Vasculitis Flashcards
What is Vasculitis
Collection of autoimmune diseases that cause inflammation of the blood vessels
Inflammation leads to damage (Impaired flow, vessel leakage, hemorrhage, orang damage, death)
Vasculitis General findings
Fever, malaise/fatigue, weight loss
Labs: leukocytosis, thrombocytosis, decreased albumin, anemia, elevation of CRP or ESR
(Small Vessel) kidney involvement
Small Vessel Casculitis Sign and Symptoms
Scleritis Palpable purpura Glomerulonephritis Inflammatory arthritis Diffuse hemorrhage Mononeuritis multiplex CNS (nerve blood supply issues)
Small Vessel Vasculitis ANCA (anti neutrophilic cytoplasmic antibody) associated
C-ANCA (cytoplastic staining), Serine protease 3 (PR3)
P-ANCA (perinuclear staining), Myeloperoxidase (MPO)
Atypical ANCA - neither c/p staining
What are the associated diseases?
Granulomatosis with polyangiitis (GPA, aka Wegener’s Granulomatosis)
Microscopic polyangiitis (MPA)
Eosinophilic granulomatosis with polyangiitis (EGPA aka Churg-Strauss)
Granulomatosis with polyangiitis signs and symptoms
C-ANCA/PR3 > -PANCA/MPO
ENT & Raspatory disease common - otitis, tracheal stenosis
Eyes - 30-60% orbital inflammation, episcleritis, scleritis, uveitis, lacrimal duct obstruction, retinal artery thrombosis.
Renal disease (esp. w/ pregnancy)
Nervous system - mononeuritis complex (foot drop. facial paralysis), pachymeninges, CN neuropathies, pituitary/hypophysis
Skin - purpura, Ulcers
MSK - arthralgia, myalgia, inflammatory arthritis
Saddle nose
Microscopic Polyangiitis (MPA)
Silent Kidney punch
P-ANCA > C-ANCA/PR3
All have rapidly progressive Glomerulonephritis
similar to GPA - except much less ENT involvement
Diffuse alveolar hemorrhage, no nodular/granulomatous lung disease
GI tract may be involved
Eosinophilic granulomatosis with polyangiitis (EGPA)
Like GPA + asthma/eosinophils, more and allergic “flavor”
Lungs with nodular lesions
Adult-onset asthma - severe, steroid dependent
mononeuritis multiplex
much less renal disease
peripheral eosinophilia, melts away with steroids
How to diagnose Small Vessel Vasculitis
Clinical syndrome
Labs: evidence of inflammation, renal disease, positive ANCAs - EGPA peripheral eosinophilia
Biopsy: renal biopsy showing immune GN, skin, lung, sinuses, sural nerve
Imaging: CXR, CT of lungs maybe sinuses
Small Vessel Vasculitis (ANCA+)treatments
Induction: IV methylprednisolone (1 G daily for 3 days -> 1mg/kg PA), RITUXIMAB, (for severe), Cyclophosphamide
Maintenance: RITUXIMAB»_space; others: Azathioprine, Methotrexate, Mycophenolate
EGPA: above plus anti-IL-5 (Mepolizumab)
Henoch Schoenlein Purpura Symptoms
Most common children 3-10 M>F 2:1 Upper respiratory infection Immune complexes again IgA deposit in blood vessels Purpuric rash Abdominal pain/blood, diarrhea inflammatory arthritis/arthralgias Glomerulonephritis
Henoch Schoenlein Purpura diagnosis
Clinical symptoms, biopsy of rash or kidney: Immune-florescence would show IgA deposition
Elevation of blood IgA in 50%
Resolves without treatment in 94% of kids, 84% of adults
Henoch Schoenlein Purpura treatment
Symptoms: NSAIDs, Acetaminophen, oral steroids
Rash: treat with dapsone
Severe kidney disease: IVIG, Cyclophosphamide, Azathioprine
Anti-GBM disease aka Goodpasture’s disease
Antibodies directed against glomerular basement membrane
Typically presents with glomerulonephritis and diffuse alveolar hemorrhage = pulmonary renal syndrome
more common in Caucasians, also some specific ethnic groups
Age 20-30 or 60-70
may be a result of environment and genetics (cocaine, infections, smoking, metal dust)
Anti-GBM disease diagnostics
Blood anti-GBM antibodies
chest imaging and potentially bronchoalveolar lavage
renal biopsy - showing linear IgG staining pattern on immunofluorescence
anti_GBM treatment
plasma exchange
high dose steroids
Cyclophosphamide
Other diseases associated with small vessel vasculitis
RA
SLE
Sjogren’s
Treatment: steroids, rituximab and/or Cyclophosphamide
Medium Vessel Vasculitis Signs and Symptoms
Livedo rash Ulcerative skin lesions Mononerutis multiplex Coronary involvement GI and renal involvement
Polyarteritis Nodosa
(Med Vessel)
Can be skin limited - livedo rash, ulcerativ skin lesions, nodular lesions, purpura - deep tissue biopsy to see med size vessels
