Busy. Please wait.
or

show password
Forgot Password?

Don't have an account?  Sign up 
or

Username is available taken
show password

why


Make sure to remember your password. If you forget it there is no way for StudyStack to send you a reset link. You would need to create a new account.
We do not share your email address with others. It is only used to allow you to reset your password. For details read our Privacy Policy and Terms of Service.


Already a StudyStack user? Log In

Reset Password
Enter the associated with your account, and we'll email you a link to reset your password.

Remove ads
Don't know
Know
remaining cards
Save
0:01
To flip the current card, click it or press the Spacebar key.  To move the current card to one of the three colored boxes, click on the box.  You may also press the UP ARROW key to move the card to the "Know" box, the DOWN ARROW key to move the card to the "Don't know" box, or the RIGHT ARROW key to move the card to the Remaining box.  You may also click on the card displayed in any of the three boxes to bring that card back to the center.

Pass complete!

"Know" box contains:
Time elapsed:
Retries:
restart all cards




share
Embed Code - If you would like this activity on your web page, copy the script below and paste it into your web page.

  Normal Size     Small Size show me how

Scleroderma

Rheumatology

QuestionAnswer
Dx gold std for PM/DM bx (open bx preferred; can do needle)
PM/DM epi 2 peaks (kids; >50 yo); very rare; often assoc w/malig (esp DM) & other autoimm dz
DM/PM Pathophys: autoimmune dz freq assoc w/ other autoi dz; injury to mx fibers is immune-mediated; pos response to immunosupp tx; unique autoAbs
DM/PM Pathophys: environmental triggers UV light, infection, drugs
Polymyositis = CD8+ T cell-mediated antigen specific toxicity, class I MHC expression on mx cell fibers
Dermatomyositis = Perivascular B cells and CD4+ T cells, Ig and complement deposition in capillaries
Inclusion body myositis = CD8+ T cells, intracellular vacuoles
Rarest of the inflammatory myopathies = PM
Considered pathognomonic of dermatomyositis Gottron’s papules
Gottron’s sign: a macular rash in same areas, no papules
V sign: macular photosensitivity rash over anterior neck; can also occur on face or scalp
Shawl sign: macular rash over posterior shoulders, neck
PM/DM: Constitutional sx: fever, malaise, weight loss, Raynaud’s phenomenon (may have Raynaud for yrs before other sx; then Raynaud remits & other sx show up)
PM/DM: Musculoskeletal: symmetric arthritis, joint contractures
PM/DM: GI: esophageal dysmotility (also stomach, small bowel), dysphagia
PM/DM: Cardiac: conduction abnormalities, dilated cardiomyopathy
PM/DM: Skin: calcinosis
PM/DM: Pulmonary: interstitial lung disease, pneumonitis
Inclusion Body Myositis (IBM): Epi In pts >50yrs: most common inflam myopathy; M 2X F
IBM: Clinical presentations: Insidious onset; slow prog; weakness & atrophy of distal mx (calves, foot & finger flexors); present w/ weak grip, foot drop or tripping; weak quads: present w/ falls, impaired mobility; Poor response to treatment modalities
Idio Inflam Myo: Clin findings sig: pt c/o weakness; strikingly elev CK; poss high LDH, aldolase; Abnml EMG (nonspecific but classic)
definitive diagnostic test for Idio Inflam Myo = Mx bx; inflammatory infiltrate; Autoantibodies
Inflam myopathies: ANA Ab 50% prev; High titers usually indicate presence of another CTD
Inflam myopathies: Anti-Jo Ab 25% prev; PM or DM & ILD, arthritis, Raynaud’s
Inflam myopathies: Anti-PM-Scl low prev; Ab to nucleolar protein complex; assoc with PM/SSc
Inflam myopathies: Anti-Mi-2 low prev; DM with V sign, shawl sign
Idio Inflam Myo: mainstay of tx = Glucocorticoids: oral prednisone start high (60mg/d); taper as CK normalizes
Idio Inflam myopathies: Tx steroids; screen for malignancies
Idio Inflam myopathies Other immunosupp tx: azothioprine, MTX, cyclophosphamide, mycophenolate mofetil
Idio Inflam Myo: Immunomodulators IV immunoglobulin: 2g/kg over 2-5 days
IBM: Tx generally non-responsive to immunosuppresive tx
