Vasculitis Flashcards
Vasculitis
define
general symptoms
Vasculitis
- inflammation of the vasculature/blood vessels
- body perceives these vessel cells as invaders, produces antibodies against these protiens: creates damange to the vessel walls
- in addition, increased WBC increase antibodies, causing a clotting cascaude of fibrinogen & decreases the size of the lumen= ischemia
arteries, veins, small, mediaum and large
Symptoms of all vasculitis
- fatigue/malaise
- fever
- weight loss
- myalgia, arthralgia
- night sweats
- specific symptoms depend on the size and location of teh vessel affected
Vasculitis
Classifcations: Large
Large Vessel Vasculiti s
- narrowing lumen = decreased blood flow = end organ ischemia or infarction
- PE: bruits or thrills due to increased constricted and force trying to make it through
Types
- giant cell arteritis/ PMR
- takayasu arteritis
- behcet’s syndrome
Symptoms
- limb caaudication
- asymmetric BP
- absent pulses
- aortic dilation
Vasculitis: Medium Vessel Classification
Medium Vessel Vasculitis
- inflammation of the blood vessel, so the blood gets backed up flowing through it
- this puts pressure on the vessel prior to the localized inflammation = ballooning outwards = ANYEURYSUM
Types
- Polyarteritis nodosa
- Kawasaki disease
Symptoms
- cutaenous nodules
- ulcers
- livedo reticularis
- digital gangrene
- microanyuresums
Vasculitis: Small Vessel Classification
Small Vessel
- the small vessels are damaged: so they will completely break since they’re like one cell thick = blood leakage
- this leak is within organ system = leading to bleed and inflammation of the organ
ANCA Related
- microscopic polyangiitis
- wegeners
- churg-strauss (eosinophliic)
Immune Complex
- Henoch-Schonlein Purpura
- Cryoglobulinemic Vasculitis
- Anti-Cttq vasculitis
Symptoms
- purpura
- lesions
- hives
- glomerulonephrisis
- alvolar hemrrhage
- splinter hemorrhages
- uveitis
- episclerisis/scleritis
secondary causes of vasculitis to occur
Infection
- menginococcal infection = inflammation of vessels = burst (purpura) or occulde (ischemia)
- stretococcal infection = erythema nodosum
- viral infection Hep B/C too
Underlying Disease
- cancer
- autoimmune diseases: RA, SLE, IBD
Cold Injury
- frost bite
- cryglobulinemia
Drugs
- antithryoid meds
- hydralazine
- minicycline
- anx.
- anticonvusants
Takayasu Arteritis (pulseless disease or aortic arch syndrome)
Etiology
Symptoms
Etiology
- inflammation of the aorta
- rare, femal and young
Symptoms
- systemic: fever, WL, NS, malaise, pain (can be early and go away)
- bruits, absent or reduced pulses
- claudication arms, legs, sping, gut
- ischemic heart disease, HF
- pulmonary HTN
- HA and amaurosis fugux (temp. blind) (NOT stroke)
- BP variability by limbs
- HTN
Takayasu Arteritis (pulseless disease or aortic arch syndrome)
Diagnosis
Treatment
Diagnosis
- imaging angiography: MR or CT
Treatment
surgery or steroids
Giant Cell Arteritis
etiology
symptoms
Etiology
- a disease of the large to medium vessels
- eldery
- systemic pan-arteritis: typically the temporal artery, carotid
- LOTS of pt. on imaging have large vessel inflammation
- realted on spectrum to polymyalgia rheumatica
Symptoms
- temporal HA (severe)
- scalp tenderness (hurts to shower)
- jaw claudication
- throat pain & systemic non-specific symptoms
PE
- can have pulseless, tender, enlarged or noducalr temporal artery
Additional Symptoms
- asymmetric pulses: if aorta involved, regugr, bruits at clavicles
- vision changes!! amerosis fugaux, diplopia, sudden blindness
- confusion, depression, tingling tongue
Giant Cell Arteritis
diagnosis
treatment
Diagnosis
- ESR!!!! GET THE ESR!!!!
- confirmatory with temporal artery biopsy
Treatment
- urgernt administration of high dose steroids: prednisone
- IV methylpred. if there is vision loss
- taper after 1 month & use ESR as aguide
- low dose asprin
Polymyalgia Rheumatica (PMR)
etiology
symptoms
diagnosis
treatment
Etiology
- same spectrum of diseases as giant cell arteritis
- cytokines in the blood/arteries
- older women!!!
Symptoms
- girdle joint pain and stiffness: shoulders and pelvis bilaterally
- wont be WEAK: but will be sore
- systemic symptoms
- can come before the symptoms of ginat cell arteritis
Diagnosis
- can be clinical
- ESR may be high
Treatment
- low-dose steroids: taper after 1 month to a much much lower dose for 1 year
Behcet’s Syndrome
Etiology
Symptoms
classic
arthritis
eyes
CNS
GI
Etiology
- vasculitis in large, medium and small vessels
- assocaited with HLA-b51 gene
Symptoms
- Behcet’s = UCLERS!!
