CT diseases 2 Flashcards
1
Q
Sjogren syndrome
A
- F>M; mid 50’s
- sicca sx’s (immune mediated dysfxn of lacrimal and salivary glands
- keratoconjunctivitis sicca (foreign body sensation- inadequate tear production)
- strong assoc w B-cell non-Hodgkin lymphoma
- 20-30% have sicca + systemic sx’s
2
Q
sicca sx’s
A
-dry eyes, xerostomia, vaginal dryness, tracheobronchial dryness, candida oral infection, dental caries, salivary gland enlargement
3
Q
keratoconunctivitis sicca
A
- dec lacrimal flow and impaired lacrimal composition (lymphocyte and plasma cell infiltration lacrimal glands)- damaged cornea and conjunctiva epithelia
- Schirmer test- measures quantity of tears secreted
4
Q
Sjogren syndrome- primary or secondary
A
- infl destruction of exocrine glands
- primary= sjogren syndrome alone
- secondary- assoc w another rheumatologic dz/autoimmune dz
5
Q
Sjogren syndrome- dx/serology
A
- lip bx- lymphoid foci in accessory salivary glands; essential for dx!
- +ANA
- +RF
- hypergammaglobulinemia
- Anti-SSA/Ro (newborn complete heart block)
- Anti-SSB/La (never present w/o Ro)
- low C4 complement
- high ESR
- anemia of chronic dz
6
Q
Sjogren syndrome- tx
A
symptomatic!
- artificial tears
- topical ocular cyclosporine
- oral hygiene
- avoid atropinic drugs and decongestants
- no immunomodulatory drug has proved efficaious
7
Q
Inflammatory myopathies
A
- myalgias
- symmetrical b/l prox m weakness- diff rising from chair or climbing stairs!!
- inc CK and aldolase
- ESR and CRP normal
- characteristic m bx
- W>M, Af Ams > caucasians
- dermal features:
- gottron’s patches/papules (DIP, PIP, MCP)
- heliotrope rash
- periungual erythema
- V-neck erythema
8
Q
Dermatomyositis
A
- 7-15 and 30-60 yo
- weakness w/o sensory sxs- proximal m’s early!
- heliotrope rash- periorbital edema, purplish suffusion over eyelids
- shawl sign- erythema over neck/shoulders, upper chest, back
- inc risk of MALIGNANCY
9
Q
Dermatomyositis- dx
A
- Bx- perimysial and perivascular ifl, perivascular atrophy
- elevated CK, aldolase
- Anti Jo-1, anti-MI2, anti-MDA5, anti-PI55/PI40
10
Q
Dermatomyositis- assoc malignancies
A
- Ovarian- TVUS, CT abd/pelvis, CA-125
- lung, pancreatic, stomach, colorectal, NHL
- when dx’ed- look for malignancy- CBC, CMP, SPEP, UA
11
Q
Dermatomyositis- skin
A
- shawl sign
- gottron’s papules
- heliotrope rash
12
Q
Polymyositis
A
- 30-50 yo
- proximal m weakness
- no skin changes (vs dermatomyositis)
- elevated serum CK
- anti-Jo1
- m bx- endomysial infl
13
Q
DM/PM management
A
-corticosteroids!!
14
Q
inclusion body myositis
A
- > 50 yo; M>F, caucasian more common
- finger flexion, quadriceps weakness
- CK is mild elevation or normal
- m bx- endomysial infl, rimmed vacuoles, invasion of non-necrotic m fibers, anti-cN1A autoab’s
- tx- supportive
15
Q
Proximal m weakness- DDx
A
- infl myopathies
- hypothyroidism, hyperthyroidism
- cushing dz
- polymyalgia rheumatic- pain, NO weakness
- neurological- MS, MG, ALS, lambert-eaton
- vasculitides
- drugs/meds
16
Q
Systemic vasculitides
A
- takayasu arteritis
- Behcet syndrome
- polyarteritis nodosa
- kawasaki dz
- granulomatosis w polyangitis (WG)
- eosinophilic granulomatosis w polyangiitis (Churg-strauss)
- thromboangiitis obliterans (buerger)
17
Q
Takayasu arteritis
A
- large vessel (aorta and branches)- subclavian, innominate
- age < 40, W>M, ASIA
- chronic- relapsing, remitting
- long smooth tapered stenosis
- pulseless dz- UE pulses
- 50% pulm involvement
- retinopathy, renal a, stenosis, aortic dilations, aortic regurgitation, aneurysm, aortic rupture
- dx- MRI or CT angiography
- histo- granuloma w some giant cells
- tx- glucocorticoids
18
Q
IgAV aka Henoch Schonlein Purpura (HSP)
A
- palpable purpura!!!
