DSA Malaise and Generalized Pain Flashcards
What does a nucleolar staining pattern mean?
Ab directed agains RNA
seen in SLE and diffuse systemic sclerosis
Ppl with scleroderma are at an increased risk of what type of cancer?
bronchoalveolar carcinoma
what are the two phases of Diffuse cutaneous systemic sclerosis?
inflammatory edematous phase –> fibrotic phase
How do you diagnose giant cell arteritis?
temporal a bx (need 1 cm segment bc granulomas are segmental)
What procedure is essential for diagnosis of sjogren’s?
lip biopsy
will see lymphoid foci in salivary glands
What drugs should ppl with sjogren’s avoid?
atropinic drugs and decongestants
What endocrine dz is a complication of scleroderma?
hypothyroid from thyroid fibrosis
What is discoid lupus erythematosus?
variant of cutaneous lupus
can be seen alone or as a manifestation of SLE
most commonly on the head
inflammatory plaques that evolve into gross scars
What is the HLA association to behcet syndrome?
HLA-B51
What labs do you see in polymyositis?
elevated CK
Anti- Jo 1 serology
What serology is positive in Wegener’s
C-ANCA
What is the serology for sjogren’s?
+ ana and RF
high ESR
hypergammaglobulinemia
+ anti SSA/Ro and anti SSB/La
What is takayasu arteritis?
large vessels
< 40 yrs, W>M
long smooth tapered stenosis
pulseless dz
can have renal A stenosis and HTN
aortic dilatations and aneurysms
granulomatous w/ some giant cells
tx: steroids
What does a speckled nuclear staining pattern mean?
anti Sm, Ro and La, Wcl-70, Jo and Mi
seen in:
Sjogren’s
Mixed CT Dz
diffuse systemic sclerosis
SLE
What is the first line tx for dermatomyositis and polymyositis?
corticosteroids (do NOT use in inclusion body myositis)
what msk pathologies can you see in scleroderma?
carpal tunnel syndrome
tendon friction rubs
fibrosis and adhesion of tendon sheaths
What is goodpasture’s syn?
anti-basiment membrane autoantibodies in kidney and lung capillaries
What is the serology specific to diffuse and limited SSc?
diffuse: anti-Scl 70 aka anti DNA topoisomerase I; anti-RNA polymerase III
limited: anti-centromere
What do you see in CREST syndrome/lcSSc
Cutaneous calcinosis
Raynaud’s
Esophageal dysmotility, GERD
Sclerodactaly (thickening and tightening of skin on fingers and hands)
Ttelangiectasia
*Anti-centromere Abs
*vascular probs more common than w/ dcssc, get pulmonary A Htn
What labs do you see in dermatomyositis?
elevated CK and aldolase
anti Jo and Anti Mi Abs
What does a centromere nucleus staining pattern mean?
seen in CREST
What CT autoimmune disorder has a strong association with B cell non-hodgkin lymphoma (MALT lymphoma)?
sjogren syndrome
What msk dz is associated with giant cell arteritis?
polymyalgia rheumatica
How do you treat inclusion body myositis?
refractory to steroids, so tx is supportive
What do you see in sjogren’s?
sicca sx = dry mouth, eyes, etc
keratoconjuctivitis sicca = foreign body sensation in eyes bc of low tear production
What are the 3 types of antiphospholipid antibodies?
type 1 - causes false-positive tests for syphilis
type 2 - lupus anticoagulant
type 3 - anti-cardiolipin antibodies, directed at Beta2GPI
What do you see on bx of polyarteritis nodosa?
fibrinoid necrosis, NO granulomas
What does a renal crisis in scleroderma look like?
abrupt onset of malignant htn, hemolytic anemia, and progressive renal insufficiency
more common in diffuse SSc
*high dose steroids can induce a crisis
What is an MSK complication of SLE managed with corticosteroids?
avascular necrosis of bone
(hips and knees most commonly)
What is the significance of the ration of ANA?
titer of < 1:40 is normal/negative
higher the ration = more clinically significant
What does eosinophilic granulomatosis w/ plyangiitis/Churg-strauss look like?
asthma + eosinophilia –> vasculitis w/ granulomas
*no nose involvement (vs wegener’s)
palpable purpura
How do you treat antiphospholipid syndrome?
anticoagulation
continue indefinitely
What is significant SLE serology?
+ ANA
+ anti-ds DNA (correlate w/ dz activity)
+ Smith
decreased C3 or C4
What is IgAV aka henoch-scholein purpura?
