Connective Tissue Disease - Darrow Flashcards
autoimmune diseases
linked to HLA antigens
class I - A, B, C class II - D
class I - men class II - female
SLE
in black women
ANA, HLA-B8, DR2 and DR3 positive
seen with complement deficiency
arthralgia, rash on face, lung field rub, proteinuria, anemia
SLE
elevated ESR and normal CRP
in SLE
pericarditis
in SLE
diagnosis of SLE
requires 4 of 11 criteria
criteria for diagnosis of SLE
B3 O1 R1 N1
with
D3ermA1titiS1
B3 - blood
1 - heme disorder - anemia, leukopenia, thrombocytopenia
2 - immunologic disorder - ds-DNA, Sm, APA, FPSTS
3 - ANA
O1
4 - oral ulcers
R1
5 - renal disorders - proteinuria or cellular casts
N1
6 - neurologic disorders - seizure or psychosis
D3
7 - malar rash
8 - discoid lupus
9 - photosensitivity
A1
10 - arthritis
S1
11 - serositis - pleuritis or pericarditis
speckled and rim pattern
in SLE
seizure of psychosis
in SLE
smoking
makes SLE rash worse
cough up blood
alveolar hemorrhage
-associated with SLE
non-infectious endocarditis
libman sacks
-associated with SLE
tx of SLE
hydroxychloroquine
- fight SLE
- stop anti-phospholipid Ab formation
before making diagnosis of SLE in patient
exclude drug induced lupus
splinter hemorrhages
with bacterial endocarditis
drug induced lupus
slow acetylators - if inhibit DNA methylation T cells - become autoreactive
sx with drug induced lupus
no renal disease
no CNS disease
+ anti-histone Abs
drugs triggering autoimmunogenicity
hydralazine isoniazid procainamide methimazole prophylthiouracil etanercept
anti-histone Abs
drug induced lupus
CI in pregnancy
CI in pregnancy
safe in pregnancy
azathioprine and HCQ
SLE vs. preeclampsia
abnormal liver test and high uric acid - preeclampsia
dsDNA, low C3/C4 and normal uric acid and liver tests - SLE
both have HTN, proteinuria, and thrombocytopenia
RO/SSA patient daughter born with
heart block
ring like or snake like plaque with central clearing
SCSLE - subacute cutaneous lupus erythematosis
-characteristic rash
RO-SSA and heart block in offspring
lupus anticoagulant
worst prognosis
venous and arterial clotting
antiphospholipid antibody syndrome
diagnosis of antiphospholipid antibody syndrome
1 clinical and 1 lab criteria - 3 months apart
lab
- anticardiolipid Ab
- lupus anticoagulant
- anti-beta2 glycoprotein 1
clinical
- spontaneous abortion
- vascular abortion
best correlation with thrombotic events in antiphospholipid Ab syndorme
lupus anticoagulant
russel viper venom test
confirmation of antiphospholipid Ab syndrome
tx with hydroxychloroquine
need to do retinal screening
small vessel vasculitis
cryoglobulinemia
giraffe type rash - livedo reticularis
cause of livedo reticularis
cryoglobulinemia
tightening skin and fibrosis
scleroderma
progressive systemic sclerosis
scleroderma
-systemic
onion skinning of renal arteries
scleroderma
GI sx of scleroderma
stay below sternum
fibroblast metabolic defect with increased collagen
scleroderma
autoimmunity
can be caused by silica**
diagnosis of scleroderma
1 major or 2 of 3 minor
major - thick, tight, induration of skin proximal to MCP
minor
- sclerodactyly
- digital pitting
- bibasilar pulmonary fibrosis
high probability of scleroderma
with 3 of 5 crest featuresq
first sign of scleroderma
raynauds
signs of scleroderma
raynauds nonpitting edema subQ calcifications finger tip ulcerations telangiectasia
tendon friction rub
scleroderma
raynauds
female
-precipitated with cold, stress, drugs
finger loss
anti-centromere
digital ulcer
anti-topoisomerase
<30yo, normal sed rate, no nail bed capillary changes
primary raynauds
visceral changes in scleroderma
linked to extent of skin thickening
what kills you in scleroderma
kidney failure with HTN
-until ACE inhibitors
now - lung issues kill you
anti-RNA polymerase III
bad Ab in scleroderma
onion skinning - renal disease**
stop progression of scleroderma
ACE inhibitors
anit-SCL-70
scleroderma interstitial lung disease**
scleroderma risk fx
AA
anti-centromere Ab and anti-B23
scleroderma pulmonary HTN
dysphagia cause
scleroderma
for solids and liquids
limited scleroderma
CREST syndrome
induces fibrosing dermopathy
gadolinum
prep for X-ray or CAT scan
trauma, following exercise, M protein
eosinophilic fasciitis
shulmans syndrome
eosinophilic fasciitis
plantar fascitia and polyarthritis syndrome
associated with malignancy**
is a paraneoplastic syndrome
fibroblast proliferation due to TGF-B
pain, stiff, swollen DIP and PIP thick palmar fascia thick plantar fascia ANA negative no RP
polymyositis and dermatomyositis
proximal muscle weakness
inclusion body myositis
distal muscle weakness
dermatomyositis
CD4 - B cells
polymyositis and inclusion body myositis
CD8 T cells
very high CPK
dermatomyositis
heliotrope rash, shawl sign, gottrons papules, nailfold capillary loop
dermatomyositis
scleroderma dysphagia
low in esophagus - smooth m
dermatomyositis dysphagia
high in esophagus - skeletal m
specific diagnosis of dermatomyositis
perivascular infiltrate with cap necrosis and fiber destruction
shawl sign
in dermatomyositis
association with malignancy**
anti-Jo1 dermatomyositis
lung involvement**
anti-p155/140
associated with cancer
also shawl sign
anti-U1 RNP
mixed connective tissue disease
-aka overlap syndrome
pt on ventilator, sepsis forms, high blucose, flaccid distal and proximal muscle groups, absent DTRs
critical illness myopathy neuropathy
sjogrens
T cell CD4 - infiltrate gland
anti-Ro and anti-La produced by B cells
cryoglobulins
small vessel vasculitis in sjogrens
Ab to type II collagen
relapsing polychondritis
polyarthritis across wrist and MCPs, also eye and ear involvement
IgG4 releated disease
relapsing polychondritis
obliterative phlebitis
inflammation of well of vein that lumen obstructed
in relapsing polychondritis
IgG4 related disease
large joint arthritis, night sweats, anorexia, diarrhea, weight loss, lymphadenopathy, hepatosplenomegaly, hyperpigmentation
whipples disease
stages of whipples
prodrome - migratory arthritis
steady state - diarrhea and weight loss
PAS positive macrophages
whipples disease
hyperpigmentation
niacin decrease
aka pellagra