- no thrombocytopenia!!!
- arthritis, abd pain, glomerulonephritis
- any age, more common in kids
- bx- IgA deposits
- tx- supportive/glucocorticoids
19
Q
Anti-GBM
A
- glomerular and pulm capillaries
- anti-basement membrane autoab’s in basement membrane!!!
20
Q
Behcet syndrome
A
- variable vessel size
- silk route- Turkey, asia, mid east
- HLA-B51
- triad- recurrent mouth ulcers, genital ulcers, uveitis!!!!
- large vessel= aneurysms
- venous involvement= DVT
- pathergy- pustules at site of sterile needle pricks
- large joint arthralgia
- neuro- mimics MS!!
- ulcers in distal ileum or cecum
- tx- low dose glucocorticoids
21
Q
Polyarteritis nodosa
A
- medium vessel
- assoc w HBV
- M>F
- fever, malaise, wt loss
- skin
- peripheral n- 80% have vasculitis neuropathy!!-foot drop!
- GI- intestinal angina
- renal
- cardiac
- lungs are spared
- bx- fibrinoid necrosis, infiltration and destruction of BVs by infl cells, NO granulomas
- angiogram- microaneurysm
- ANCA neg
- tx- corticosteroids
22
Q
Kawasaki dz
A
- medium vessel
- <5 yo (asian ancestry)
- mucocutaneous LN syndrome
- strawberry tongue
- ddx- toxic shock syndrome, scarlet fever
- death- coronary involvement (aneurysm or MI)
- tx- IVIG within 10 days of sxs and high dose ASA
23
Q
GPA (granulomatosis w polyangiitis: aka Wegener’s Granulomatosis)
A
- small vessel; M>F
- ANCA+- C-ANKA (PR3-ANCA)
- resp tract- saddle nose!!, crusting/bleeding/obstruction
- CXR- cavitary lesions
- kidney involvement
- hallmarks- granulomatous infl, necrotizing vasculitis, segmental GN
- tx- cyclophosphamide and high dose corticosteroids or rituximab
24
Q
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
A
- ANCA + (<50% of pts!)- MPO-ANCA
- granulomas (w eosinophilia)
- hallmarks- asthma + eosinophilia–> vasculitis
- prodromal phase- allergic dz (asthma)
- eosinophilia-tissue infiltration phase
- vasculitis phase- systemic necrotizing heart/lungs/n’s/skin
- ddx- GPA
- tx- glucocortiocids
25
Q
Thromboangiitis obliterans (Buerger dz)
A
- medium vessel
- Young males (<35yo)!!- only in smokers
- distal vessels 1st
- thrombosis- loss of digits- hands/feet
- dx- angiography- corkscrew appearance
- tx- STOP SMOKING
26
Q
Raynaud
A
- episodic, rarely thumbs
- pallor (vasoconstriction)-> cyanosis (ischemia)-> erythema (reperfusion)
- nailfold capillaroscopy- normal in primary; secondary = distorted w widened and irregular loops, dilated lumen and areas of vascular dropout
27
Q
Primary Raynaud
A
- benign, symmetric!
- cold or emotion response
- W>M, ages 15-30
28
Q
Secondary Raynaud
A
- occurs in CTD, hematologic, endocrine conditions, B blockers or cancer drugs cisplatin and bleomycin
- > 30 yo
- unilateral
- More severe- ischemia!!
29
Q
raynaud- tx
A
- wear gloves when T outside is low
- lotions
- stop smoking
- limit use or stop sympathomimetic drugs
- ca channel blockers
- sx
30
Q
Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA)
A
- W>M, >40-50, white>Af am
- freq co-exist
- constitutional sx- fever, malaise, wt loss
- normal WBC count
31
Q
Giant cell arteritis (GCA) aka temporal arteritis
A
- large vessel- cranial a’s (temporal/facial/ophthalmic), aortic arch!!!
- HA, jaw claudication, visual abnormalities (amaurosis fugax, diplopia), inc ESR (>50)
- HLA-DR4
- assoc w PMR!!
- temporal a bx!!- gold standard- need 1.0 cm segment!!- segmental granulomatous vasculitis w multinucleated giant cells
- start corticosteroids b/f bx!!!!
- no tx- blindness!
32
Q
Polymyalgia Rheumatica
A
- assoc w GCA!!
- proximal severe, symmetrical morning and daylong stiffness, soreness, and pain in shoulder, neck, pelvic girdles
- pain- no true weakness- trouble combing hair, putting on coat
- no infl on m bx
- m enzymes and EMG normal
- tx- corticosteroids