- ulcers in the mough, toungue and cheecks
- gnenital ulcers
- skin lesions: tender erythemadous papular (look like erythemia nodosum) that ulcerate
pathergy: when you needle stick them, they form a pustule at the prick site
Arthritis
- knees and ankles
- no joint erosins: xray looks ok
Eyes
- posterior uveitis (retina)
- anterior uveitis : red eye, photophobia, ** hypophon (WBC!!)**
- treat eye issues with dialtion and steroids
CNS
- sterile meningitis: PL shows lymphcytic pleocytosis
GI
- ulcers in ileum and cecum
Pulmonary
- pulm. artery anyuresum & hemorraage
Hematology
- increase clotting: DVT, PE and clot risk
Behchet’s
Diagnosis
Treatment
Diangosis
- elevated ESR/CRP
- no ab. to show
- HLA B51
- clincial
Treatment
-for mucoccutaneous = colchicine
- for severe symptoms = corticosteroids: prednisone
- for occular/CNS = infliximab, cyclosporines
Polyarteritis Nodosa (PAN)
- etiology (everywhere but)
- symptoms
Etiology
- a medium vessel disease: causing necorsis to the arteries
- impacts everywhere except lungs
- commonly impacts the kidneys
- RISK FACTOR: Hep B (or familial adenosine gene mut.)
Symptoms: depend on what organ is invovled
- general: fever, weigh tlss
- infarction or ischmia to the organ invovled
- kidney: renal artery stenosis, anyureusm or HTN
- brain: stroke
- heart: MI, myocarditis
- live r
- skin: gangrene, purpura, nodules
- PNS: pain in nerves, sensory motor loss
- testes: pain/infarct
Additional Symptoms
- myalgias, neuropathy and arthralgias
- mononueritis multiplex: foot drop
- GI: pain with eating, appendicitis, perfoated bowel, cholecystisi
- Skin: skin ulcers at the malleoli
- gangree, livedo reticularis & nodules
Polyarteritis nodosa (PAN)
diagnosis
treatment
Diagnosis
- tissue biopsy at the site of the effected area (skin, nerve, organ)
- angiography: to see the small anyuresums/dilations in teh arteries
Treatment
- high dose steroids: if criticall ill IV methylpred.
- cyclophosphamide
treat the Hep N with antivirals and plasmapharesis and prednisone
survival good if they get treated
Buerger’s Disease
etiology
symptoms
Buerger’s Disease
Etiolgoy
- vasculitis with inflammation specific to the hands and feet
- thromboangiitis oblierans is the other name
- caused mostly by smoking: triggers autoimmune reaction against healthy tissue
- men, smokes
Symptoms
- pain in hands and feet
- red, enlarged cord veins in hhands
- sores that dont heal
- leg pain, numbeness, tingling, burining
- gangrene if untreated
- usuall 2+ limbs affected
- can see decreased pulses
- worsened sx. with stress or cold expsoure
Buerger’s Disease
Diagnosis
Treatment
Diagnosis
- rule out raymauds!
- angiogram or arteriogram of UE and LE
- doppler US
- skin bx. can be done but they dont heal well so not often done
Treatment
- STOP SMOKING
- no cure: symptomatic
- massage to increased warmth, avoid cold
Henoch-Schonlein Purpura (HSP)
most commony HYPERSENSITIVTY svasculitis
Etiology
Symptoms
Etiology
- hypersensitivity: a leukocytoclasic vasculitis with IgA deposition
- very common in kids
Symptoms
Arthritis
- knees and ankles
Palpabale Purpura
- lower extremities (butt, legs, feet)
Hematuria
- renal invovlement
Abd. Pain
- in kids this is common, vasculitis of teh abd. tract and GI bleeding can occur
Henoch-Schonlein Purpura (HSP)
symptoms as they differ by adults to kids
Kids
- large joint arthritis: knees, ankles
- edema
- purpura on the limbs
- abd. pain
- not the kidneys
Adults
- small joint arthritis: wrists, MCP, PIP, MTP
- no edema or GI invovlement often
- purpura on limbs and torso
- kidney involvement
- often familail
Henoch-Schonlein Purpura (HSP)
Diagnosisi
Treatement
Diagnosis
- kidney Biopsy: see segmental glomerulonephritis with creseant and mesangial deposition of IgA in the BM
Treatment
- adults: this will porsb become CKD and have chronic skin disease
- adults = steroids: prednisone, azathioprine, mycophenolate
Kids: steroids not helpful, symptomatic
Cryoglobulinemia
etiology
symptoms
Etiology
- cold antibodies in the blood
- immune complex mediated: small vessel vasculitis (hemorrahges)
- most commonly assocaited with Hep C
- RH + IgG = complex = vasculitis
Symptoms
- palpabale purpura that reoccur
- periphearl neuropathy
- proliferative glomeularnephritis
- digital gangere
- abd. pain
meltz triad: purpura, arthraliga and weakness
Cryoglobulinemia
diagnosis (TESTS!!!)
