Scleroderma

Question 1

Answer 1

morphea

Question 2

types of scleroderma

Answer 2

Question 3

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

Answer 3

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%

Question 4

skin changes in scleroderma

Answer 4

early: puffy/edematous, erythema, pruritis
later: shiny, tight, thick skin

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

Question 5

do all scleroderma pts have skin involvement?

Answer 5

no! 5% have scleroderma sine sclerosis

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

Question 6

systemic sclerosis - limited disease

Answer 6

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

Question 7

Answer 7

calcinosis in CREST

Question 8

Answer 8

dilated nailfold capillaries - highly specific for connective tissue disease

Question 9

vasculopathy in scleroderma

Answer 9

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

Question 10

prognostic factor for scleroderma renal crisis

Answer 10

anti-RNA polymerase III antibody

Question 11

pathogenesis of fibrosis in scleroderma

Answer 11

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

Question 12

pulmonary aspect of scleroderma

Answer 12

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

Question 13

GI complications of scleroderma

Answer 13

present in 90%

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

Question 14

Answer 14

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

Question 15

musculoskeletal sx in scleroderma

Answer 15

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

Question 16

scleroderma epi

Answer 16

30-50 yo females

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

Question 17

strongest risk factor for scleroderma

Answer 17

positive FH

Question 18

tx for scleroderma

Answer 18

lots of things have been tried, nothing works very well

Question 19

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

Answer 19

ILD

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

Question 20

what is sjogren’s?

Answer 20

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)

Question 21

sjogren’s epi

Answer 21

90% women, usually occcurs 45-55 yo

Question 22

antibodies in Sjogren’s

Answer 22

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

Question 23

clinical features of sjorgren’s

Answer 23

Ocular - very dry, gritty eyes

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

Question 24

tests for sjogren’s

Answer 24

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

Question 25

complications of Sjogren's

Answer 25

LYMPHOMA: 18x more likely

Question 26

tx for sjogren's

Answer 26

moisture replacement/capture: ex punctal plugs, artificial tears stim endogenous secretion: ex pilocarpine immunosuppression for systemic disease