a practical approach to vasculitis and management

1. VASCULITIS
A PHYSICIAN’S PERSPECTIVE
Dr A P Naveen Kumar
DNB FICP
Chief Specialist – General Medicine
Visakha Steel General Hospital

2. • 54 Male
• Fever joint pains loss of weight – 2 months
• Severe pain in the tips of fingers and toes
• Tingling in lower limbs rt > lt
• Developed ulcers in his mouth
• Anemic TC 4200 ESR 110
• LFT RFT -N

4. • CTD
• Solumedrol
• ANA Negative
• SLE Antibodies negative
• ANCA positive
• Azathioprine
• Rituximab

5. INTRODUCTION
• Vasculitis is a heterogenous group of disorders characterised
by inflammatory destruction of blood vessels
• Inflammed blood vessels are liable to occlude, rupture or
develop a thrombus leading to ischemia of tissues and
organs supplied by the vessel
• Because of multisystem involvement , it begins with non
specific symptoms and has a tendency to affect multi organs
• Involves immunological mechanisms and can result in variety
of presentations depending upon the type and size of the
vessel involved

7. GENERAL DIAGNOSTIC APPROACH
Five important questions to ask
1.Is this a condition that could mimic the presentation of vasculitis
2. Is there a secondary underlying cause
3. What is the extent of vasculitis
4. How do I confirm the diagnosis
5. What specific type of vasculitis is this

8. 2012 Revised Chapel Hill Nomenclature

10. DRUGS
• Pencillin
• Cephalosporin
• Sulfonamide
• Thiazides
• Phenytoin
• Allopurinol

11. Systemic inflammatory response
Results from release of chemical mediators from
inflammed vessels
• Fever
• Night sweats and weight loss
• Arthralgia and myalgia
• Lab - Normocytic normochromic anemia , Leucocytosis ,
Thrombocytosis ,raised ESR and CRP
Conversely systemic systems are not seen
in localised forms of vasculitis

12. Large vessels
Aorta and its
Major branches
Medium vessels
Visceral Arteries
Muscular Arteries
Small vessels
Intraparenchymal
Arteries Arterioles
Capillaries
Limb claudication Ulcers Purpura
Assymetric blood pressure Livedo reticularis Urticaria
Bruits Digital gangrene Glomerulonephritis
Aortic dialatation Mononeuritis multiplex Alveolar hemorrhage
Renovascular Hypertension Microaneurysms Splinter hemorrhages
Absence of pulses Uvietis Episcleritis Scleritis

14. • 44 male
• PUO
• Only cervical lymph nodes
• CT chest and abdomen normal
• Leukocytosis
• Increased ESR

15. AORTO ARTERITIS

16. TAKAYASU ARTERITIS
• 22 male presented with high BP
• Clinically he had aortic regurgitation
• On evaluation found to have Renal
artery stenosis
• Finally it turned out to be
Aortoarteritis
• Renal artery stenting done and he
did fine
• Aortic valve replacement

17. • 48 Female
• Presented with decreased vision in right eye
• HTN
• Investigated
• Biochemistry – N
• ANA SLE –N
• MRA – N
• Eye examination-AION

19. • Opined to r/o CTD
• Inspite of negative workup gave three doses of
Solumedrol
• No significant improvement in vision
• Followed up
• Found that her BP is increasing
• Right Radial artery weak pulsation

22. • Started MMF
• Steroids and tapered off

23. Size of blood vessel Blood vessel
involved
Clinical features
Small vessel
vasculitis
Cutaneous post-
capillary venules
Palpable purpura
Glomerular
capillaries
Hematuria, red cell casts in
urine,proteinuria and decline in renal
function
Pulmonary
capillaries
Lung hemorrhage manifesting as
breathlessness, hemoptysis and
widespread alveolar shadowing on chest
radiograph
Medium vessel
vasculitis –small and
medium sized arteries
Small cutaneous
arteries
Necrotic lesions and ulcers , nail fold
infarcts
Epineural arteries Mononeuritis multiplex
Mesentric artery Abdominal pain, GIT bleeding and
perforation because of gut infarction
Branches of coeliac
artery
Infarction of liver ,spleen or pancreas

24. Size of blood
vessel
Blood vessel
involved
Clinical features
Renal artery Renal infarction
Coronary artery MI or Angina ,coronary artery
aneursym,ischemic cardiomyopathy
Small
pulmonary
arteries
Necrotic lesions leading to cavitating lung
shadows on chest radiograph
Small arteries in
ear, nose and
throat region
Nasal crusting , epistaxis , sinusitis ,deafness ,
stridor because of sub glotic stenosis
Large vessel vasculitis
Aorta and branches
Extra cranial
branches of
carotid
Temporal headache ( temporal artery ),
Blindness ( ophthalmic artery ),
Jaw claudication ( vessels supplying muscles
of mastication )
Thoracic aorta
and its branches
Limb claudication , absent pulses and
unequal blood pressure, bruits , thoracic
aortic aneursyms

26. Vasculitic mimickers
• Infections
SBE
Meningitis- Neisseria
Viral infections
• Malignancies
• CTD
SLE
APLA
RA
• Occlusive vascular disease
Atrial myxoma
Cholesterol embolism
Mycotic infections
Ergotism

27. • 44 female
• Severe pain in neck and pain and numbness in
right tips of all fingers
• Relentless pain and more in the night
• Right radial pulse weak
• No deficit

29. • 56 Female
• Recurrent URTI
• Malaise weakness loss of weight
• Breathlessness
• Pansinusitis – took treatment

31. • Underwent nasal endoscopy
• Biopsy s/o granulomas
• CECT Chest done
• Shows ILD like picture
• ANCA strongly positive
WEGENER’S GRANULOMATOSIS –
GRANULOMATOSIS with POLYANGITIS GPA
• Solumedrol
• Pulse cyclophosphamide

33. • 58 female T2DM
• Recurrent attacks of fever
• Treated with antibiotics recovering
• Cough ,vomitings and loss of weight
• Examination - right axillary lymph node
• FNAC – non specific lymphadenitis
• Leucocytosis , ESR 75 , increased transaminases

34. • Mammography –normal
• CT Chest – multiple lymph nodes in axilla,
lungs –normal, bulky thyroid
• Bone marrow study –megakaryocytic
hyperplasia ,no malignancy

35. PET CT Scan

36. Lymph node excision biopsy

37. SLE
• ANA – Positive
• DsDNA – 199
• Smith positive
• C3 and C4 decreased
• Started on steroids
• Azathioprine added
• Doing well

38. 46 Female diabetic
Presented with peripheral
neuropathy
Joint pains swelling of both
ankles
Rash over the legs
Malaise and low grade fever
NCV – bilateral sensory
axonal neuropathy
Rapid onset of symptoms

39. PURPURA

41. • PUO
• Swelling of eyes lips and oral ulcers
• Bilateral cervical lymphadenopathy
• Multiple subcutaneous nodules
• Hepatosplenomegaly
• Leucopenia and thrombocytopenia
• HTG increased Ferritin
• Hemophagocytosis

42. HEMOPHAGOCYTIC
LYMPHOHISTIOCYTOSIS

46. LIVEDO RETICULARIS

48. MANAGEMENT
• Steroids
• Immunomodulants
• Biologicals

49. SUMMARY
• A comprehensive approach is needed for diagnosis
• Prognosis and therapy depend upon on the extent of
organ involvement
• High degree of suspicion is necessary
• Cutaneous involvement is a universal finding
• Mortality has been reduced with aggressive therapy
however morbidity is still high