CT Diseases Flashcards
slicc classification criteria for sle
more/= 4 criteria (at least 1 clinical and 1 lab) OR biopsy proven sle with positive ana/anti-dna
for long standing disease
2019 acr eular criteria
ana >/= 1:80 on HEp-2 cell or equivalent positive test + additive crtieria (>/= 10 points)
clinical domain in acr
SOAP BRAIN MD
serositits oral ulcer arthritic photosensitivity blood disorders renal disease ana immunophenomenon neurological disorders malar rish discoid rash
immunologic criteria in acr
ana, anti-dsdna, anti-smith, anti-i1rnp, anti-ro/ss-a, anti-la/ss-b, anti-histone, anti-phospholipid
diagnostics for sle
read
all patients with sle should be started on __
hydroxychloroquine
other treatments for sle
- corticosteroids
- iv methylprednisolone pulses
- oral prednisone
- plasmapheresis ivig
what is apas
autoantibody-mediated acquired thrombophilia characterized by recurrent arterial or venous thrombosis and/or pregnancy morbidity
criteria for apas
- venous/arterial thrombosis
- ob complications
- lupus anticoagulant
- anticardiolipin antibodies
- anti-b2 gylcoprotein -i antibodies
at least 1 clinical and 1 lab
treatment of apas
- first event: warfarin (2.5-3.5 inr)
- pregnant: low molecular weight heparin with aspirin
what is scleroderma
excessive production and accumulation of collagen (fibrosis) in skin and internal organs and injuries to small arteries
clinical presentation of scleroderma
- mucocutaneous telangiectasia
- raynaud’s digital ischemic ulcers
- pulmonary hypertension
- pericarditis
- skin induration
- calcinosis cutis
- hyperpigmentation
diagnosis of scleroderma
read
types of scleroderma
- limited cutaneous: indolent onset, limited, slow, anticentromere
- diffuse cutaneous: rapid onset, diffuse, antitopoisomerase I, anti rna polymerase III
hallmarks of scleroderma
- widespread capillary loss
- obliterative micoangiopathy
- combined with fibrosis in the skin and internal organs
diagnosis for scleroderma
- cbc
- cpk
- anas
- antiscl 70, anti centromere ab
- skin biopsy
treatment of scleroderma
- vascular therapy (arb, clopidogrel, prostacyclin, sildenafil, bosentan)
- immunosuppressive agents (methotrexate, aza, mycophenolate mofetil and cyclophosphamide)
- hydroxycholoroquine
- low dose prednisone
clinical presentation of sjorgen
- ocular symptoms
- dry mouth
- ear
- nose and paranasal sinuses
criteria for sjorgen syndrome
- histopath: focal lymphocytic sialoadenitis
- (+) anti-ro/ss-a
- sicca ocular score >/= 5
- schirmer test = 5 mm per 5 min
- unstimulated whole salivary flow =0.1 ml/min
+ = more/= 4 and symptoms of ocular/oral dryness
pathogenesis of sjorgen
- mutation of hla-dr and hla-dq
- environmental factors
- b-lymphocyte activation = exocrine gland dysfunction and destruction
diagnosis of sjorgen
- esr and cbc
- anas
- rheumatoid factor
- serum igg
- creatinine clearance
treatment of sjorgen
- dry eyes: artificial tears with cyclic amp or cyclosporine
- dry mouth: inc water
- parotid gland: antibiotics, assess for lymphoma
- arthritis: hydroxychloroquine/ methrotrexate + low dose prednisolone
- raynaud’s: vascular therapy or vasodilators
- vasculitis: steroid / immunosuppression
what is dermatomyositis
- symmetric, proximal greater than distal weakness
- heliotrope rash
- gottron’s sign
- gottron’s papules
- v-sign
- shawl sign
- telangiectasia
- sc ca deposits
antibodies and labs for dermatomyositis
- mda5, tif1, mi2, nxp2
- increased muscle enzymes
- emg: inflammatory myopathy
- biopsy
what is immune mediated necrotizing myopathy
- acute or insidious onset of symmetric, proximal more than distal weakness
- dysphagia, dysarthria, myalgia
labs for immune mediated necrotizing myopathy
- anti hmgcr, anti signal recognition particles
- elevated ck
- emg abnormal
- biopsy: multifocal, necrotic and regenerating muscle fibers with paucity of inflammatory cells
what is anti-synthetase syndrome
- anti aminoacyl rna synthetases
- anti jo1
- elevated ck
- perimysial fragmentation
what is inclusion body myositis
- slowly progressive weakness and muscle atrophy
- distal muscles
- asymmetric
- dysphagia
- cn-1a
- increased cd8
- biopsy: endomyismal inflammatory infiltrates
treatment for inflammatory myopathies
- steroids
- methylprednisolone IV
- ivig or rituximab
large and medium vessel vasculitis
large:
- takayasu arteritis
- giant cell arteritis
medium:
- polyarteritis nodosa
- kawasaki disease
small vessel vasculitis
- immune: hsp, cryoglobulinemic vasculitis, hypocomplementemic urticaria vasculitis
- anca: granuloma with polyangiitis, eosinophilic granulomatosis with polyangitis, mpa
what is granulomatosis with polyangiitis (wegener’s)
- upper and lower respi tracts with glomerulonephritis
- histo: necrotizing vasculitis of small arteries and veins, granuloma formation
diagnosis of wegener’s
- upper airway biopsy!!: granulomatous inflammation with necrosis
- kidney biopsy: pauci-immune gn
- immuno: antiproteinase 3 anca!
