Vasculitis Flashcards
Half of this is also in nephro-glomerulonephritis includes GCA, IGA, and other vasculitis
Classic triad of IgA vasculitis
purpura (rash on abdomen, legs), abdominal pain, and arthalgia
Definitive: Diagnosis of IgA vasculitis
Biopsy of skin with immunofluorescence
Henoch Schonlein purpura AKA
IgA vasculitis
happens after URI or respiratory infections
IgA vasculitis is a (small/medium/large) vessel dx?
small vessel - affects skin, bowel (leading to pain, bleeding, intussusception, less commonly affects kidneys and rarely lungs)
General diagnosis of IgA vasculitis
clinical picture of adults and kids. Can get biopsy
Don’t get IgA levels. not sensitive or specific.
long term prognosis of Kawasaki Dx
persistent coronary artery aneurysms coveys greatest risk for CAD in pts who had Kawasaki dx as a kid
Kawasaki Dx presentation
fever, rash, cervical LAD, prominent nonexudative conjunctivitis, oral mucosal and lip changes.
How to reduce Kawasaki coronary artery aneurysm?
IVIG but 10-20% don’t respond.
Surveillance of Kawasaki dx and coronary artery aneurysm
may need regular EKG, ECHO, and/or coronary artery imaginge
most common idiopathic glomerulonephritis in developed countries?
IgA nephropathy
What affects adverse prognosis of IgA nephropathy?
elevated BP and lower than normal GFR.
Who gets biopsies with suspected IgA nephropathy?
if protein excretion is >500 mg/day, elevated serum creatinine >1.5 and HTN.
what establishes glomerular dx as being the cause of patient’s hematuria?
significant proteinuria, and dysmorphic red blood cells on urinalysis.
anti basement membrane antibody is seen with
good pastures syndrome. Can see hematuria recurrently and see significant pulmonary hemorrhage
Giant cell arteritis presentation
constitutional symptoms, headache, transient vision loss ESR.
Monocular vision loss and fundoscopic examination typically normal or signs of ischemia
essential mixed (type 2) cryoglobulinemia
lymphoproliferative disorder with immune complex deposition in small to medium sized blood vessels.
presentation of cryoglobulinemia
palpable purpura, arthralgias, membranoproliferative glomerulonephritis, low serum complement, cutaneous vasculitis and neuropathy.
Purpura is seen in legs
vasculitis may show skin ulceration and necrosis
diagnosis of cryoglobulinemia
serum cryoglobulins
also check for hep C (>90% of cryoglobulinemia also are positive for hep C)
see low complement levels and falsely positive RF and ANA
usually will have history of IVDA
churg strauss syndrome has 3 stages
prodromal - asthma allergic rhinitis, or atopic dermatitis
eosinophilic phase - peripheral eosinophilia, lung eosinophilia with infiltrates, and a
vasculitic phase (polyangiitis) up to 10 years later.
how does hepatitis C cause false neg RF and ANA for cyroglobulinemia?
hep c causes non specific stimulation of lymphocytes resulting in excess production of immunoglobulin - can cause false positives for ANA and RF.
criteria for diagnosis of GCA
age >50 yrs
new onset localized headache with fever and visual disturbances
ESR>50
tenderness or decreased pulse of temporal artery
temporal artery biopsy showing necrotizing arteritis with mainly mononuclear cells.
3 out of 5 above criteria is 94% sensitive and 91% specific for GCA
Gold standard for diagnosis of GCA
temporal artery biopsy.
negative biopsy results are common because this is patchy disease
Thus we need second biopsy or can treat clinically regardless
management of GCA after achieving initial remission
keep patient on high dose steroids and slowly taperover a few months. Some pts may need 1-2 yrs of steroids.
Premature discontinuation may have high risk of relapse with GCA
Add aspirin to prevent long term complications of GCA (TIA and stroke)
Needs surveillance for aortic dissection long term with CXR for 10 years.
DEXA scan for osteoporosis
long term complications of GCA and how to prevent them?
TIA and stroke
add aspirin
Henoch Schonlein purpura is tetrad of
IgA vasculitis
abdominal pain, arthritis, and glomerulonephritis and palpable purpura on lower extremities
skin involvement (96%) of pts with HSP (IgA vasculitis)
ecchymoses, petechiae and palpable purpura
MSK involvement (60% of pts) in HSP
arthritis, arthralgia, involving 1-4 large joints (hips knees and ankles)
GI involvement in HSP (50%)
nausea, vomiting, abdominal pain and GI hemorrhage
renal involvement (33%) in HSP
hematuria, proteinuria, and renal insufficiency
Biopsy of palpable purpura of HSP will show
IgA deposition and HSP is immune mediated vasculitis
Rash associated with HSP (IgA vasculitis)
leukocytoclastic vasculitis with lots of IgA immune complexes within affected organs.
