Scleroderma Flashcards Preview

SBM IV CT/MS > Scleroderma > Flashcards

Flashcards in Scleroderma Deck (26):
1

Q image thumb

morphea

2

types of scleroderma

A image thumb
3

difference b/t limited and diffuse scleroderma - sx, progression and markers

Diffuse: much worse - early organ involvement, renal crisis, pulmonary fibrosis

  • Topo I (Scl-70) - pos in 20-30%

Limited: CREST, pulmonary HTN

  • Centromere - pos in 50-90%

A image thumb
4

skin changes in scleroderma

early: puffy/edematous, erythema, pruritis

later: shiny, tight, thick skin 

  • pigment changes
  • sclerodactyly
  • digital ulcers/pitting at fingertips

5

do all scleroderma pts have skin involvement?

no!  5% have scleroderma sine sclerosis

  • raynauds, GI probs, autoantibodies telangiectasia
  • delayed dx
  • prognosis similar to limited disease

6

systemic sclerosis - limited disease

CREST:  Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasias

 

7

Q image thumb

calcinosis in CREST

8

Q image thumb

dilated nailfold capillaries - highly specific for connective tissue disease

9

vasculopathy in scleroderma

obliterative vasculopathy (NOT VASCULITIS) --> raynauds, pulmonary artery HTN, scleroderma renal crisis

10

prognostic factor for scleroderma renal crisis

anti-RNA polymerase III antibody

11

pathogenesis of fibrosis in scleroderma

monocytes/macrophages activated by endothelial damage --> T cells activated --> profibrotic cytokines

fibroblasts stimulated --> increased deposition of Type 1 and 3 collagen --> skin changes, pulmonary fibrosis, GI manifestation

12

pulmonary aspect of scleroderma

ILD  - more risk in diffuse scleroderma

Test: PFTs for restrictive pattern, HRCT for ground class opacities, honeycombing

ILD often occurs w/in first 3 years of disease

13

GI complications of scleroderma

present in 90%

hypomotility (esophagus, entire bowel), GERD, gastroparesis, small-bowel and colon stuff too

14

Q image thumb

watermelon stomach in scleroderma: musclar atrophy, fibrosis, thickening of vessels

15

musculoskeletal sx in scleroderma

  • arthralgias and stiffness
  • synovitis 
  • tendon friction rubs - spelcific
  • joint contractures
  • myopathy

16

scleroderma epi

30-50 yo females

severe phenotypes in young AAF, increase prevalence in oklahoma choctaw native americans

17

strongest risk factor for scleroderma

positive FH

18

tx for scleroderma

lots of things have been tried, nothing works very well

19

what is the most common scleroderma-related cause of death?

ILD

(used to be renal crisis but now we can tx this w/ ACE inhibitors)

20

what is sjogren's?

primary: dry eyes and mouth secondary to autoimmune dysfunction of exocrine glands

secondary: dry eyes and mouth in presence of another autoimmune CTD (usually RA)

21

sjogren's epi

90% women, usually occcurs 45-55 yo

22

antibodies in Sjogren's

SS-A (Ro), SS-B (La) nuclear antigens - these are part of a complex that process RNA polymerase III transcripts

23

 clinical features of sjorgren's

Ocular - very dry, gritty eyes

Oral - dry mouth, loss of saliva, tooth decay, susceptible to oral candidiasis, parotid enlargement

24

tests for sjogren's

eyes:

  • rose bengal and lissamine green stains for devitalized tissue
  • filamentary keratitis
  • corneal scarring
  • Schrimer's test (filter paper)
  • Slit lamp exam: best way to observe clinical signs of SS

mouth:

  • observation of salivary pooling
  • salivary gland biopsy

25

complications of Sjogren's

LYMPHOMA: 18x more likely

26

tx for sjogren's

moisture replacement/capture: ex punctal plugs, artificial tears

stim endogenous secretion: ex pilocarpine

immunosuppression for systemic disease