VASKULITIS

SRI VITAYANI
 VASCULITIS: principles 1
group of clinical syndromes characterized
by inflammation
of blood vessels

Artery: WBC
Normal Artery
inflammation in wall
 VASCULITIS: principles 2
systemic diseases that can affect many different
organ systems
can be difficult to diagnose: challenging clinical
picture even for experienced clinicians

can be life-threatening
VASCULITIS: classification

www.wegenersgranulomatosis.net/imageRJN.JPG
 When should vasculitis be
suspected? 1

MULTISYSTEM inflammatory disease

Significant CONSTITUTIONAL SYMPTOMS
RAPIDLY PROGRESSIVE organ dysfunction
HIGH ESR
SEVERE anemia
PLATELETS > 500K
 When should vasculitis be
suspected? 2
CLINICAL FEATURES PARTICULARLY
SUGGESTIVE of small vessel inflammation:
SKIN: palpable purpura *
LUNGS: pulmonary infiltrates /
hemoptysis
KIDNEY: active urinary sediment
NEURO: foot drop **
 Vasculitis: Basic Facts
Vasculitis affects all ages, although some
types are restricted to certain age groups
Vasculitis tends to affect Caucasians, although
many African-Americans are affected
Vasculitis has a genetic component, but is not
heritable
Vasculitis is a chronic relapsing disease,
although some patients experience prolonged
remission
 Vasculitis: Definition
Pathologist Rheumatologist
Inflammatory destruction of blood A clinicopathologic process
vessels characterized by
Infiltration of vessel wall with inflammatory destruction of
inflammatory cells blood vessels that results in
Leukocytoclasis occlusion or destruction of
Elastic membrane disruption the vessel and ischemia of
Fibrinoid necrosis of the vessel the tissues supplied by that
wall vessel.
Ischemia, occlusion, thrombosis Systemic vasculitides
Aneurysm formation
Rupture, hemorrhage
 Vasculitis: Classification
Large-vessel vasculitis
Giant cell arteritis, Takayasus arteritis
Behcets disease, Cogans syndrome
Medium-vessel vasculitis
Polyarteritis nodosa
Buergers disease, Central nervous system vasculitis,
Kawasakis disease, Rheumatoid vasculitis
Small-vessel vasculitis
Wegeners, microscopic polyangiitis, Churg-Strauss
Cryoglobulinemic vasculitis, Henoch-Schnlein
purpura,
 Vasculitis: Classification
Large-vessel vasculitis
Giant cell arteritis, Takayasus arteritis
Behcets disease, Cogans syndrome
Medium-vessel vasculitis
Polyarteritis nodosa
Buergers disease, Central nervous system vasculitis,
Kawasakis disease, Rheumatoid vasculitis
Small-vessel vasculitis
Wegeners, microscopic polyangiitis, Churg-Strauss
Cryoglobulinemic vasculitis, Henoch-Schnlein
purpura,
 Vasculitis: Classification
Large-vessel vasculitis
Aorta and the great vessels (subclavian, carotid)
Claudication, blindness, stroke
Medium-vessel vasculitis
Arteries with muscular wall
Mononeuritis multiplex (wrist/foot drop), mesenteric
ischemia, cutaneous ulcers
Small-vessel vasculitis
Capillaries, arterioles, venules
Palpable purpura, glomerulonephritis, pulmonary
hemorrhage
 ANCA-associated vasculitides
Wegeners granulomatosis: granulomatous
inflammation involving the respiratory tract and
necrotizing vasculitis affecting small to medium-
sized vessels
Microscopic polyangiitis: Necrotizing vasculitis
affecting the small vessels.
Churg-Strauss Syndrome: Eosinophil-rich and
granulomatous inflammation involving the
medium-sized vessels, and associated with
asthma and eosinophilia
 Sinusitis
Necrotizing Granuloma Subglottic stenosis
Pulmonary nodules
Orbital pseudotumor

Pulmonary capillaritis
Glomerulonephritis Hypereosinophilia
Sensory neuropathy Asthma
Mononeuritis multiplex Pulmonary infiltrates
Myocarditis
 Vasculitis: Diagnosis
Diagnosis of a systemic vasculitis is often a diagnosis of
exclusion, based on recognition of the clinical syndrome
e.g. Churg-Strauss: adult onset asthma x 2 years, followed by
atypical pneumonias, followed by peripheral nerve involvement
Biopsy of involved organ is the most straightforward method
of establishing a diagnosis
Biopsy may be helpful to exclude infection/malignancy
Other tests may be suggestive, but not diagnostic
ESR, CRP
CT: pulmonary hemorrhage, cavitary lesions
Bronchoscopy: pulmonary hemorrhage (hemosiderosis)
Urinalysis: for patients with kidney vasculitis
ANCA (antineutrophil cytoplasmic antibodies)
Angiogram (including MRA, CT-angiogram)
 Vasculitis: Treatment
Remission induction:
Cyclophosphamide 2mg/kg po qd x 3-6 months
[or 15 mg/kg IV q 2 wk x3 then q 3 weeks x 6-12 months]
Prednisone 1mg/kg po qd x 1 month, then taper
[Bactrim, Calcium, Vitamin D]
Remission maintenance (minimum 2 years)
Methotrexate 20-25 mg po q week + folate
Azathioprine 2mg/kg po qd
Mycophenolate mofetil 1.5 g po BID
Leflunomide 20-30 mg po BID
 Vasculitis: Monitoring
Large-vessel vasculitis
MRI/MRA chest/abdomen/pelvis every 6-12 months
Medium-vessel vasculitis
Mesenteric angiogram to assess disease activity
EMG/NCV to monitor nerve damage
Wound care for cutaneous ulcers
Small-vessel vasculitis
Chest CT every 6-12 months
Blood and urine tests every 1-4 weeks
 Vasculitis: Burden of Disease
32 year old woman with Wegeners granulomatosis
Drugs: cyclophosphamide, prednisone, TMP/SMX,
Calcium+vitamin D
Monitoring:
Lab tests every 1-4 weeks
CT scans every 6-12 months
PFTs with flow-volume loops, EMG/NCV
Consultants:
Otolaryngology: Sinusitis, subglottic stenosis
Nephrology: Chronic renal insufficiency
Gynecology: Fertility counseling
Neurology: Management of peripheral neuropathy
Rehabilitation, pain management
 COMPLEX MEDICAL PROBLEMS 4

