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MDTI > Scleroderma > Flashcards

Scleroderma Flashcards

1
Q

three pathogenic processes in systemic scleroderma

A

immune dysregulation, vascular dysfunction, fibrosis

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2
Q

most important cytokine in scleroderma

A

TGF-B

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3
Q

mechanism that leads to increased collagen synthesis in scleroderma

A

increased collagen gene transcription

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4
Q

primary clinical consequences of scleroderma

A

skin fibrosis & ischemia/fibrosis of visceral organs

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5
Q

what does scleroderma mean?

A

hard skin

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6
Q

definition of systemic scleroderma

A

an autoimmune connective tissue disorder of unknown etiology characterized by the triad of fibrosis, vascular dysfunction, and immune dysregulation

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7
Q

gross appearance of skin in patients with scleroderma

A

tight and shiny. sebacous glands, hair follicles, and sweat glans get occluded. salt and pepper pigment changes.

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8
Q

name when scleroderma affects fingers?

A

sclerodactyly

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9
Q

raynaud’s phenomenon

A

spastic effect on vessels in fingers, which completely occlude and blood pools near base. not unique to scleroderma

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10
Q

calcinosis

A

calcium deposits in tissue. not unique to scleroderma

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11
Q

effect of scleroderma on GI?

A

esophageal dysmobility. difficulty swallowing and digesting

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12
Q

telangiectasias

A

dilated ends of vessels. not unique tp scleroderma. most common cause of a GI bleed in scleroderma though

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13
Q

most common cause of death in scleroderma patients?

A

bilateral interstitial lung fibrosis

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14
Q

classification of scleroderma

A

Systemic (diffuse, limited, overlap) & localized (morpheme, linear scleroderma)

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15
Q

systemic vs localized scleroderma

A

in systemic, internal organs are always involved. just the skin is affected in localized scleroderma

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16
Q

diffuse cutaneous scleroderma

A

symmetric, widespread skin fibrosis that starts at the toes and moves up to head. RAPID progression and early visceral involvement. poor prognosis (10 yrs)

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17
Q

limited cutaneous scleroderma

A

symmetric skin fibrosis limited to the distal extremities and face. Raynauds manifests ten years before skin and late appearing organ manifestations. SLOW progression. good prognosis

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18
Q

anticentromere antibody

A

present in limited cutaneous scleroderma

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19
Q

CREST (scleroderma symptoms)

A

calcinosis, raynauds phenomenon, esophageal dysmotility, sclerodactyly, telangectasias

20
Q

overlap syndromes (of systemic scleroderma)

A

mix of diffuse and limited scleroderma. often associated with other rheumatic disease

21
Q

common facial characteristics of scleroderma

A

beaking nose, pursed lips, facial muscle atrophy, tightening of skin, telangestasias on forehead

22
Q

pathogenesis of scleroderma

A

genetic predisposition + environmental trigger=immune system activation. Causes B cells to start producing autoantibody and T cells to activate and infiltrate skin/vascular epithelium and release profibrotic cytokines (TGF-B).

23
Q

antibodies produced in scleroderma

A

ANA (90%), anti-scl, anti-centromere, anti-topoisomerase, anti-RNA polymerase 3

24
Q

anti-scl 70

A

antibody indicative of diffuse cutaneous scleroderma (African Americans)

25
Q

anticentromere antibody

A

antibody indicative of limited cutaneous scleroderma (Caucasians)

26
Q

genes whose transcription is unregulated in scleroderma

A

COL1A2 (type 1 collagen), TGF-B1, Fibrillin-1

27
Q

tight skin mouse model

A

mutate fibrillin gene leads to disorganized, unstable microfibrils that inappropriately signal fibroblasts to increase TGF-B and deposit type 1 collagen in ECM

28
Q

tissue inflammation in early scleroderma

A

lymphocyte infiltrates skin (mostly CD4 T cells) and release cytokines and growth factors. CD8 T cells predominate in lung tissue

29
Q

important cytokines released by inflammatory cells of scleroderma

A

TGF-B, CTGF, PDGF

30
Q

TGF-B

A

sensitizes fibroblasts to stay persistently active and decreases the production of collagen degrading metaloproteinases

31
Q

role of cytokines released by inflammatory cells in scleroderma

A

induce alterations of the ECM gene expression

32
Q

CTGF (connective tissue growth factor)

A

stimulated by TGF-B. sensitizes fibroblasts to stay persistently activated

33
Q

PDGF (platelet derived growth factor)

A

TGF-B increases PDGF receptors on fibroblasts, which make them more sensitive to the mitogenic effects of PDGF (which is required for cellular division)

34
Q

characteristics of damaged vessel walls in scleroderma

A

thickened and damaged wall, occluded lumen, up regulation of agents that cause vasoconstriction, and down regulation of agents that cause vasodilation. presence of myofibroblasts rather than fibroblasts

35
Q

how can you easily/quickly test a patient for scleroderma vs Raynauds?

A

Do exam, get history, test for ANA and ESR. Then look at the capillaries of their nail folds. If someone has autoimmune scleroderma, you will see dilated loops.

36
Q

tissue fibrosis in scleroderma

A

overproduction of type 1 collagen and other ECM macromolecules by fibroblasts. (up regulation of COL1A1)

37
Q

clinical consequences of skin fibrosis in scleroderma

A

inflammatory infiltrates sparse, thickened dermis, replacement of dermis and subcutaneous fat with collagen bundles, loss of skin appendages, late atrophy of epidermis

38
Q

clinical consequence of renal necrosis in scleroderma

A

vascular damage (narrowing/ischemia/cortical hemorrhage/onion skin) leading to scleroderma renal crisis. occurs early with rapidly progressing skin thickening. abrupt onset of malignant hypertension

39
Q

how to prevent renal damage?

A

ACE inhibitors forever once symptoms appear. needs to be caught early though

40
Q

clinical consequence of lung fibrosis in scleroderma?

A

interstitial lung disease (diffuse fibrotic tissue replacement, anti-topoisomerase) & pulmonary hypertension

41
Q

GI manifestations of scleroderma

A

gastroparesis, malabsorption, pseudoobstruction, diverticuli, anorectal sphincter dysfunction, biliary cirrhosis

42
Q

immune dysregulation in scleroderma

A

activated b cells, autoantibody production

an early predominant t cell inflammatory infiltrate

43
Q

vascular abnormalities in scleroderma

A

initial unknown endothelial cell injury leading to structural damage, local production of GFs and cytokines, thickening of vessel wall, increased contractility of vessel

44
Q

fibrosis in scleroderma

A

fibroblast chemotaxis and proliferation, excessive production of type 1 collagen, excessive production of other ECM molecules

45
Q

most important cytokine in scleroderma

A

TGF-B

MDTI (42 decks)

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