Diagnosis
- abnormal LFTs
- + serum cryoglobulins
- + RF
- low C4
- clincial dx. based on situation
Treatment
- antivirals for Hep C
- if severe: steroids + cyclophosphamide OR rituximab
Wegener Granulmatosis
(granulomatosis with polyangiitis)
etiology
Etiology
- C-ANCA: anti-neutrophil cytoplasmic antibody disease
vasculitis of small arteries, arterioles and capillaries
- necrotizing granulomatous: getting lesions of the upper and lower respiratory tracts
- glomerulonephritis
Symptoms
- URI symptoms: sinusitis, OM, etc. : can have ENT deformities like saddle nose from perforated nasal septum
- renal: proteinuria and hematuria
- LRI symptoms: cough, dyspnea, hemoptysis
Additional Symptoms
- migratory arthritis of large joints
- eye: ulilater ptosis, scleritis, uveritis, keratitis
- purpura/skin lesions
- neuropathy/parasthesias
- systemic fever, WL and fatigue
- increased VTE risk
Wegener Granulmatosis
(granulomatosis with polyangiitis)
Diagnosis
Diangosis
- CBC: anemia, leukocytosis, thrombocytosis
- elevated CRP/ESR
- Urine: proteinuria & RBC with RBC casts and mayeb WBC
- C-ANCA autoantibodies : most will have these
-
CT chest: granulomatous disease
- masses, cavities etc. = plueral effusions and hilar adenopathy
Tissu Bx.
lung biopsy is preferred to find granulomaous inflammation
Kideny Bx.
- segemental necrotizing cresents
can get nasal biopsy
Wegener Granulmatosis
(granulomatosis with polyangiitis)
treatment
Treatment
induction therapy
methotrexate = mild
cyclophosphamide + steroids OR rituximab + steroids = severe
Maitenance
- methotrexate (not in renal failure)
- rituximab
- azathioprine
end up on dialysis
Microscopic Polyangitis
etiology
symptoms
Microscopic Polyangitis
Etiolgoy
- P-ANCA
- Pauci-immune nongranulomatous necotrtizing vasculitis
- affects small vessels:capillaries
- glomerulonephritis and pulmonary capillaritis
- rarely = med induced
Symptoms
-purpura
cutaneous vasculitis:ulcers, splinter hemorrhages, vasciulobullous lesions
interstitail lung fiberosis: pulmonary hemorrhage = hempotysis
- mononeuritis multiplex
Microscopic Polyangitis
Diagnosis
Treatment
Diagnosis
- GET A P-ANCA
- Esr/CRp
- RBC casts, proteinuria and hemoturia
- Renal biopsy: to see necotritizng
REMEBER THESE HAVE NO GRANULMOA!! just ruputres capillaires leading to bleeding in skin and kidney and lungs
Treatment
induction
methotrexate = mild
cyclophosphamide, rituximab = severe
maitence
- methotrexate, azathioprine, rituximab
Churg-Strauss Immune Complex
etiology
symptoms
diagnosis
treatemnt
Etiology
- eosinophilic granulomatous with polyangitis
SYmptoms
- think asthma: common in these pt.
- eosinophilia rich granulomas in blood
- in lungs!!!
- peripheral neruopathy
Diagnosis
- blood eosinophila level
- CXR or CT lungs
- Biopsy of lungs!!!
Treatment
- palliative
- steroids or immunosup.
Raynaud’s Phenomenon
etiology
stages/Symptoms
risk factors
Etiology
- primary: unknown just response of the arterioles to colr or stress
- secondary: undelying rheumatic disease (scleroderma MC)
Phases
- well-demarkated excessive vasoconstriction: fingers turn white the blue (spares thumb)
- excessive voasdilation = fingers turn red and burn and thorbbing pain
can be symmetric (unilateral more if its secondary)
if see nail fold chagnes: think scleroderma related
Risks
- smoking
- chemical exposure
- autoimmune conditions
- injury/trauma
- meds
Raynauds Phenomenon
diagnosis
treatement
Diangosis
- ab. to rule out secondary causes
- look at capillaries on nail bed to see if its secondary!!
Treatement
- lifestyle modifications
- mittnes, lotions, avoid vasoconstrictions (decongestants),smoking
- etc.
Medication Treatemnet = nifedipine CCB
can do surgery sympathectomy (cerival or digital)
