Vasculitides are a hetrogenous group of conditions characterized by inflammation and necrosis of blood vessels. A broad group of syndromes may result from this process,since any type,size, and location of vessel may be involved.

1. Vasculitis Syndrome
An Approach
And
Basic Principles of Treatment
Dr. Sachin Verma MD, FICM, FCCS, ICFC
Fellowship in Intensive Care Medicine
Infection Control Fellows Course
Consultant Internal Medicine and Critical Care
Ivy Hospital Sector 71 Mohali
Web:- http://www.medicinedoctorinchandigarh.com
Mob:- +91-7508677495

2. Introduction
• Vasculitides are a hetrogenous group of
conditions characterized by inflammation
and necrosis of blood vessels.
• A broad group of syndromes may result
from this process,since any type,size, and
location of vessel may be involved.

3. Classification of vasculitides:
 Various attempts made to create a classification of
vasculitides.
 But it remains a matter of controversy.
 Most classifications are based on:
1. Size vessels it involves.
2. Histological findings from involved vessels.
3.Combination of both vessel size and histological
findings.
But all scheme of classification is imperfect.

4. Pathophysiology and pathogenesis
 Pathogenic immune-complex formation:
 1.Most widely accepted mechanism
 2.Casual role is not clearly established
Examples- PAN, EMC
 Antineutrophilic cytoplasmic antibodies(ANCA):
 Two types- 1. c-ANCA, Examples; WG
2. p-ANCA,Examples;MPA, CSS, Crecentric
GN, GPS , WG
 Pathogenic T-Lymphocytes response and granuloma
formation:
Examples; WG , Giant cell arteritis,Takayasu arteritis
CSS

5. Etiopathogenic classification incorporating the
modified CHCC Classification by Lie
DOMINENT PRIMARY VASCULITIS PATHOGENESIS
VESSELS
LARGE ARTERIES Temporal arteritis T cell mediated
Takayasu’s arteritis
MEDIUM ARTERIES Classical PAN Immune complex related
Kawasaki’s disease Antibody mediated
hypersensitivity
SMALL AND MEDIUM Wegener’s granulomatosis
SIZE ARTERIES CSS Antibody mediated
MSA (ANCA)
SMALL VESSEL VASCULITIS HSP
(Leukocytoclastic) EMC Immune complex mediated
CUTANEOUS LCV

6. The most recent classification scheme proposed
by the American College of Rheumatology (ACR)
• Uses both vessel size and type of inflammatory infiltrate.
• It classifies vasculitis as follows:
Polyarteritis nodosa (PAN),
Churg-Strauss syndrome,
Wegener's granulomatosis,
Hypersensitivity vasculitis,
Henoch-Schönlein purpura,
Giant cell arteritis,
Takayasu's arteritis,
Granulomatous angitis of CNS, Berger's
disease, and
Kawasaki disease

7. INDIAN PERSPECTIVE
• Reliable epidemiological data from india is
not available
• Takayasu’s arteritis is commonest
vasculitis described.
• Temporal arteritis is extremly uncommon
• Patients with WG are seen in significant
number in north,but rare in south.
• Classical PAN has been described all over.

8. Frequency distribution of vasculitic disorders in
India (N=1064)*
DISEASE NO. PERCENTAGE
Aortoarteritis 215 20.20
Giant cell arteritis 35 3.36
Polyarteritis nodosa (PAN) 95 8.83
Cutaneous PAN 13 1.22
Wegener’s granulomatosis 147 13.83
Microscopic polyangiitis 42 3.94
Churg Straus syndrome 19 1.78
Henoch Schonlein purpura 232 21.80
Small vessel vasculitis 61 5.73
Behcet’s disease 145 13.62
Kawasaki disease 05 0.46
Undiagnosed 50 4.69
Others** 6 0.56

9. CLINICAL MANIFESTATIONS
• The Vasculitides are truly a ‘multisystem’
diseases. No organ or system is spared.
• General Symptomatology :
Fever
Weight loss
Malaise
Fatigue
Night sweats
Anemia
Generalised aches and pain

10. CLINICAL MANIFESTATIONS : A GUIDE TO THE TYPE OF VESSELS INVOLVED
CLINICAL MANIFESTATIONS:
LARGE VESSELS MEDIUM VESSELS SMALL VESSELS
Limb claudication Red and blue Panniculitis Purpura
Asymmetric blood pressure Ulcer Vesicobullous lesions
Absence of pulses Livedo reticularis. Urticaria
Bruits Digital gangrene Glomerulonephritis
Aortic dilatation Mononeuritis multiplex Alveolar haemorrhage
Renovascular Hypertension Microaneurysm Splinter haemorrhage
Reno-vascular hypertension Uveitis,episcleritis,scleritis
Mucosal ulcer in bowel

11. Continue…
• SKIN: Skin commonly involved.
o Palpable purpura is commonest dermal
lesion.
o Other specific lesions are:
 nodules and plaques which may ulcerate
 Infarcts
 Ulcers
 Pyoderma gangrenosum
 Wide spread skin necrosis and gangrene.
 Macules, papules,vesicles, blisters, and small
bullae has been described.