OR
systemic: Oset 40-60 - skin + fever, weight loss, night sweats, fatigue peripheral nerves renal infarcts mylagias, arthralgias Abdominal pain (least common) Stroke, seizure, heart failure, MI, retinal hemorrhage, potic ischemia, orchitits/testicular pain
Poly arteritis Nodosa diagnosis
Labs: elevated ESR/CRP
CBC: anemia, thrombocytosis, leukocytosis
CMP elevated creatinine, LFT abnormalities, low albumin
May be associated with Hep B
Poly nodosa diagnosis
Differential: segmental arterial mediolysis, fibromuscular dysplasia, Ehlers-Danlos, pseudoxanthoma elasticum
Dx on presentation, angiogram or CT radiogram showing typical vasculitis changes in vessels. Biopsy often shows fibrinoid necrosis and inflammatory infiltration of vessel wall
Polyarteritis Nodosa treatment
Severe: pulse steroids, Pred 1mg/kg. Cyclophosphamide for induction, azathioprine, or methotrexate form maintenance
Mild-moderate: Prednisone tapered for 6-12 months, Methotrexate or Azathioprine
HepB associated: Pred 60mg/day for a week, taper 50% day till off, Plasma exchange, HepB antiviral treatment
Kawasaki disease demographics
Medium Vessel Vasculitis
Peak incidence 9-12 months, 80% <5 yo
Male>Female 2:1 (Japaneses 17x)
Prolonged, high fevers with conjunctivitis, desquamative rash, strawberry tongue
Can cause aneurysm/pseudoaneurysm affecting the Heart
Kawasaki Disease Key symptoms
Conjunctivitis
Rashed with cracked and red lips
Strawberry tongu
Desquamation (skin peeling)
Kawasaki disease complete diagnosis
Polymorphous rash, oropharyngreal mucous membrane change, conjunctival injection, extremity changes, lymphadenopathy
Supportive labs: ESR & CRP
Suspected diagnosis: EKG, Echo, discuss with pediatric cardiology
Kawasaki disease treatment
Acute: IVIG 2g/kg & high dose aspirin 80-100mg/kg/day; once fever resolves, decrease aspririn, risk of aneurysms controlled with corticosteroid, warfarin
Treatment failures: repeat second IVIG course, IV solumedrol, infliximab
Serial echocardiograms: At 1, 2, 6, 12 weeks then every 5 years
Large vessel vasculitis
Aortic arch disease in Takayasu
Giant cell arteritis
Giant Cell Arteritis Overview
Most common vasculitis for people over 50
Caucasian, F>M
Giant Cell Arteritis Sympoms
Constitutional Symptoms: fever, weight loss (15-20%)
Cranial: Jaw claudication, headache, scalp pain/tenderness
Eye: vision changes (blurry, double, loss, optic neuritis/atrophy) - loss in up to 15%
CV: unequal BP (aortic involvement) bruits
Resp: cough
MSK: PMR symptoms (shoulder/hip girdle stiffness)
Gian Cell Arteritis Labs
Nonspecific: Elevated ESR & CRP, CBC of anemia and thrombocytosis, CMP elevated alk phos
Cranial disease - TA US showing characteristic findings of temporal artery biopsy
Extracranial disease - PET, MRA, CTA
Giant Cell Arteritis Differential
Takayasu Bechet's IgG4 RD Heritable CTD Lymphoma
Jaw claudication amyloidosis
Zoster
GCA Treatment
High dose steroids (IV if threatened vision, otherwise 1mg/kg daily for 1 month bolowed by lsow taper),
Low dose aspirin
Tocilizumab
Methotrexate
Monitoring - ESR/CRP, imaging, lipids, LFTs, PLTs. on Tocilizumab
Takayasu Arteritis overview
young onset (~25)
F»M 8:1
Asian ethnicities
Hypertension - unequal BPs (>10 systolic) take BP in all 4 extremities
Takayasu Arteritis Phases
Phase 1: fevers, arthralgias, weight loss
Phase 2: vessel pain, tenderness
Phase 3: fibrotic, bruits and ischemic symptoms (limb claudication, lightheadedness/syncompe)
Takayasu arteritis Labs
Nonspecific - ESR & CRP elevation (or normal in 30%)
Imaging: MRA preferred, CTGA, FDG-PET
Takayasu Arteritis treatment
high dose steroids - Prednisone 30 mg BID with slow taper Methotrexate, Azathioprine Maybe anti-TNF before IL6 etc. Relapse is common Surgery not indicated
Bechet’s overview
M:F 1:1
Mean onset 30
more common in ethnicities of the silk road
HLA–B51 association outside US more severe course
Bechet’s clinical syndrome
Recurrent painful mouth/genital ulcers
May have inflammatory eye disease - anterior/posterior uveitis, retinal vasculitis
Skin manifestations - pseudofolliculitis, papulopustorlar lesions, E nodosum
Bechet’s differential
Differential: Crohn’s disease, recurrent aphthous stomatitis, reactive arthritis, lupus and others
