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
Most common serious complication of DM/PM
Malignancy, more with DM. Frequently in cervix, breasts, lung, pancreas, and ovaries
TX for DM and PM
steroids
refractory use azathioprine or methotrexate
still refractory try ritux or IVIG
hydroxychloroquine for skin findings
which muscles foes inclusion body myositis target
both proximal and distal but distal upper extremity flexors are particularly affected- slowly progressive
markers elevated in inclusion body myositis
elevated CK, confirm w biopsy
Effective tx for inclusion body myositis
none
Sx of mixed connective tissue dz (6)
- Hand Edema
- Synovitis
- possible myositis and pulmonary HTN
- Sclerodactyly, calcinocis, mlar rash, gottrans papules
- Serositis and sicca symtoms (50%)
- Kidney involvement (25%)
Most Specific test MCTD
anti-U1 ribonucleoprotein (anti RNP)
MCTD tx
steroids
azathioprine or methotrexate
Cyclophosphamide for ILD
Fibromyalgia sx (5)
- muscle aches and stiffness
- trigger points on palpation
- non refreshing sleep
- exercise intolerance
- depression and anxiety are common, cognitive fatigue
