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rheumatology > Scleroderma > Flashcards

Scleroderma Flashcards

1
Q

two things frequently seen in connective tissue diseases

A

raynaud’s phenomenon
non-erosie inflammatory arthritis– damaging surrounding structures not the joint itself

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2
Q

what is scleroderma

A

CTD characterized by sclerodactyly– skin thickening and tightening

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3
Q
  • unilateral patches (morphea), en coup de sabre, linear scar tissue w/o injury
  • negative antibodies, workup might be normal
A

localized scleroderma- sclerodactyly confined to skin and subcutaneous tissue

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4
Q

4 components of systemic sclerosis (SSC)

A
  • sclerodactyly
  • raynaud’s phenomenon
  • internal organ involvement
  • positive ANA
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5
Q

sex & ethnicity that scleroderma is most prevalent in

A

Females & african americans

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6
Q

what are the two arms of the disease? Inflammation leading to ____ and ____.

A

vasculopathy and fibrosis

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7
Q

when is immunosuppressive therapy used in scleroderma?

A

only in cutaneous & pulmonary manifestations
steroids are NOT normal treatments

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8
Q

what category of the disease does this fall under?

  • face and distal to elbows & knees
  • CREST
  • associated w/ centromere antibody
  • risk of pulmonary artery HTN
A

Limited cutaneous systemic sclerosis

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9
Q

what category of the disease does this fall under?

  • distal + proximal regions– neck, arms, trunk & thighs
  • associated w/ scl70 antibody
  • risk of ILD
A

diffuse cutaneous systemic sclerosis

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10
Q

what category of the disease does this fall under?

  • positive antibodies but no skin thickening
  • still has other manifestations
A

sclerosis sine scleroderma

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11
Q

5 examples of fibrosis

A
  • skin thickening/tightneing
  • subcutaneous calcinosis
  • salt & pepper hyperpigmentation
  • GI dysmotility
  • ILD
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12
Q

4 examples of vasculopathy

A
  • cutaneous telangiectasias
  • nailfold capillaroscopy
  • digital ulceration and pitting in raynauds– sign that it is not primary raynauds
  • watermelon stomach– AV malformations
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13
Q

1st line therapy for raynaud’s phenomenon

A

DHP CCBs— nifedipine XR, amlodipine

titrate up

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14
Q

2nd line therapy for raynaud’s

A
  • PPD5 inhibitors– sildenafil
  • alpha blockers– prazosin or ARBs like Lorsartan
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15
Q

tx of acute digital ischemia (2)

A

IV prostaglandins for 3 days
endothelin 1 receptor antagonists

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16
Q

tx for GERD? tx for gastroparesis

A

GERD– PPO +/- H2 blockers
gastroparesis– prokinetic agents (metoclopramide, cisapride) if refractory

17
Q

everyone who gets diagnosed with scleroderma should get what imaging

A

CAT scan to monitor for fibrosis esp w/ ILD (basilar crackles)

18
Q

3 tx of ILD

A

mycophenolate, tocilizumab or cyclophosphamide

19
Q

what is this condition? which category is it seen in? 3 tx options?

SoB w/ signs of right sided HF
normal resp. exam
low DLCO
TTEcho shows elevated pulmonary artery pressures

A

pulmonary artery HTN
seen in CREST/ limited cutaneous sytemic
confirm w/ right heart cath
tx: IV prostaglandins, endothelin receptor antagonists and sildenafil

20
Q

what is this complication? how is it tx?

  • hypertensive emergency d/t RAS activation
  • hemolytic anemia and thrombocytopenia w/ elevated Cr
  • risks includes under 5 yrs since diagnosis, steroid use
  • renal artery stenosis
  • UA shows no infection
A

scleroderma renal crisis
tx: emergent hospitalization and ACE inhibitor!!!

21
Q

5 major idiopathic forms of inflammatory myositis; not a form of connective tissue disease

A
  • polymyositis– w/o skin
  • dermatomysitis– w/ skin
  • antisynthetase
  • necrotizing
  • inclusion body myositis
22
Q

age & racial predominance of dermatomyositis & polymyositis vs inclusion body myositits

A
  • derma & poly: 30 to 50 Black female
  • inclusion body: over 50 male with no racial predominance
23
Q

painless progressive symmetric proximal muscle weakness
elevated CK (2 to 100x)& aldolase
heliotrope rash, gottron’s papules on MCP, PIP, DIP (not in spaces)
V sign, shawl sign

A

dermatophysis

24
Q

painless loss of muscle mass in proximal muscles & flexion of neck and torso
dysphagia can be present
elevated CK (2 to 100x), abnl EMG, positive muscle biopsy

A

polymyositis

25
Q
  • antibody against tRNA (Jo1); positive ANA
    - inflammatory myopathy, ILD, mechanics hands, inflammatory arthritis & raynaud syndrome
A

anti-synthetase syndrome

26
Q
  • painful symmetric muscle weakness
  • extensive necrosis on biopsy
  • typically + anti-HMGCOA or SRP autoantibodies
  • hx of statin use or red rice yeast
A

immune mediated necrotizing myositis

27
Q
  • painless, asymmetric, slowlyprogressive muscle weakness over several yrs
  • proximal + distal
  • absence of cutaneous or pulmonary manifestations
  • may also cause neuropathy
  • increased ESR/CRP, CK (under 10k), increased tissue enzymes
A

inclusion body myositis

28
Q

3 things you could do to work up inclusion body myositis

A
  • electromyography— location & detect inflammatory myopathy
  • nerve conduction study– detect neuropathy
  • MRI– show edema and alt. causes
29
Q

muscle biopsy of dermato/poly vs inclusion body myositis

A
  • dermato/polymyositis: inflammatory infiltrate
  • inclusion: rimmed vacuoles, triangular/angulated cells; in flammatory infiltrate
30
Q

tx of dermato/polymyositis/necrotizing (2)? refractory (1)? skin disease (1)?

A
  • high dose steriods x 3 days
  • methotrexate, azathioprine, mycophenolate
  • IVIG for refractory
  • hydroxychloroquine or IVIG for skin disease
31
Q

tx of inclusion body myositis

A

no curative tx
PT & nutrition support

32
Q

which condition has greatest risk of cancer? when should you suspect it?

A
  • dermatomyositis
  • suspect in anyone not responding to therapy
33
Q

what should you screen for in all people diagnosed with dermato/polymyositis

A

cancer

34
Q

what are the 4 presentations seen in mixed connective tissue dz (MCTD)?

A
  • swollen hands
  • raynauds
  • arthritis
  • myositis

MIX OF LUPUS, MYOSITIS, SCLERODERMA

35
Q

positive RNP antibody is associated with what condition?

A

MCTD

36
Q

how is MCTD diagnosed

A

presence of mixed fx of CTD (at least 1 feature in 2 or 3 diseases) required

37
Q

what is this? how is it treated?

  • in those with few clinical features seen in CTD
  • most have non-organ threatening features– dry eye, dry mouth, alopecia, ulcers, positive ANA, etc
  • do not fit entirely into a specific CTD
A

undifferentiated CTD
tx: NSAID and hydroxychloroquine

rheumatology (10 decks)

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