Systemic sclerosis, Scleroderma Flashcards Preview

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Flashcards in Systemic sclerosis, Scleroderma Deck (13)
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1
Q

Classification for diagnosing scleroderma

A

ACR/EULAR criteria again

Skin thickening on fingers of both hands extending beyond the metacarpophalangeal joints - 9 points, sufficient for diagnosis

Fingertip pitting scars - 3

Fingertip ulcers - 2

Telangiectasia - 2

Visible/abnormal Nailfold capillaries - 2

Pulmonary hypertension or interstitial lung disease - 2

Raynauds phenom - 3

Scleroderma related antibodies - 3
anti-centromere
anti-topoisomeraise 1
anti-RNA pol

2
Q

First signs of scleroderma and diagnosing very early SSc

A

Puffy fingers - sclerodactyly is the most defining characteristic, and earliest
Raynaud phenomenon - is almost always present in SSc

If it is suspected, preform:
Capillaroscopy
Serology for anti-TOPO1 and anti-Centromere abs

if either one is positive diagnose as very early SSc., and investigate whether it has progressed to early SSc with evidence of systemic/organ sclerosis:

1) Esophageal manometry - a tube inserted into the stomach, that measures the force applied to it by the esophagus and sphincter when you swallow.
2) Chest HRCT for lung evaluation
3) Echocardiography for Restrictive CMP eval

3
Q

Treatment of Scleroderma and treatment window

A

Treatment window is only in very early/early stage SSc, Before established skin fibrosis and atrophy has set in.

When it is still in the puffy fingers stage with only mild digital ulcers.

4
Q

Skin symptoms of scleroderma

A

Puffy fingers
Raynauds phenom
Ulceration on fingertips
Scarring on fingertips

Subcutaneous calcinosis

Taut, flat face without wrinkles

Telangiectasia, petechia, spider nevi

5
Q

Name of the skin scoring system for SSc

A

Rodnan skin score measuring theckening of the skin over the body regions.

6
Q

What are the two types of scleroderma and their relative incidence

A

Limited scleroderma - 80%

Diffuse scleroderma - 20%

7
Q

Characteristics of diffuse scleroderma

A

Skin on the entire body is involved

Raynauds phenomenon only precedes skin disease by 1 or 2 years, or presents simultaneously

Organ systems are involved, lung, heart, GI, Kidney, present early in course

Nailfold capillaries are dilated and also have dropout, avascular areas

Anti-topo1 is highly specific for diffuse form

8
Q

Characteristics of limited scleroderma

A

Only the facial skin and skin on the distal arms and legs are affected.

Raynauds precedes skin changes by many years

GI and pulmonary are the only organs affected, and to a milder degree

Nailfold dilation, no dropouts

Anti-Centromere abs highly specific

Slowly progressive with better prognosis, except in those that develop pulmonary hypertension

9
Q

Vasculopathy of scleroderma

A

A proliferative vasculopathey that progresses to an obliterativ e vasculopathy of the arterioles.

Can cause acute renal disease and scleroderma renal crisis.

Increased vasoconstrition
Myo-intimal proliferation and fibrosis of the vessels
Thrombosis increased

Causes rapidly progressive AKI
Malignant hypertension

Treat with ACEIs/ARBs, NOT with corticosteroids.

10
Q

GI dysfunction of SScs

A

Esophageal dysmotility

Esophageal reflux

Impaired gastric emptying and gastric paresis

Bacterial overgrwoth, Malabsorption syndrome,

Large colonic diverticuli

Anal sphincter dysfunction

Primary billiary cirrhosis

11
Q

Main cause of death in scleroderma,

Survival/prognosis of the diffuse and limited cases

A

End stage Pulmonary fibrosis and its complications

10 year surv. ~50% in diffuse form
Normal life span for most cases of limited form, with the exception of patients that develop pulmonary HTN.

12
Q

SSc treatment of lung fibrosis

A

Low, medium, and high intensity treatments, stepped up until remission is induced

Low: Azathiprine, mycophenolate mofetil
Medium: Cyclophosphamide, Rituximab
High: Hematopoietic stem cell transplant

Lung transplantation if pulmonary status indicates it.

13
Q

SSc treatment of renal disease

A

manage the hypertension

Mild/moderate hypertension: ACE-Is and close monitoring

Severe htn: ACEIs/ARBs and CCBs,
IV vasodilators, nitroprusside,