Scleroderma Flashcards
two things frequently seen in connective tissue diseases
raynaud’s phenomenon
non-erosie inflammatory arthritis– damaging surrounding structures not the joint itself
what is scleroderma
CTD characterized by sclerodactyly– skin thickening and tightening
- unilateral patches (morphea), en coup de sabre, linear scar tissue w/o injury
- negative antibodies, workup might be normal
localized scleroderma- sclerodactyly confined to skin and subcutaneous tissue
4 components of systemic sclerosis (SSC)
- sclerodactyly
- raynaud’s phenomenon
- internal organ involvement
- positive ANA
sex & ethnicity that scleroderma is most prevalent in
Females & african americans
what are the two arms of the disease? Inflammation leading to ____ and ____.
vasculopathy and fibrosis
when is immunosuppressive therapy used in scleroderma?
only in cutaneous & pulmonary manifestations
steroids are NOT normal treatments
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
Limited cutaneous systemic sclerosis
what category of the disease does this fall under?
- distal + proximal regions– neck, arms, trunk & thighs
- associated w/ scl70 antibody
- risk of ILD
diffuse cutaneous systemic sclerosis
what category of the disease does this fall under?
- positive antibodies but no skin thickening
- still has other manifestations
sclerosis sine scleroderma
5 examples of fibrosis
- skin thickening/tightneing
- subcutaneous calcinosis
- salt & pepper hyperpigmentation
- GI dysmotility
- ILD
4 examples of vasculopathy
- cutaneous telangiectasias
- nailfold capillaroscopy
- digital ulceration and pitting in raynauds– sign that it is not primary raynauds
- watermelon stomach– AV malformations
1st line therapy for raynaud’s phenomenon
DHP CCBs— nifedipine XR, amlodipine
titrate up
2nd line therapy for raynaud’s
- PPD5 inhibitors– sildenafil
- alpha blockers– prazosin or ARBs like Lorsartan
tx of acute digital ischemia (2)
IV prostaglandins for 3 days
endothelin 1 receptor antagonists
tx for GERD? tx for gastroparesis
GERD– PPO +/- H2 blockers
gastroparesis– prokinetic agents (metoclopramide, cisapride) if refractory
everyone who gets diagnosed with scleroderma should get what imaging
CAT scan to monitor for fibrosis esp w/ ILD (basilar crackles)
3 tx of ILD
mycophenolate, tocilizumab or cyclophosphamide
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
pulmonary artery HTN
seen in CREST/ limited cutaneous sytemic
confirm w/ right heart cath
tx: IV prostaglandins, endothelin receptor antagonists and sildenafil
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
scleroderma renal crisis
tx: emergent hospitalization and ACE inhibitor!!!
5 major idiopathic forms of inflammatory myositis; not a form of connective tissue disease
- polymyositis– w/o skin
- dermatomysitis– w/ skin
- antisynthetase
- necrotizing
- inclusion body myositis
age & racial predominance of dermatomyositis & polymyositis vs inclusion body myositits
- derma & poly: 30 to 50 Black female
- inclusion body: over 50 male with no racial predominance
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
dermatophysis
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
polymyositis
- antibody against tRNA (Jo1); positive ANA
- inflammatory myopathy, ILD, mechanics hands, inflammatory arthritis & raynaud syndrome
anti-synthetase syndrome
- painful symmetric muscle weakness
- extensive necrosis on biopsy
- typically + anti-HMGCOA or SRP autoantibodies
- hx of statin use or red rice yeast
immune mediated necrotizing myositis
- 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
inclusion body myositis
3 things you could do to work up inclusion body myositis
- electromyography— location & detect inflammatory myopathy
- nerve conduction study– detect neuropathy
- MRI– show edema and alt. causes
muscle biopsy of dermato/poly vs inclusion body myositis
- dermato/polymyositis: inflammatory infiltrate
- inclusion: rimmed vacuoles, triangular/angulated cells; in flammatory infiltrate
tx of dermato/polymyositis/necrotizing (2)? refractory (1)? skin disease (1)?
- high dose steriods x 3 days
- methotrexate, azathioprine, mycophenolate
- IVIG for refractory
- hydroxychloroquine or IVIG for skin disease
tx of inclusion body myositis
no curative tx
PT & nutrition support
which condition has greatest risk of cancer? when should you suspect it?
- dermatomyositis
- suspect in anyone not responding to therapy
what should you screen for in all people diagnosed with dermato/polymyositis
cancer
what are the 4 presentations seen in mixed connective tissue dz (MCTD)?
- swollen hands
- raynauds
- arthritis
- myositis
MIX OF LUPUS, MYOSITIS, SCLERODERMA
positive RNP antibody is associated with what condition?
MCTD
how is MCTD diagnosed
presence of mixed fx of CTD (at least 1 feature in 2 or 3 diseases) required
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
undifferentiated CTD
tx: NSAID and hydroxychloroquine
