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
Symptoms of Interstitial Lung Disease seen with Diffuse Scleroderma?
Dyspnea and velcro crackles
26
Renal crisis can also occur with Diffuse Scleroderma. What are the manifestations of that?
HTN Anemia Proteinuria
27
What will the CXR look like with Interstitial Lung Disease with Diffuse Scleroderma?
Ground glass/honey comb appearance
28
How do you diagnose Interstitial Lung Disease with Diffuse Scleroderma?
Pulmonary Function Tests
29
List 3 other manifestations of Scleroderma?
GERD Hypothyroid Carpal Tunnel
30
Autoimmune destruction of exocrine (salivary) glands
Sjogren Syndrome
31
What are the "sicca" symptoms with Sjogren syndrome?
- Keratoconjunctivitis sicca = dry eye with foreign body sensation and decreased tear production - Dry mouth and vagina - Bilateral parotid gland enlargment
32
What are the "sicca" symptoms of Sjogren Syndrome?
- Keratoconjuncitivits Sicca = dry eye with foreign body sensation and decreased tear production - Dry mouth and vagina - Bilateral parotid gland enlargement
33
Those with Sjogren Syndrome have an increased incidence of?
Oral (candida) infections Dental Caries MALT Lymphoma (non B cell NHL)
34
Serology of Sjogren Syndrome?
Polyclonal Hypergammaglobulinemia | +) Anti-RO (SSA) and Anti-LA (SSB
35
What is essential for diagnosis of Sjogren Syndrome?
Labial (lip) salivary gland biopsy
36
Is there treatment for Sjogren Syndrome?
No - treat symptoms
37
Polyclonal Hypergammaglobulinemia | +) Anti-RO (SSA) and Anti-LA (SSB
Sjogren Syndrome
38
What can Glucocorticoids induce?
Renal crisis -- bad for patients with renal issues already