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M2F > CT diseases 2 > Flashcards

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
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2
Q

sicca sx’s

A

-dry eyes, xerostomia, vaginal dryness, tracheobronchial dryness, candida oral infection, dental caries, salivary gland enlargement

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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
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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
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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
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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
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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
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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
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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
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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
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11
Q

Dermatomyositis- skin

A
  • shawl sign
  • gottron’s papules
  • heliotrope rash
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12
Q

Polymyositis

A
  • 30-50 yo
  • proximal m weakness
  • no skin changes (vs dermatomyositis)
  • elevated serum CK
  • anti-Jo1
  • m bx- endomysial infl
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13
Q

DM/PM management

A

-corticosteroids!!

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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
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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
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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

M2F (15 decks)

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