IgA depositis in SMALL vessels
usually in kids
tetrad: palpable purpura (no thrombocytopenia), arthralgia, abd pain, renal dz
What is buerger dz/thromboangiitis obliterans
smoker –> vasculitis in fingers and then fingers turn black
tx = Stop smoking
What do you see in polymositis?
proximal m weakness
NO skin changes like DM
endomysial inflamation on bx
What do you see in inclusion body myositis?
weakness in finger flexion or quads
rimmed vacuoles on bx
CK mild elevation or normal
anti-cN1A Abs
What is the feared association with dermatomyositis?
assoc with malignancy
Ovarian most common
What are the internal organ involvements in dcSSc?
renal crisis w/ hemolytic anemia on labs
interstitial lung dz, PAH
How do you tx kawasaki dz?
IVIg and aspirin
What is the first line treatment for SLE?
avoid sun exposure
NSAIDs
corticosteroids
hydroxychloroquine
What is neonatal lupus and what is its feared complication?
affects kids of mothers w/ anti Ro or La Abs
can cause permanent complete heart block
How do you treat discoid lupus?
photoprotection + topical anti-inflammatory agents or systemic antimalarial drugs
(treat early to minimize scarring)
What serology is sometimes positive in eosinophilic granulomatosis w/ polyangiitis?
MPO-ANCA
How do sulfa drugs affect SLE?
induce a lupus flair (doesn’t cause lupus-like syndrome)
How do you treat IgA vasculitis?
supportive/steroids
What are the 3 types of scleroderma?
diffuse (dcSSc)
limited (lcSSc)/ CREST
localized
What is a homogeneous staining pattern indicative of?
(entire nucleus is diffusely stained)
anti DNA, histone, or DNP
drug-induced lupus
sjogren’s syndrome
SLE
What vasculitis is assoc w/ HBV?
polyarteritis nodosa
Why do ppl with scleroderma have dry, itchy skin?
there is obliteration of eccrine sweat and sebaceous glands
What are the main causes of death early and late in SLE?
early: infections, kidney, or CNS dz
Late: accelerated athersclerosis and MI, Thromboembolic events
How do you dx raynaud’s?
nailfold capillaroscopy:
normal in primary
secondary = distorted w/ widened and irregular loops, dilated lumen and areas of vascular dropout
What do you see in dermatomyositis?
gottron’s papules
heliotrope rash
periungual erythema
shawl-shaped erythema
perimysial and perivascular inflammation
perifascicular atrophy
What type of hypersensitivity is SLE?
type 3
(deposition of antigen-Ab complexes –> complement and leukocyte recruitment)
What is the triad of sx of behcet syndrome?
recurrent mouth ulcers
genital ulcers
eye inflammation
What is localized scleroderma?
benign skin condition most often in kids
discreet areas of discolored skin induration
histologically indistinguishable from SSc
patches = morphea
What drugs can cause a lupus-like syndrome?
hydralazine
Isoniazid
minocycline
TNF inhibitors
quinidine
chlorpromazine
methyldopa
procainamide
What gastric pathology is specific to Scleroderma?
gastric antral vascular ectasia (GAVE)
aka watermelon stomach
What is kawasaki dz?
medium vessel vasculitis
asian kid w/ fever, LAD, rash, strawberry tongue
can die from coronary aneurysm years later
What does granulomatosis w/ polyangiitis/wegener’s look like?
granulomatous inflammation and necrotizing vasculitis
*nasal involvement + eyes, kidneys, lungs, and skin
What are the main causes of death in the systemic scleroses (3)?
aspiration pneumonia bc of GERD
diffuse = interstitial lung dz (dry cough, velcro crackles, dx by PFT)
limited = Pulmonary A htn
Why do SLE pts have an increased risk of MI?
have accelerated atherosclerosis
What serology do you see in a lupus-like syndrome/drug-induced lupus?
+ ANA
+ anti-histone Abs (95%, diffuse staining pattern)
What can discoid lupus be mistaken for?
tinea infection (ring worm)
psoriasis
morphea (limited scleroderma)
Which type of raynaud’s is more severe?
secondary, get ischemia
What is libman-sacks endocarditis?
seen in SLE
vegetations are microthrombi
What autoimmune dz can cause cotton wool spots on retina exam?
SLE/ antiphospholipid syn retinopathy
What is the hallmark of scleroderma (SSc)?
thickening and hardening of the skin
micrangiopathy and fibrosis of skin and viscerlal organs