SYSTEMIC VASCULITIS ?

Are there additional tests which could help

confirm this suspicion?

Serologic tests
Imaging studies
Tissue biopsy
 VASCULITIS: additional testing 1

Serologic tests
ANCA
Hepatitis B surface antigen
Hepatitis C, C3 & C4
HIV
ANA
ACA, lupus anticoag panel
 VASCULITIS: additional testing 2

Imaging studies
Sinus CT scan
Chest CT scan
Mesenteric
angiogram
 VASCULITIS: additional testing 3

Tissue biopsy
Temporal artery
Sural nerve
Muscle
Lung
Renal
 Common Clinical Manifestations
Respiratory
Systemic Sinusitis / Epistaxis
Fever, sweats, Pulmonary infiltrates
weight loss
Skin Gastrointestinal
Palpable Purpura Abdominal Pain
Neurologic Bloody stools
Mononeuritis Multiplex Renal
Musculoskeletal Glomerulonephritis
Arthralgia / arthritis Hypertension
Muscle pain / claudication
 CUTANEOUS

Palpable Purpura
Livedo Reticularis
Splinter Hemorrhages
 NEUROLOGIC
Mononeuritis multiplex:
check for FOOT DROP

Sural nerve biopsy showing vasculitis

 RESPIRATORY: upper

Sinusitis
Or

www.conseils-orl.com/.../sommaire_epistaxis.htm
 RESPIRATORY: lower
Pulmonary infiltrates
Nodules
Cavities
 GENITOURINARY

Glomerulonephritis
Hypertension
Hematuria
RBC casts

Testicular pain
(especially PAN)

www.bio.davidson.edu/.../Cresgn.jpg
 MUSCULOSKELETAL
Polyarthralgias - common
Polyarthritis - less common

Myalgias - common
Myositis - biopsy may demonstrate
vasculitis in muscle
 GASTROINTESTINAL
Mesenteric ischemia
pain 30 minutes after eating
bloody diarrhea
bowel perforation
hepatitis
pancreatitis
cholecystitis

library.med.utah.edu/WebPath/COW/COW125.html
 OCULAR
Scleritis
Retinal Vasculitis

Iritis

http://www.uveitis.org/images/sa
rcoid6.jpg

eyelearn.med.utoronto.ca/.../RedE
ye/10Sclera.htm http://webmedia.unmc.edu/eye/iritis.jpg
 Common Laboratory Findings
INFLAMMATION:

Elevated ESR (can be > 100)

Elevated CRP
Leukocytosis
Thrombocytosis
Anemia
Low Albumin
 VASCULITIS MIMICS
INFECTIOUS DISEASES
Endocarditis
HIV
DRUGS
Cocaine
Methamphetamine
CHOLESTEROL EMBOLI
ANTIPHOSPHOLIPID ANTIBODY SYNDROME
 Vasculitis: Long-term Damage
Large-vessel vasculitis
Blindness, Stroke
Claudication: Angina of the arms
Medium-vessel vasculitis
Foot drop: inability to lift a foot
Wrist drop: inability to lift a hand
Cutaneous ulcerations
Small-vessel vasculitis
Oxygen dependence
Renal insufficiency/failure
Vasculitis: Patient Perspective

Herlyn K, Arthritis Rheum 2010; 659

 Vasculitis: Patient Perspective
Pain Fatigue
Disease-Specific Vasculitis-induced
Sensory neuropathy Loss of proprioception
Cutaneous ulcerations
Glucocorticoid-induced
Arm claudication Muscle loss, Weight gain
Emotional liability
Non-specific
[Arthritis] Immunosuppression-induced
Vertebral fracture Cognitive impairment
Drug-associated fatigue
 Vasculitis: Summary
The systemic vasculitides are chronic diseases,
characterized by relapse and remission
Achieving remission requires intense monitoring by a
multidisciplinary team with expertise in these diseases
Even after achieving disease remission, patients will
continue to suffer from the chronic, irreversible
consequences of both the disease and its therapies
Pain and fatigue are common consequences of
vasculitis that are independent of disease activity and
generally fail to respond to immunosuppression