5 - Systemic Sclerosis

Question 1

Who gets systemic sclerosis?

Answer 1

Choctaw Indians

Black women >50yrs

Question 2

Systemic sclerosis is aka?

Answer 2

Scleroderma

Question 3

Scleroderma has a STRONG association w?

Answer 3

Raynaud’s phenomenon

Question 4

What is scleroderma?

Answer 4

Heterogeneous rheumatic diseaese characterized by thickening and fibrosis of the skin with accompanying vascular lesions especially in lungs and kidneys

Question 5

Strongest risk factor for scleroderma?

Answer 5

Family history

Question 6

Besides fhx what else is linked to sclerosis?

Answer 6

Autoimmunity
CMV
Environmental (silica dust)

RAYNAUDS phenomenon

Question 7

Types of sclerosis?

Answer 7

Systemic

• limited
• diffuse

Localized

• morphea
• linear

Question 8

S/S of systemic sclerosis?

Answer 8

Skin thickening (puffy)
Carcinosis (PIP, fingertips)
Skin tightening
Digital flexion contracturs
Telangectasis
Vascular 
Cardiac (arrhthmias/pericardial effusions)
Pigmentation/depigmentation (vitiligo-like)

Question 9

Steroids and systemic sclerosis?

Answer 9

Dont help and

Cause renal crisis

Treat with ACEIs

Question 10

Sclerosis Most frequent symptoms?

Answer 10

1. Raynaud phenomenon
2. GERD w/ w/o dysmotility
   - skin changes
   - swollen fingers
   - arthralgias

Question 11

Labs for scleroderma?

Answer 11

ANA (95% sensitive)

Anti-SCL 70
Anticentromer AB (limited scleroderma)

Question 12

Imaging/tests for systemic slcerosis?

Answer 12

CSR
High res CT (GI)
EKG
Echo  (pulm HTN)
Stress testing 
PFTs

Question 13

Why order PFT (scleroderma)

Answer 13

Baseline + q 4-12 mo
Looking for:
- reduction in DLCO
- reduction OOP to decline in FVC

Question 14

Youre working up you systemic sclerosis pt and there is a nagging thin in your mind brain, what office test should you run?

Answer 14

Nailfold capillary evaluation

Bedside exam using ophthalmoscope to visualize nailfold capillaries

Normal: thin, linear, uniform
Scleroderma: capillary dropout and dilated capillary loops

Question 15

What meds will help cure systemic sclerosis?

Answer 15

No drug will alter the natural course of the disease

Question 16

What is the progression for the skin manifestations?

Answer 16

Reach peak involvement over first 18-24 months then gradually improve w or w/o therapy

Question 17

Immune system targeted therapy for systemic scleroderma?

Answer 17

Cyclophosphamide - pulmonary sxs

Don’t use
Corticosteroids - RENAL CRISIS

Question 18

Symptom specific tx with scleroderma?

Lots of info, not a great flash card

Answer 18

Raynauds: CCB
HTN/Renal crisis: ACEI
Intersitital lung disease: cyclophosphamide
Pulmonary HTN: endothelin receptor antagonist, PDE5 inhibitor
GERD: PPI
Delayed gastric emptying: metoclopramide/domperidone
Mild lower GI: loperamide/fiber
Small bowel hypermotility: ocreotide
Bacterial overgrowth: tetracycline/metronidazole

Question 19

Describe limited scleroderma?

Answer 19

Symmetrical skin thickening of :

• fingers
• distal arms
• legs
• face
• neck

Question 20

Limited scleroderma onset post raynauds?

Answer 20

Slow progression after onset of raynauds

Question 21

Limited scleroderma is a later visceral disease but:

Answer 21

Digital ischemia and pulmonary HTN are more prominent

Question 22

Labs for limited scleroderma?

Answer 22

ANA (80-95%)
SCL-70 Pos (20%)
Anticentromere pos (50%)
- (HIGHLY SPECIFIC)

Question 23

What is crest syndrome?

Answer 23

A subtype of limited scleroderma

- invovles fingers distal to MCP

Question 24

WTF does CREST mean?

Answer 24

C: calcinosis cutis
R: raynauds 
E: esophageal dysmotility
S: sclerodactyly
T: Telangectasias

Question 25

CREST syndrome labs | - hint its the same as limited scleroderma

Answer 25

ANA (80-95%) SCL-70 Pos (20%) Anticentromere pos (50%) - (HIGHLY SPECIFIC)

Question 26

What is raynauds syndrome?

Answer 26

Episodic exaggerated vasospasm of the digital arteries in association with exposure to cold temp Can be primary (idiopathic) or secondary

Question 27

raynauds is highly associated with?

Answer 27

Scleroderma (90%) | - may precede diagnosis by years

Question 28

Pts >30 with new onset raynauds?

Answer 28

ANA | Nailfold capillary exam

Question 29

Other raynauds warning signs (for scleroderma)

Answer 29

- Severe painful episodes of vasospasms - Signs of digital ischemia - Tissue damage - Systemic s/s of 2ndary dx

Question 30

Tx for raynauds?

Answer 30

Avoid cold CCB

Question 31

Diffuse scleroderma affects?

Answer 31

Same as limited - fingers - distal arms - distal legs - face - neck + - trunk - proximal aspect of limbs

Question 32

Diffuse vs limited scleroderma?

Answer 32

Diffuse is worse It has more significant visceral disease

Question 33

Limited is more prone to pulmonary HTN but diffuse is more prone to?

Answer 33

Renal crisis Interstitial lung disease Pulmonary fibrosis

Question 34

Other less common problems with diffuse?

Answer 34

Cardiac disease - pericarditis - myocardial fibrosis - heart failure

Question 35

Labs for diffuse scleroderma?

Answer 35

ANA SCL-70 pos Anticentromere NEG