Vasculitis - Wegners, churg strauss,PAN, Temporal arteritis, Takayasu, Kawasaki : Incidence, Epidemiology, Pathogenesis, Clinical Features, Diagnosis, Investigations, Management

1. Dr. Subhash Thakur
Sr. Lecturer, CMC, Bharatpur
Date: 14/06/2021

2.  Introduction
 Etiology and pathogenesis
 Types
 Large Vessels
 Medium Vessels
 Small Vessel
 Incidence, Epidemiology,
Clinical Features, Pathology
and Investigations,
Diagnosis and Treatment:
 Wegners granulomatosis:
 Polyarteristis Nodosa
 Churg Strauss Syndrome
 Temporal Arteritis
 Takayasu Arteritis
 Burgers' Disease
 Kawasaki Disease

3.  Inflammatory disorder of blood vessels
 Etiology – Inflammation caused by either
 Autoantibody dependent cytotoxicity (type II hypersensitivity)
 Antigen – autoantibody complex deposition (type III
hypersensitivity)

4.  Categorized by size of vessels involved:
 Large Vessels:
 Giant Cell arteritis (Temporal arteritis)
 Takayasu arteritis
 Medium sized vessels:
 Polyarteristis nodosa
 Kawasaki disease
 Small Vessels:
 Wegner’s granulomatosis
 Buerger disease

5.  Acute inflammation
 Either granulomatous (large vessel
vasculitis), necrotizing most others or
both (wegners)
 Healing by fibrosis

6.  Due to vascular insufficiency due to either
 Narrowing of vessels by inflammation or fibrosis
 Thrombosis and infarction

7.  Small Vessel Vasculitis
 Epidemiology
 Rare, male > female
 Peak incidence, age 40 – 60
 Distribution of disease
 Necrotizing vasculitis with granuloma
 Classically involves the nose, sinuses, lungs and kidneys
 Small size arteries, capillaries and vein

8.  Bilateral pneumonitis with nodular and cavitary pulmonary
infiltrates
 Chronic sinusitis
 Respiratory tract
 Upper: sinusitis, rhinitis
 Lower: hemoptysis
 Renal Disease: nephritis (Necrotizing Glomerulonephritis)
 Focal necrotizing glomerulonephritis
 Crescentic glomerulonephritis

9.  Consider chronic sinusitis
 Nasopharyngeal ulcerations

10.  Lab findings:
 C-ANCA
 Autoantibody against proteinase
3
 Correlates with disease activity
 Diagnosis: Biopsy
 Micro: fibrinoid necrosis,
neutrophils and granulomas

11.  Steroids + Cyclophosphamide
 Prognosis:
 Untreated: 80% 1 year mortality rate
 Treated: 90% long term remission
 Before, when Dr. Wegner discovered it, >95% of patients died with 5
years, NOW
 When diagnosis is confirmed by biopsy and treated with steroids and
cyclophosphamide, >95% of patients survive for >5 years

12.  Medium vessel vasculitis No lung involvement
 Mononeuropathy (foot drop or wrist drop)
 Epidemiology
 Young adult
 male >Female
 Kidneys
 GI tract (angiogram: aneurysm, vague abdominal pain)

13.  Distribution of disease
 Systemic vasculitis – any organ except lung
 Kidney, Heart, GI tract, muscle etc
 Small and medium size arteries

14.  Symptoms are varied and depend
on system involved
 Low grade fever
 Hematuria, renal failure,
hypertension
 Abdominal pain, diarrhea and GI
bleeding
 Myalgia and arthralgia
 Skin: tender subcutaneous
nodules, digital gangrene, livedo
reticularis and subcutaneous
ulcerations.

15.  Segmental necrotizing vasculitis
 Three stages
 Acute lesions: fibrinoid necrosis and neutrophils
 Healing lesions: fibroblast proliferation
 Healed lesions: nodular fibrosis and loss of internal elastic lamina
 Sequale:
 Thrombosis and Infarction
 Aneurysms (Kidneys, Heart and GI tract)

16.  Hepatitis B antigen in 30% of patients
 Perinuclear antineutrophil cytoplasmic
autoantibodies (p-ANCA)
 Autoantibody against
myeloperoxidase
 Correlates with disease activity
 P-ANCA is only found in microscopic
form of polyarteritis (microscopic
polyangitis)
 Diagnosis: arterial biopsy

17.  Hepatitis B + (20-30 % of patients), important to identify coz if you
don’t identify this and give Rx, it will cause flare up of hepatitis
 P-ANCA + (non-specific)
 Biopsy: nerves (confirmatory)
 Angiogram + aneurysms
 Treatment: Steroids + Cyclosphosphamide (Chaug strauss same Rx
too)

