Master the Boards: Rheumatology 3 Flashcards
3 main Sx of scleroderma
- Skin- tight fibrous thickening- sclerodactyly
- Raynaud phenomenon(vascular hypersensitvity) white ➡️ blue ➡️ red, can be painful
- Joint Pain- mild and symmetrical
Tight skin+heart burn+ Raynaud dx
scleroderma
Additional findings in diffuse scleroderma (4)
- Lung: fibrosis and pulmonary HTN (leading cause of death)
- GI: esophageal dysmotility leading to reflux and sometimes barrels, PBC 15%, wide mouth colonic diverticula
- ❤️:restrictive cardiomyopathy and premature CAD
- Renal: Malignant HTN
Scleroderma serology
ANA + 95% but not specific
Antitopoisomerase (SCL-70) only present 30%
Tx for scleroderma
- ACEi for HTN and renal involvement
- Bosentan(endothelia antagonist), prostacyline analogs (epoprostenol/ iloprost), or sidenofil for pulmonary HTN
- CCB for Raynaud
- PPI for GERD
- Cyclophosphimide for lung fibrosis
- methotrexate or mychophenolate for skin thickening
Limited Scleorderma- CREST stands for
Calcinosis of fingers Raynaud Esophageal dysmotility sclerodactyly Telangiectasia
CREST syndrome serology
anti-centromere and sometimes antitopoisomerase (scleras-70)
Eosinophilic Fasciitis sx, and tx
eosinophillia
peau d’orange
sx worse with exercise
tx = steroids, 2nd line methotrexate
What does scleroderma have that Eosinophilic Fasciitis does not
Hand involvment
Raynaud
♥️,lung, kidney involvement
weakness + ⬆️ CPK+ ⬆️Aldolase+Biopsy =
Polymyositis
weakness + ⬆️ CPK+ ⬆️Aldolase+Biopsy +Rash=
Dermatomyosytisis
what muscles are weak in PM, DM
Proximal
3 rashes seen with Dermatomyosytisis
Gottron Papules on MCP’s
Heliotrope Rash- purple lesions around eyes
Shawl sign-shoulder and neck erythema
Diagnostic tests for PM/DM
Elevated CPK and Aldose
Abnormal electromyogram
for CCS order LFT’s and ANA
Biopsy is most accurate test
What does the presence of anti-JO-1 in DM/PM correlate with an increased risk for
Interstitial lung dz