prodromal phase of Churg Strauss (seen in 20-30yrs)
atopic dx with: asthma allergic rhinitis and recurrent sinusitis
eosinophilic phase or Churg Strauss syndrome
eosinophilic infiltration of multiple organs
migratory pulmonary opacities
>10% peripheral eosinophilia
eosinophilia gastroenteritis
Vasculitis phase seen in 3 or 4th decade of life of Churg Strauss
life threatening systemic vasculitis- see tender subcutaneous nodules on extensor surfaces of arms (Granulomas)
heart failure and or conduction defects
peripheral neuropathy (mononeuritis multiplex)
necrotizing glomerulonephritis (usually ANCA positive)
migratory polyarthritis
what is allergic granulomatosis and angiitis
Churg Strauss syndrome
in the Allergic granulomatosis angiitis vasculitis phase what blood vessels are affected?
medium and small vessels (kidney)
presentation of someone with difficult to control asthma and >10% peripheral eosinophilia and mono or polyneuropathy and transient pulmonary opacities and sinus disease
presentation of allergic granulomatosis angiitis or Churg Strauss syndrome
what do you see if biopsy a skin rash of Churg Strauss dx?
see eosinophilic vasculitis
diagnosis of allergic granulomatosis angiitis vasculitis
suspected in 4 or more of the following:
asthma and >10% peripheral eosinophilia and mono or polyneuropathy and transient pulmonary opacities and sinus disease and biopsy showing eosinophilic vasculitis
treatment of granulomatosis angiitis vasculitis and Churg Strauss
IV steroids and sometimes cyclophosphamide
what is aspirin exacerbated respiratory disease
seen with asthma and chronic rhinosinusitis and nasal polyposis and eosinophilia but this will not cause a pulmonary infiltrates or renal involvement.
pt has fever, fatigue, weight loss, new unilateral headache, jaw claudication and visual symptoms and non productive cough
think giant cell arteritis
aortic aneurysm and dissection seen with polymyalgia rheumatica in 50% of these pts
Giant cell arteritis
Lab findings of giant cell arteritis
elevated ESR and CRP and normocytic anemia
will see temporal artery biopsy that confirms diagnosis. Remember biopsy can be negative due to patchy dx so repeat biopsy on other side and don’t delay treatment with steroids
long term treatment of pts with giant cell arteritis
perform yearly CXR (up to 10 years) to identify pts with thoracic aortic aneurysms prior to rupture or dissection
if on steroids, needs a DEXA scan for baseline
systemic symptoms of giant cell arteritis?
fever, fatigue, malaise, weight loss
localized symptoms of giant cell arteritis?
headaches, located in temporal areas
jaw claudications: most specific symtpoms of GCA
PMR
Arm claudication associated with bruits in subclavian and axillary areas
aortic wall thickening and anuerysms
CNS: TIA/stroke, vertigo, hearing loss
pt presents with arm claudication associated with bruits in subclavian and axillary areas
aortic wall thickening and anuerysms
need to screen for Giant cell arteritis with ESR and ask about TIAs vision changes and jaw claudication
visual symptoms of GCA
amaurosis fugax, transient visual field defect progressing to monocular blindness
See anterior ischemic optic neuropathy (AION) is most common ocular manifestation
Lab findings of GCA?
normocytic anemia,
elevated ESR and CRP
need to get a temporal artery biopsy.
if negative biopsy still treat empirically and repeat biopsy on other side or get ultrasound.
Treatment of GCA with PMR only?
PMR only: low dose oral glucocorticoids (prednisone 10-20 mg daily for maybe a year)
GCA treatment is
intermediate to high dose oral glucocorticoids (prednisone 40-60 mg daily)
GCA treatment with vision loss
pulse high dose IV glucocorticoids (methylprednisolone 1000 mg daily) for 3 days followed by intermediate to high dose oral glucocorticoids.
start low dose aspirin (recommended) to reduce risk for stroke or intracranial complications.
jaw claudication but no temporal or scalp tenderness and fatigue and loss of appetite and vertigo and TIA
check for GCA
most pts don’t have scalp tenderness.
Is ESR elevated always in GCA?
no. up to 20% of them are negative in GCA.
do we look at Creatine kinase to make determinations on GCA?
no because they can be mildly elevated for multiple of reasons, like medications.
criteria for diagnosing Giant cell arteritis
age >50 yrs
new onset localized headache with fever and visual disturbances
ESR>50
tenderness or decreased pulse of temporal artery
temporal artery biospy showing necrotizing arteritis with mainly mononuclear cells
3 out of 5 criteria is 94% sensitive and 91% specific for GCA
atypical or extracranial giant cell arteritis presentation:
can affect the great vessels of the chest
can cause upper extremity claudication and or aortitis
aortitis can cause aortic root dilation, aortic regurgitation and heart failure
what test should be done for someone who has medium vessel vasculitis?
renal arteriography
for someone with polyarteritis nodosa.