18.  Treatment: Corticosteroids and Cyclophosphamide
 Prognosis
 untreated- fatal in most cases
 Treated – 90% long term remission rate

20.  Medium vessel vasculitis
 Variant of PAN
 Associated with bronchial asthma
 Systemic vasculitis with granulomas and eosinophilia

21.  Involves the lung, spleen
and kidney
 Similar to PAN
 Lung involvement
 Asthma

22.  Consider: new diagnosis asthma in older patients
 Esonophilia
 P-ANCA may be present
 Biopsy: Lung

23.  Rx: Steroids + Cyclophosphamide

24.  Large Vessel Vasculitis
 Epidemiology
 Most common form of vasculitis
 Female>male, age> 60 years
 Primarily affects the elderly
population
 Associated with HLA DR-4
 Most common type of vasculitis

25.  Distribution of Disease
 Small and medium sized arteries
 Cranial arteries (temporal, facial,
ophthalmic arteries)
 Aortic arch-giant cell aortitis(uncommon)
 Pathology
 Segmental granulomatous vasculitis
 Multinucleated giant cells and
fragmentation of internal elastic lamina
 Intimal fibrosis with luminal narrowing

26.  Throbbing headache
 Tender, firm temporal arteries
 Visual disturbances
 Blurred or double vision
 Visual loss
 Facial pain
 Fever, malaise, weight loss,
muscle aches, anemia
 Polymyalgia rheumatica: systemic
flu like symptoms and joint
involvement

27.  Lab tests: elevated ESR
 Diagnosis:
 Temporal arterial biopsy
 Classic presentation or rapid onset may be treated empirically
 Biopsy: Temporal arteris, take big chunk as they have skip lesions

28.  Rx: Prednisolone 60 mg
 If the patient has symptom, do not wait for biopsy report because
patient may go blind, just do ESR and start on Prednisolone
 If biopsy comes +, continue with prednisolone, if -, stop prednisolone
 Prognosis:
 Treated: dramatic response to steroids
 Untreated: blindness due to occlusion of ophthalmic arteryESR > 60

29.  A 72 year old woman comes to you with right sided headache for 4 weeks. She does not have a
history of migraine and does not report visual changes. She also has a low grade fever and hip
stiffness. The exam is unremarkable. What is the next step?
 Ans: Next Step is ESR
 If ESR > 110, What is the next step now?
 You confirm the diagnosis by finding the characteristic giant cells on the right temporal artery.
She is taking 60 mg prednisolone and feeling better. What is the next step?
 How do you know if patient is improving?
 Ans: Subjective
 Objective: follow with ESR
 You can decrease prednisolone dose

30.  Epidemiology
 Most common in asia
 Affects young and middle aged women (age 15-45)
 Distribution of disease:
 Medium size to large arteries
 Aortic arch and major branches
 Pathology:
 Granulomatous vasculitis with extensive intimal fibrosis
 Irregular fibrous thickening of the wall of the aortic arch
 Narrowing of the orifices of major arterial branches

31.  Clinical Features
 Loss of pulse in the upper extremity
 Ocular manifestations:
 Visual loss or field defects
 Retinal hemorrhages
 Neurological abnormalities
 Treatment: Steroids
 Prognosis: Variable course

32.  Epidemiology
 Occurs in young male, usually under 40
years old
 Associated with heavy cigarette smoking
 Common in Israel, India, Japan and S
America
 Distribution of disease:
 Small and medium sized arteries and veins
 Involves the extremities

33.  Pathology:
 Recurrent neutrophilic vasculitis with micro abscesses
 Segmental thrombosis leads to vascular insufficiency
 Clinical features: Buerger disease
 Severe pain (claudication) in the affected extremities
 Thrombophlebitis
 Secondary Raynaud phenomenon
 Ulceration and gangrene

35.  Treatment:
 Smoking cessation

36.  Epidemiology:
 Commonly affects infants and young children (<4 years)
 Japan, Huwaii and US mainland
 Clinical Features:
 Acute febrile illness
 Conjunctivitis
 Erythema and erosion of oral mucosa
 Generalized maculopapular skin rash
 Lymphadenopathy

37.  Distribution of disease
 Large, medium sized and small arteries
 Coronary artery commonly affected
 Pathology:
 Segmental necrotizing vasculitis
 Weakened vascular wall may undergo aneurysm formation
 Prognosis:
 Self limited course
 Mortality rate 1-2% due to rupture of a coronary aneurysm or
coronary thrombosis