9 - Vasculitis Flashcards
Vasculitis breaks down into?
Large vessel
Medium vessel
Small vessel - immune-complex mediated
Small vessel - ANCA associated vasculitis
Large vessel includes
Large vessel
- giant cell arteritis
- Polymyalgia rheumatica
- takaysu’s arteritis
- behcet syndrome
Medium vessel includes
Medium vessel
- polyarteritis nodosa
- buerger’s disease
- Kawaski s disease
Small vessel immune complex mediated vasculitis includes
Small vessel - immune-complex mediated
- cutaneous (leukocytoclastic) vasculitis
- henoch-schonlein purpura
- cryglobulinemia
Small vessel ANCA-associated vasculitis includes
Small vessel - ANCA associated vasculitis
- granulomatosis w polyangitis (wegners)
- churg-strauss syndrome
- microscopic polyangiitis
MC type of vasculitis in adults?
Giant cell arteritis (GCA)
Who gets GCA?
Female >50 yo
S/S of GCA?
HA Scalp tenderness Fever Visual symptoms Jaw claudication
GCA has a risk of?
Sudden blindness due to ischemic optic neuritis
Diagnostics for GCA?
ESR - very high (80-100)
Temporal artery biopsy (w/in 2 wks of steroids)
Tx for GCA?
PROMPT initiation of HIGH DOSE corticosteoids
- Prednisone until better (3-4 wks)
Low dose aspirin
- vision loss or stroke prevention
Dont delay steroids for?
The temporal artery biopsy. Start that shit
GCA also needs to worry about?
Osteoporosos
Focus on prevention
- smoking
- exercise
- calcium
- D
- biphosphonate (BMD)
Age for polymyalgia rheumatica (PMR)
> 50
PMR is like ___ but ___
Like GCA but 2-3x more common
Unlike GCA PMR…
Has no risk for blindness with “pure PMR
But it may progress to GCR
S/S of PMR?
Stiffness + pain in shoulders and hips
- esp mornings (>30min)
“I feel old” Muscle pain (not in joint) >1mo
PMR has stiffness in 2 of 3 ares:
Neck/torso
Shoulders
Hips
Diagnostics for PMR?
ESR > 40
Tx for PMR?
Low dose prednisone
- x 1-2 yrs
Weekly methotrexate increases success
Prognosis for GCA?
Thoracic aortic aneurysms
aortic dissection
are late complications
Takayasu’s arteritis is aka?
Pulseless disease
Occlusive thromboaortopathy
Granulomatous vasculitis of the aorta and major branches
Who get takayasu’s arteritis?
MC = asian females age 10-30
What distinguishes takaysu’s arteritis?
Aorta/branch involvement
Pt presentation for takayasu’s arteritis?
Appear chronically ill
- fatigue
- wt loss
- low grade fever
- upper extremity claudication
PE for takayasu’s arteritis?
Abnormal pulse exam
- Unequal blood pressures
- Carotid/subclavian bruits
- Hypertension
Cardiac:
- aortic regurg
- angina
Labs for takayasu’s arteritis?
high ESR
high CRP
Tx for takayasu’s arteritis?
Prolonged corticosteroid treatment
- HIGH DOSE prednisone x 1 mo then taper 4-6 mo
+ methotrexate/mycophenolate mofetil
Surgery for takayasu’s arteritis?
Surgery/revascularization therapy
- PCA (renal arteries)
- surgical revascularization
- aortic valve replacement
Prognosis for takayasu’s arteritis?
10 yr survival»_space; 80-90%
Who get behcet syndrome?
Young adults 25-35
Asian
Turkish
Mid eastern
Genetic risk factor for behcet syndrome?
HLA B51
What is unique about behcet syndrome?
It is an inflammatory vaasculitis that affects large, medium and small blood vessels
All 3 sizes, both arteries and veins
It gets around it you know what i mean
If large vessels are involved (behcet syndrome)
Pulmonary artery aneurysm
Pulmonary hemorrhage
Hallmark of behcet syndrome?
Painful aphthous ulcers in mouth
- erythema nodosum-like lesions that ulcerate
behcet syndrome also has? (Eyes)
Anterior or posterior uveitis
Labs for behcet syndrome?
No specific confirmation labs
ESR/CRP often elevated
Tx for behcet syndrome?
Multiple:
- corticosteroids
- dapsone
- colchicine
- azathioprine
behcet syndrome sever ocular and CNS complication tx?
Pick one:
- infliximab
- cyclosporine
- cyclophosphamide
What is polyarteritis nodosa (PAN)?
Necrotizing inflammatory vasculitis of med arteries and muscular arterioles
PAN spares?
Venous circulation and capillaries
PAN presentation?
