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VasculitisEvelyn Osio-Salido, MD, MSc, FPCP, FPRA
Associate Professor
Section of Rheumatology, UPCM
Nothing to disclose
Objectives
At the end of the SYMPOSIUM, we hope the attendee will be able to:
1. Discuss the importance of basic knowledge about the vasculitides.
2. Suspect the possibility of vasculitis among patients in daily practice.
3. Institute initial management, as necessary
Objectives
At the end of the LECTURE, we hope the attendee will be able to:
1. Define vasculitis and vasculopathies.
2. Classify vasculitides
3. Recognize common presenting manifestations
1.KEEP CALM.2.You do not need
to memorize these.
3.Remember the essentials.
Definition
Vasculopathy –ANY disease of blood vessels
● Degenerative
● Metabolic
● Clotting disorders
● Embolic
● Inflammatory
ATHEROSCLEROSIS VASCULITIS
Deposition of plaque, made up of fat, collagen, elastin, macrophages, platelets, etc.
Inflammation then scarring of vessel wall.
https://cdn.davidwolfe.com/wp-content/uploads/2017/10/vasculitis-FI-800x419.jpg
Systemic vasculitis
INFLAMMATION of blood vessels
Involve SINGLE organ; often MULTISYSTEMIC
KNOWN or UNKNOWN causes
UNCOMMON but often SERIOUS May lead to death, organ damage, poor QOL.
BETTER OUTCOME with treatment; (+) Relapses
HIGHER RISKS of infection, CVD, malignancy
Kidder D, et al. Medicine (2017) https://doi.org/10.1016/j.mpmed.2017.11.001
Inflammation• Granulomatous
– Giant cell arteritis (GCA)
– Takayasu arteritis (TAK)
– Granulomatosis with polyangiitis (GPA)
– Eosinophilic granulomatosiswith polyangiitis (EGPA)
• Mononuclear cells without granuloma – Kawasaki disease (KD)
• Fibrinoid necrosis– Polyarteritis nodosa (PAN)
• Combined perivascular infiltrates
– Behcet’s disease
• Leukocytoclasis- nuclear debris of neutrophils– Henoch- Schonlein purpura
Inflammation• Granulomatous
– Giant cell arteritis, Takayasuarteritis, Granulomatosis with polyangiitis (GPA)
• Mononuclear cells without granuloma – Kawasaki disease
• Fibrinoid necrosis– Polyarteritis nodosa
(PAN)
Combined perivascular infiltrates - Behcet’s disease
Leukocytoclasis- nuclear debris of neutrophils
- Henoch- Schonlein purpura
Inflammation• Granulomatous
– GCA, TAK, GPA, EGPA
• Mononuclear cells without granuloma - KD
• Fibrinoid necrosis- PAN
Combined perivascular infiltrates- Behcet’s disease
Leukocytoclasis- nuclear debris of neutrophils
- Henoch- Schonlein purpura (HSP)
Single organ or multisystem
• SINGLE organ
– Skin
– CNS
• Multisystem
– G P A
– Granulomatosiswith polyangiitis
https://encrypted-tbn0.gstatic.com/images?q=tbn:ANd9GcR45AoXAWTSYFzXcfZGtmEaPZ-93g2kbQDFySb0rgEnBppOWORdhttps://encrypted-tbn0.gstatic.com/images?q=tbn:ANd9GcQ90s72Evo9VihzioI8NVcsv1eRCGnn7NnU1Wz6Uccpto_ZcFoQ
https://encrypted-tbn0.gstatic.com/images?q=tbn:ANd9GcR45AoXAWTSYFzXcfZGtmEaPZ-93g2kbQDFySb0rgEnBppOWORd
ORGAN GPA MPA EGPA PAN
ENT +++ + ++ polyps --
Kidney ++ +++ + + HPN
Nervous System
++ + +++ +++
Lungs ++ nodules +(+) diffuse + transient --
Eyes ++ + (+) --GIT +(+) +(+) ++ +++
Heart + (+) ++ --
Skin ++ ++ ++ ++
Muscle/Joint
++ ++ ++ ++
ANCA PR3 MPO MPO negative
Known or unknown cause
• PRIMARY-
• SECONDARY-
1. Infection- Hepatitis B, C, Syphilis, Salmonella, TB, Candida, Aspergillus
2. Radiation
3. Drugs- penicillin, quinolone, NSAID, PTU
4. Cancer
5. Connective tissue disease- SLE, RA, sarcoidosis
ORGANS