Scleroderma: Pathogenesis: defect in fibroblast metabm: fibrosis of skin & internal organs (lungs, kidneys, heart, GI tract); vascular dysfunction; (auto)immune cell activation; interaction among the 3
Scleroderma: vascular dysfunction (vasculopathy) Raynaud; digital ischemia, infarction
Scleroderma: immune cell activation increased levels of cytokines & T-cell receptor molecules; disease specific autoantibodies
Scleroderma Epidemiology F 3-4 x M; US: 10-20/ million; age, gender & ethnic sig factors in suscept; more common in AA pts; usu 3d / 4th decade
Scleroderma: Environmental factors: viral infection; silica
Scleroderma: vasculopathy: earliest manifestation = Raynaud’s phenomenon
Scleroderma: Cellular / humoral autoimmunity: Early in dz, monos & T cells accumulate in skin & affected organs
Scleroderma: Circulating autoantibodies present in nearly all pts; highly specific for sclero; exact role in pathogenesis unclear
Scleroderma: Fibrosis: progressive replacement of normal tissue w/ dense conn tissue: leads to exuberant matrix remodeling & scar formation; fibrosis dev in skin, lungs, tendon sheaths, heart, GI tract, some endocrine organs; functional impairment
Sclerodactyly = localized thickening / tightness of skin of the fingers or toes
CREST syndrome: assoc w/lcSSc; calcinosis, Raynaud; esophageal dysmotility sclerosis & telangiectasias
Scleroderma: Skin: lcSSc limited to face, fingers and toes
Scleroderma: Skin: dcSSc fibrosis is widespread
Scleroderma: Lungs: leading cause of death in dcSSc = interstitial disease
Scleroderma: GI tract: dysmotility of esophagus, stomach, small bowel; GERD sx develop early
Scleroderma: MS periph neuropathies; genl arthralgias (hands, wrists); tendon rubs; myositis (dcSSc, not common in lcSSc)
Scleroderma: ANA prev 95%; both lcSSc & dcSSc
Scleroderma: anti-topoisomerase (Scl-70) more assoc w/diffuse dz; assoc w/ tendon rubs, pulmo fibrosis, inc mortality
Scleroderma: anti-centromere more in LcSSc; assoc w/ CREST, pulmonary HTN, Raynaud
2 autoAbs unique to scleroderma: anti-topoisomerase (Scl-70); anti-centromere
Scleroderma: Tx tx manifestn (eg, Raynaud)
Scleroderma: Tx: GI sx: PPI /H2 blockers, Abx, prokinetics; frequent small meals
Scleroderma: Tx: Vasculopathies: Raynaud: smoking cessation, CCBs, topical nitroglycerin, losartan, prazosin; statins: delay progression?
Scleroderma: Tx: Cardiopulmonary: tx right-sided failure, pulmonary HTN; O2; cyclophophosphamide; trepotinil
Polymyositis tx HD steroids, MTX, or azathioprine until sxs resolve
Scleroderma tx: renal ACEI
DM/PM S/S: progressive, symmetric proximal weakness (neck, pharynx, pelvis/thighs, shoulder); inc mx enzymes; histologic changes (inflam infiltrate) in striated mx tissue
PM/Dermatomyositis dx assoc with: assoc w/occult malig
Dermatomyositis cutaneous sx heliotrope; Gottron’s papules; Gottron’s sign; V sign; Shawl sign
Dermatomyositis: heliotrope: erythematous or violaceous macular rash of one or both eyelids; usually accompanied by edema
Dermatomyositis: Gottron’s papules: sl raised pink, dusky red or violaceous papules over dorsal sides of MCP/PIP and/or DIP joints. Can also occur over wrists, elbows or knees
PM/DM: Pulmonary: interstitial lung disease, pneumonitis
Inclusion Body Myositis: Clinical presentations: Insidious onset; slow prog; weakness & atrophy of distal mx (calves, foot & finger flexors); weak grip, foot drop or tripping; weak quads: falls, impaired mobility; Poor response to tx
Scleroderma: Pathogenesis: defect in fibroblast metabm: fibrosis of skin & internal organs (lungs, kidneys, heart, GI tract); vascular dysfunction; (auto)immune cell activation; interaction among the 3
Scleroderma: vascular dysfunction (vasculopathy) Raynaud; digital ischemia, infarction
Scleroderma: immune cell activation increased levels of cytokines & T-cell receptor molecules; disease specific autoantibodies
Scleroderma: Environmental factors: viral infection; silica
Scleroderma: Cellular / humoral autoimmunity: Early in dz, monos & T cells accumulate in skin & affected organs
Scleroderma: Skin: hallmark is: fibrosis of skin
Scleroderma: Skin: lcSSc vs dcSSc lcSSc (limited cutaneous systemic Sc): limited to face, fingers and toes; dcSSc: fibrosis is widespread
Created by: Abarnard