12. SKIN LESIONS

13. Muscles and Joints:
 Features are common, but nonspecific.
Arthralgia and Arthritis
Generalised Myalgia and Weakness
Claudication, Pain
Eye ,Ear ,Nose and Throat:
Scleritis
Scleromalacia
Perforation of globe
Uveatis
Recurrent Otitis media
Hearing loss
Recurrent sinusitis, nasal septum damage
Several and recurrent oral ulcerations

14. Airways and Lungs:
Stridor
Ventilatory compromise
Cough
Chest pain
Expectoration and Hemoptysis
Pulmonary lesions like:
infiltrate
nodules
cavities
mass lesions
abscess

16. Gastrointestinal tract:
 most important G.I. manifestations are due to
bowel ischemia.
Kidneys:
 Great diversity of lesions:
Renovascular hypertension
Infarction and Hematoma of kidneys
Mild GNs to rapidly progressive GNs

17. Nervous system:
Peripheral Neuropathy, Stroke
Reproductive system:
Testicular Infarction
Scrotal ulcers
Penile ulcers on Glans and Shaft
Cardiovascular system :
Pericarditis, Myocardial infarction
Aneurysmal rupture

18. Approach to the patient
• Diagnosis of vasculitis is considered in an any
patient with unexplained illness.
• Certain clinical abnormalities when present
alone or in combination suggest a diagnosis of
vasculitis like…
1.Palpable purpura 2.Pulmanary infiltrates
3. Microscopic hematuria
4.Chronic inflammatory sinusitis
5.Unexplained ischemic events
6.Glomerulonephritis
with evidence of multisystem disease

20. Laboratory Work-up
• First to exclude the diseases which can
mimic vasculitis.
• To establish the category of vasculitis
syndrome.
 Hemogram :
Normocytic Normochromic anemia
Leukocytosis(>10% Eosinophils in CSS)
Acute phase reactants:
Raised ESR, CRP, alpha-2 globulin,
fibrinogen, Thrombocytosis, ALP

21. Continue….
Urine analysis:
Hematuria
Proteinuria
Serum proteins:
Hypergammaglobulinemia
(mostly IgG type, IgA in HSP, WG)
Complements levels usually decreased
Antineutrophil cytoplasmic
autoantibodies (ANCA):
c-ANCA p-ANCA A or x-ANCA

22. Continue….
Organ biopsy:
Gold standard for diagnosis of vasculitis.
Angiography :
Especially for medium and large vessels.
 Others:
X-rays, CT Scan, MRI of thorax
MRA
USG Studies

23. Basic principals of treatment
• Glucocorticoids
• Glucocorticoids + cytototoxic drugs
• Antiviral therapy if indicated
• Plasma exchange and IV Ig + or aspirin
• Surgical correction and Angioplasty

24. Continue…
• Sympotomatic mangement in cases of
hypersensitivity vasculitis
• White blood cell counts every 1-2wks and
WBC count should be maintained above >
3000/microL.
• TMP-SMX should be given to every patient
receiving glucocorticoids and cytotoxic
drugs combination therapy.

25. SMALL AND MEDIUM
SIZED
VASCULITIS SYNDROMES

26. WEGENNER’S CHURG SRAUSS MICROSCOPIC
GRANULOMATOS SYNDROME POLYANGITIS
IS PAN
1.2:1 OVER ALL OVER ALL
1:1 NO MUCH DIFF. UNKNOWN UNKNOWN
M:F FROM WESTRN
1:14(INDIA) DATA 4.5:1(INDIA)
TYPE OF VESSELS SMALL ARTERIES SMALL AND SMALL VESSELS SMALL AND
INVOLVED AND VEINS MEDIUM SIZED (ARTERIES, MEDIUM SIZED
VESSELS CAPILLARIES, ARTERIES ONLY
VENULES)
SPECIFIC TRIAD: UPPER EXTRA PULMONARY PULMONARY
FEATURE AND LOWER AIR VASCULAR CAPILLARIES ARTERY NOT
WAYS WITH GRANULOMA INVOLVED, INVOLVED,
KIDNEY LESIONS GNs +NT ANEURYSMS
LABORATORY C-ANCA(>90%) p-ANCA (>48%) p-ANCA (75%) HEP B
FINDINGS FALSE +VE ANTIGENEMIA,
REPORTED HAIRY CELL
LEUKEMIA

27. LARGE SIZED VESSELS
VASCULITIS SYNDROME

28.  TAKAYASU’S ATERITIS  GIANT CEL ATERITIS
(NON-SPECIFIC AORTO-ARTERITIS) (TEMPORAL ARTERITIS)
 SPECIFIC FEMALE SEX MORE COMMON IN FEMALE
PREDILICTIONS
MORE COMMON IN WESTERN
MORE COMMON IN ASIA COUNTRIES
M:F- 1:1.6 (INDIA) RARE IN INDIA
1:9 (JAPAN)
AGE: 15-25 YEARS
AGE: MORE THAN 50 YEARS
C/F: GENERAL SYMPTOMS WITH
VASCULAR SYMPTOMS
 HYPERTENSION IS MOST COMMON ASSOCIATED WITH POLYMYALGIA
PRESENTATION. RHEUMATICA.
DISEAESE CLASSIFY ON THE BASIS OF
SITE OF INVOLVEMENT OF AORTA AND DISEASE INVOLVES
ITS BRNCHES. CHARECTERISTICALLY ONE OR MORE
BRANCHES OF CAROTID ARTERY.
MIXED INVOLVEMENT IS THE

29. CLASSIFICATION OF TAKAYASU’S ARTERITIS

31. SUMMARY
• PATIENT SUSPECTED FOR PRIMARY VASCULITIS.
• RULE OUT THE CAUSES WHICH CAN MIMIC VASCULITIS.
• LOOK FOR DEMOGRAPHIC CHARACTERISTICS LIKE AGE,SEX,
ETHINICITY,SMOKING STATUS.
• DETERMINE THE SIZE OF VESSELS INVOLVED AND CATEGORIES THE
TYPE OF VASCULITIS.
• EXTENT OF ORGAN DAMGE TO BE ASSESSED.
• SPECIFIC LABORATORIES WORK UP REQUIRED FOR CONFIRMATION OF
DIAGNOSIS.
• DECIDE THE APPOPRIATE TREATMENT

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