• Single
• Multiple
CAUSE
• Primary
• Secondary
Vessel SIZE
• Large
• Medium
• Small
• Variable
Classification
http://www.vasculitis.org.nz/__data/assets/image/0005/27536/Vasculitis_Image_1.jpg
Kidder D, et al. Medicine (2017) https://doi.org/10.1016/j.mpmed.2017.11.001
Large
•TAK
•GCA
Medium
•PAN
•KD
Small
•ANCA GPA EGPA MPA
•IC HSP anti GBM Cryoglobulin
CLASSIFICATION ACCORDING TO VESSEL SIZE
VARIABLE VESSEL: Behcet’s disease Cogan’s syndrome
http://www.vasculitis.org.nz/__data/assets/image/0007/27538/Vasculitis_Image_3.jpg
PAINSKIN
NERVES
SKINKIDNEYSLUNGSEYES
Copyrights apply
HSP
CV
MPAGPA
EGPA
Uncommon
Variable incidence of vasculitis depending on:
• TYPE OF VASCULITIS
– GCA 20 per 100,000 vs AAV 20 per million
• GEOGRAPHY
– MPA Japan > UK- 18.2 versus 6.5 per million adults
GPA Japan < UK- 2.1 versus 14.3 per million adults
– West- HSP is most common in children
Asia- Kawasaki disease is most common
If it is rare, why do we need to know it?
Case of Robert
32 year old healthy male, married, employed, physically active
• Sore throat, odynophagia, fever, body aches
• Erythema at pharynx
WHAT IS YOUR DIAGNOSIS?
Case of Robert
32 year old healthy male, married, employed, physically active
• Sore throat, odynophagia, fever, body aches
• Erythema at pharynx
• No response to antibiotics, disappear after weeks
• Recurrent 1x a year or EOY for 7 years
• Pain at R calf with non-itchy papules; similar papules at chest
• Review of history: recurrent oral ulcers (single or multiple) lasting for 1-2 weeks since > 15 years ago
BEHCET’s DISEASEoral ulcers, genital ulcers, uveitis, folliculitis,
erythema nodosum, myositis
Why we need to know vasculitis
• Symptoms & signs are like those of common diseases (a mimic).
• Symptoms & signs may seem mild/innocuous.
• Early treatment is better.
When to suspect vasculitis
• Unexplained systemic signs and symptoms, multisystem
– Fever, weight loss, rash, Proteinuria + casts, High ESR/CRP
• Arterial occlusion and ischemia- Raynaud’s, gangrene, claudication, infarction, stroke, hypertension in young adults
• Skin lesions- splinter hemorrhages (nails), palpable purpura, erythema nodosum, livedo reticularis, urticaria
• GN, Intestinal angina, Mononeuritis multiplex, Headache
• Inflammatory eye disease, sudden visual loss/retinal vascular disease (no HPN/DM)
• Arthralgias/arthritis, myalgias
Mimics of vasculitis
• Bacterial endocarditis• Antiphospholipid antibody syndrome (APAS)• Viral infections- HIV, CMV, HBV, HCV• Cholesterol emboli syndrome• Hypertensive arteriopathy• Vasoconstrictor drugs (ergot poisoning)• Thoracic outlet syndrome• Sepsis, DIC• Sarcoidosis• Thrombotic thrombocytopenic purpura (TTP)• Lymphoma, hair cell leukemia
Summary: Vasculitis
• Uncommon diseases that may present with common symptoms
• Manifestations depend on size and location of blood vessel involved
• Need for thorough investigation by the physician and health care team.
Thank you for listening.
04. Which of these microbes cause vasculitis through immune complex deposition?
A. Hepatitis B
B. Mycobacterium tuberculosis
C. Salmonella
D. Syphilis
05. Which vasculitis occurs among children less than 5 years old?
A. Giant cell arteritis
B. Polyarteritis nodosa
C. Kawasaki disease
D. Takayasu arteritis
06. Which is the correct match?
A. GPA- asthma
B. EGPA-swollen ear lobe
C. Behcet- oral ulcer
D. Takayasu- blindness