Insidious onset over weeks to months
- w/in 6 months of Hep B infection
Presentation of PAN? (S/S)
Mononeuritis multiplex (foot drop) Freq abdominal pain (post eating) - if mesenteric vessel involved HTN (2/2 renal artery involv) Derm presentatinos
Derm presentation of PAN?
Palpable purpura
Livedo reticularis
Skin ulcers
Digital gangrene
Diagnostic for PAN?
- HBV testing (at diagnosis)
- ANCA-neg(true PAN)
- Biopsy (skin, nerve, muscle)
- Angiography (mesenteric)
Tx for PAN?
Prolonged corticosteoids
Cyclophosphamide (cytoxan)
Most achieve remission w this
Prognosis of PAN?
W/o tx - 5 yr = 10%
W tx 5 yr = 60-90%
Classic clinical finding of small vessel vasculitis?
Palpable purpura
Small vessel vasculitis includes?
Arterioles
Venules
Capillaries
MC vasculitis in kids <6yo?
Henoch-schonlein purpura (HSP)
HSP is strongly associated with?
IgA deposition
HSP usually follows?
Viral URI But also: - EBV - Varicella - Parvovirus - Strep - Staph - Mycoplasm
S/s of HSP?
Palpable purpura (lower extremeties)
Arthralgias/arthritis
GI - colocky abd pain
Renal - IgA immune complex deposition -> glomerulonephritis
Diagnostic for HSP?
Clinical symptoms
Biopsy w predominant IgA deposition
Mandatory finding for HSP?
Palpable purpuric rash
Tx for HSP?
Supportive care
Tylenol/NSAIDs
Cryoglobulinemia caused by?
Immunoglobulins (antibodies IgG, IgM) that precipate out of blood at low temp (<37*C)
Cryoglobulinemia is associated with?
Hep C infection
Types of Cryoglobulinemia?
Type I - hematologic syndrome
- hyperviscosity
Type II and III - can cause small vessel vasculitis
Clinical triad of Cryoglobulinemia?
Purpura
Arthralgias
Myalgias
Also includes peripherial neuropathy
And renal disease
Cryoglobulinemia lab work?
RF POS
Cryoglobulins in conjunction
Tx of Cryoglobulinemia?
Treat the Hep C
- sofosburiv
- simeprevir
- harvoni
Severe cases get steroids or immunosuppressive agents (ESRD)
Granulomatosis with polyangitis (GPA) is a?
Small vessel ANCA associated vasculitis
A necrotizing vasculitis
GPA is aka?
Wegener’s
Who gets wegeners?
White N. Europeans in 40-50’s
GPA (wegner’s) triad?
Manifestations of:
- Upper respiratory
- Lower respiratory
- Renal
Hallmark of GPA (wegner’s)?
- Granulomatous inflammation
- Vasculitis
- Necrosis
Upper respiratory disease with GPA (wegner’s)?
Nasal manifestations
Sinus pain Otitis media Subglottic stenosis Epistaxis Persistent rhinorrhea Septal perforation Saddle nose
Lower respiratory disease GPA (wegner’s)?
- Alveolar capillaritis resulting in hemoptysis
- Granulomatous nodules and caveties
- Glomerulonephritis»_space; renal involvement
Diagnostics for GPA (wegner’s)?
C-ANCA positive (unlike CSS)
Absent pANCA (less useful)
Biopsy showing granulomatous inflammation
UA - RBC casts and hematuria
CT>CXR
Tx for GPA (wegner’s)?
1st line:
- corticosteroids/cyclophosphamide or ruximab
Post remission:
- azathioprine/methotrexate
Churg strauss syndrome is aka?
Eosinophilic granulomatosis with polyangiitis
What is churg-strauss syndrome?
Necrotizing vasculitis associated with
- allergic rhinitis
- reactive airway disease
- peripheral eosinophilia
Clinical findings for churg-strauss syndrome?
Allergic rhinitis + nasal polyps
Adult onset asthma
Clinical phases of churg-strauss syndrome?
Prodromal phase: allergic rhinitis or asthma
Eosinophilic phase
- peripheral blood eosinophilia and eisinophilic infiltration of multiple organs
Vasculitic phase:
- life threatening systemic necrotizing vasculitis of small and med vessels
- multi organs
churg-strauss syndrome does not have ?
Renal involvment (vs GPA)
labs for churg-strauss syndrrome?
P-ANCA pos (vs GPA)
Biopsy Vascular Extravascular (nerve, muscle, heart) Typical clinical features: - asthma - allergic rhinitis - eosinophilia
Tx for churg-strauss syndrome?
Corticosteroids
Cyclophosphamatide for neuropathy/glomerulonephritis
Azathioprine/Methotrexate can be added to corticosteroids