what is eosinophilic granulomatosis with polyangiitis (churg-strauss)
- lung predominant
- histo: granulomatous retraction with eosinophils
- s/sx: pulmo findings, mononeuritis multiplex peripheral neuropathy, allergic rhinitis and sinusitis
diagnosis of churg-strauss
- labs: eosinophilis (>1000), elevated esr and fibrinogen, anti-myeloperoxidase (mpo anca)
- 4/6: eosinophilia, asthma, pulmo infiltrated non-fixed, neuropathy, paranasal sinus abnormalities, extravascular eosinophils
what is microscopic polyangitis
- necrotizing vasculitis with few or no immune complexes affecting small vessels
- no granulomatous inflammation
- biopsy: necrotizing inflammation, no granulomas or eosinophils
- myeloperoxidase p-anca
treatment for anca vasculitis
- non organ threatening: methotrexate or mycophenolate mofetil with glucocorticoid
- organ or life threatening: cyclophosphamide or rituximab with glucocorticoid
- rapidly progressive renal failure of pulmo hemorrhage: plasmapheresis
what is polyarteritis nodosa
- necrotizing inflammation of small and medium sized muscular arteries
- associated with hepatitis b
- aneurysmal dilatation (upto 1 cm) are characteristic
lab tests for polyarteritis nodosa
- biopsy
- arteriogram: aneurysm
criteria for pan
3/10
- weight loss >/= 4 kg
- livedo reticularis
- testicular pain
- myalgia or leg weakness
- mono/poly neuropathy
- dbp > 90
- bun or crea elevated
- hepbsag or ab
- arteriogram with aneurym
- pmn in biopsy
treatment for polyarteritis nodosa
- treat underlying cause
- no major organ involvement: glucocorticoid +/- immunosuppressant
- organ or life threatening, >5 ffs, rapid progression: glucocorticoid + immunosuppressant
what is giant cell arteritis / temporal arteritis
- panarteritis with inflammatory mononuclear cell infiltrates within the vessel wall with giant cell formation
- proliferation of intima
- fragmentation of internal elastic lamina
- classic sx: fever + anemia + inc esr
classification of gca
more/= 3:
- > 50 yo
- new onset headache
- temporal artery abnormality
- elevated esr >/= 50 mm/h
- abnormal tab
treatment for gca
- prednisone
- visual loss: methylprednisolone pulsing
- low dose aspirin
- dmards: methotrexate, azathioprine
- tocilizuzmab
what is takayasu’s arteritis
- inflammatory stenotic at aortic arch an branches
- absent pulses, hpn
- labs: inc esr, mild anemia, inc immunoglobulin
criteria for takayasu’s arteritis
3/6
- = 40 yo
- claudication of extremities
- dec pulsation
- <10 mmhg sbp
- bruit at subclavian arteries
- narrowing or occlusion of entire aorta
treatment for takayasu’s arteritis
- corticosteroids +/- methrotrexate, leflunomide, mycophenolate mofetil, azathioprine, tocilizumab
what is iga vasculitis (henoch schonlein purpura)
- anaphylactoid purpura
- immune complex deposition (iga)
- common with strep infection (aso titers)
- can cause intussusception
criteria for iga vasculitis
mandatory: purpura or petechiae with lower limb predominance
1/4:
- skin biopsy: leukocytoclastic vasculitis / kidney biopsy: proliferative gn + iga deposits
- diffuse abdominal pain with acute onset
- arthritis or arthralgia
- proteinuria / hematuria
tx for iga vasculitis
- self-limiting
- oral corticosteroid
- cyclophosphamide
what is behcet’s syndrome
- recurrent oral and genital ulceration + ocular involvement
- systemic perivasculitis with early neutrophil infiltration and endothelial swelling
- pseudoaneurysms
criteria for behcet’s syndrome
recurrent oral ulceration + 2 of:
- recurrent genital ulcers
- ocular lesions
- skin lesions
- positive pathergy test
tx for behcet’s syndrome
- thalidomide
- apremilast
- colchicine
- systemic glucocorticoid (prednisone)
- azathioprine or cyclosporine
