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Senoir 21-2 > Scleroderma > Flashcards

Scleroderma Flashcards

1
Q

Scleroderma aka…

A

Systemic Sclerosis

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2
Q

What are the two most distinguishing features in Scleroderma

A

Raynauds and ANA abs

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3
Q

What is the most common cause of death for a pt with scleroderma

A

Cardiac (30%)

Lung (25%)

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4
Q

What separates limited vs diffuse scleroderma

A

Limited (65%)
-Skin thickening confined to extremities, distal to elbows/knees
-Face involvement
Does NOT mean they lack internal organ involvement

Diffuse (30%)

  • Skin thickening extending to the proximal portion of the extremities
  • Trunk involvement
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5
Q

If a person has diffuse scleroderma

When would we suspect onset of Raynauds

A

Close to the Dx of scleroderma

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6
Q

What systemic manifestations are more common in diffuse Scleroderma compared to limited

A

ILD, often severe

Myocardial Dysfunction

Pulm HTN (group 2 and 3)

Renal crisis

More severe GI Dysmotility/ Gut Failure

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7
Q

What are the two Abs found in diffuse Sclerodrema

A

Antitopoisomerase (Scl-70), anti-RNA polymerase III

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8
Q

If a pt has limited scelroderma when would we suspect onset of Raynauds

A

Onset several years before scleroderma diagnosis

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9
Q

What type of PULM HTN is seen in Limited vs Diffuse Scleroderma

A

Limited: Group 1

Diffuse: Group 2 and 3

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10
Q

What are the Abs seen in limited scleroderma

A

Anticentromere, anti-Pm-Scl, anti-Th/To

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11
Q

What is CREST syndrome

A

Synonym for limited scleroderma

Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, & Telangiectasis

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12
Q

What is a sign of early scleroderma manifestation

A

Diffuse puffiness

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13
Q

What is the common Tx for Raynauds

A

Cold avoidance and CCB

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14
Q

What is the approach for a pt over the age of 30 with new onset Raynauds

A

Patients >age 30 with new onset Raynaud’s should have ANA and nailfold capillary examination

Esp if have severe painful episodes of vasospasm, signs of digital ischemia or tissue damage, or systemic signs/sx of secondary dx

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15
Q

How does scleroderma effect the heart

A

Ischemia-reperfusion injury secondary to small arterial disease of the myocardium leads to contraction band necrosis and tissue fibrosis

  • Arrhythmias
  • Cardiomyopathy
  • Overt symptoms of heart failure

Pericardial effusion = poor prognosis

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16
Q

How does scleroderma effect teh GI systems

A

Small and large brown peristalsis

Intermittent Constipation and Diarrhea
(Mimics IBS)

Can have bacterial overgrowth and malnutrition

May even lead to bowel rupture

17
Q

A scleroderma with an ubrupt onset of HTN

What is the concern

A

Renal Crisis

More common in Diffuse Type Scleroderma(10-15%)
Abrupt onset of hypertension—malignant hypertension
-MOST DREADED complication of systemic sclerosis

10% present without hypertension and have poor outcome

Elevated creatinine, proteinuria, hematuria

Onset usually within 3-5 years of disease onset

TREAT with ACEI: Captopril

18
Q

What makes somone high risk of developing renal crisis in Scleroderma

A

Early stages of diffuse scleroderma

Corticosteroid use

Pts who produce antibodies to RNA-polymerase III

19
Q

What is the risk with w pregnant pt and scleroderma

A

increased risk for pre-term delivery, small for gestational age newborns, and possibly hypertension

20
Q

Pulmonary fibrosis is more common in limited or diffuse scleroderma

A

Diffuse

21
Q

What is the Ab specific for Diffuse Sclerodema

A

Antitopoisomerase I antibodies (Anti-SCL 70)

22
Q

What is the Ab specific to limited scleroderma

A

Anticentromere AB

23
Q

How often should scleroderma pts get baseline PFTS

A

Baseline and every 4-12 months depending on stage of disease and pt symptoms

24
Q

What are the two suggestive factors for pulm HTN on PFTs for Scleroderma

A

Isolated reduction in Diffusing Capacity of Lung for Carbon Monoxide (DLCO)

OR

A reduction out of proportion to the degree of decline in FVC

25
Q

What are the findings in the nail beds of scleroderma pts

A

Nailfold capillary dropout and dilated capillary loops

26
Q

What is the treatment approach for skin conditions of scleroderma

A

Both mycophenolate mofetil (MMF) and cyclophosphamide (CYC) have shown evidence of benefit in the management of active scleroderma skin disease.

mycophenolate mofetil (MMF) is preferred FIRST due to milder side-effect profile!

27
Q

What medication should be avoided in Sclerodemra

A

Steroids

28
Q

What CCB should be used for Raynauds

A

Nifedipine
Most studied

DO NOT use diltiazem or verapamil (less selective for vascular smooth muscle)

29
Q

What two medications can be used to treat delayed gastric emptying

A

Metoclopramide and erthyromycin

30
Q

What is the role of octreotide in scleroderma

A

Used to treat severe GI Dysmotility

31
Q

What is the treatment for bacterial overgrowth

A

Rotating ABX : tetracycline and metronidazole

32
Q

Systemic therapy for MSK s/s in scelroderma

A

Overlapping inflammatory features may benefit from systemic therapy

  • Methotrexate
  • Azathioprine
  • IVIG
  • Rituximab
33
Q

What are the Rx for MGMT of ILD

A

Both mycophenolate mofetil (MMF) and cyclophosphamide (CYC) have shown evidence of benefit in the management of ILD

Lung transplant in severe disease

Senoir 21-2 (77 decks)

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