skin and internal organ involvement?

A

Patches of thickened and hardened skin

- NO organ involvement

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8
Q

Discreet areas of discolored skin induration

(morphea = patches) is seen with?

A

Localized Scleroderma

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9
Q

Is Raynaud’s phenomenon present with Localized Scleroderma?

A

NO

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10
Q

Symptoms of Localized Scleroderma?

A

Usually Asymptomatic or itching/pain of localized areas of discolored skin induration patches (morphea)

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11
Q

A biopsy of the skin with Localized Scleroderma looks the same as?

A

Other forms of Scleroderma

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12
Q

Limited Scleroderma - skin and organ involvement?

A

Thickening and hardening of skin of fingers/toes, face/neck and distal extremities
- SOME organ involvement

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13
Q

Limited Scleroderma CREST symptoms?

A

CREST

  • Calcinosis Cutis
  • Raynaud’s Phenomenon
  • Esophageal Dysmotility
  • Sclerodactyly
  • Telangiectasia
  • progressive pulmonary A. HTN = dyspnea
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14
Q

Limited Scleroderma CREST symptoms?

A

CREST

  • Calcinosis Cutis
  • Raynaud’s Phenomenon
  • Esophageal Dysmotility
  • Sclerodactyly
  • Telangiectasia
  • Pulmonary A. HTN = Dyspnea
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15
Q

What pulmonary change is seen with Limited Scleroderma?

A

Pulmonary A. HTN = Dyspnea

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16
Q

Pulmonary A. HTN with Limited Scleroderma puts the patient at an increased risk for what malignancy?

A

Bronchoalveolar Carcinoma

17
Q

How do you diagnose Pulmonary A. HTN with Limited Scleroderma?

A

Right Heart Catheterization

18
Q

What antibody is positive with Limited Scleroderma?

A

(+) Anti-Centromere

19
Q

(+) Anti-Centromere antibodies

A

Limited Scleroderma

20
Q

What are some ways to treat the symptoms of Limited and Diffuse Scleroderma?

A
  • Calcium, ACE and PDE5 (-)
  • Glucocorticoids
  • Anti-reflux meds
21
Q

Diffuse Scleroderma - skin and organ involvement?

A

Thickening and hardening of skin and INCLUDES internal organs!

22
Q

What antibodies are (+) with Diffuse Scleroderma?

A

(+) Anti-scl 70 (anti - DNA Topoisomerase 1)

(+) Anti-RNA Polermase 3

23
Q

What antibodies are (+) with Diffuse Scleroderma?

A

(+) Anti-scl 70 (Anti-DNA Topoisomerase 1)

(+) Anti-RNA Polymerase 3

24
Q

What lung manifestation will be seen with Diffuse Scleroderma?

A

Interstitial Lung Disease = dyspnea and velcro crackles

25
Q

Symptoms of Interstitial Lung Disease seen with Diffuse Scleroderma?

A

Dyspnea and velcro crackles

26
Q

Renal crisis can also occur with Diffuse Scleroderma. What are the manifestations of that?

A

HTN
Anemia
Proteinuria

27
Q

What will the CXR look like with Interstitial Lung Disease with Diffuse Scleroderma?

A

Ground glass/honey comb appearance

28
Q

How do you diagnose Interstitial Lung Disease with Diffuse Scleroderma?

A

Pulmonary Function Tests

29
Q

List 3 other manifestations of Scleroderma?

A

GERD
Hypothyroid
Carpal Tunnel

30
Q

Autoimmune destruction of exocrine (salivary) glands

A

Sjogren Syndrome

31
Q

What are the “sicca” symptoms with Sjogren syndrome?

A
  • Keratoconjunctivitis sicca = dry eye with foreign body sensation and decreased tear production
  • Dry mouth and vagina
  • Bilateral parotid gland enlargment
32
Q

What are the “sicca” symptoms of Sjogren Syndrome?

A
  • Keratoconjuncitivits Sicca = dry eye with foreign body sensation and decreased tear production
  • Dry mouth and vagina
  • Bilateral parotid gland enlargement
33
Q

Those with Sjogren Syndrome have an increased incidence of?

A

Oral (candida) infections
Dental Caries
MALT Lymphoma (non B cell NHL)

34
Q

Serology of Sjogren Syndrome?

A

Polyclonal Hypergammaglobulinemia

+) Anti-RO (SSA) and Anti-LA (SSB

35
Q

What is essential for diagnosis of Sjogren Syndrome?

A

Labial (lip) salivary gland biopsy

36
Q

Is there treatment for Sjogren Syndrome?

A

No - treat symptoms

37
Q

Polyclonal Hypergammaglobulinemia

+) Anti-RO (SSA) and Anti-LA (SSB

A

Sjogren Syndrome

38
Q

What can Glucocorticoids induce?

A

Renal crisis – bad for patients with renal issues already

2 - CPR (cardio) (44 decks)

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