This document summarizes different types of small vessel vasculitis. It defines vasculitis as inflammation and damage of blood vessel walls. It describes common clinical features and divides vasculitis by vessel size involved - large, medium, small. It provides detailed information on ANCA-associated vasculitis including definitions, types of ANCA, associated diseases, investigations and treatment. Other small vessel vasculitis discussed include immune complex mediated types like SLE and cryoglobulinemia.

1. Small Vessel Vasculitis
Dr. Julfikar Saif
Resident, Phase A
Department of oncology
Violet Unit, Internal Medicine

2. Definition
• Vasculitis is charecterised by inflammation
and necrosis of blood-vessel walls, with
associated damage to skin, kidney, lung, heart,
brain and GIT.

3. Why we need to learn vasculitis?

4. Lets see some common clinical
features
• fever
• weight loss
• Fatigue
• raised inflammatory marker
• evidence of multisystem involvement

5. classification of vasculitis
• According to the size of the vessel involved
1. Large vessel vascculitis
– Takayasu arteritis
– giant cell arteritis/temporal arteritis and
polymyelgia rheumatica

6. Cont…
• Medium vessel vasculitis
– Polyarteritis Nodosa
– Kawasaki disease

7. Cont…
• Small vessel Vasculitis
– ANCA associated vasculitis
– Immune complex mediated vasculitis

9. ANCA associated vasculitis
• Definition
– A life threatening disorder charecterised by
inflammatory infiltration of small blood vessels,
fibrinoid necrosis and the presence of circulating
antibodies to ANCA
– Combined incidence 10-15/1000000 (million)

10. ANCA
• ANCA stands for anti-nutrophil cytoplasmic
antibody
• Are a group of antibody directed against
cytoplasm of granulocyte and monocyte
• Detected by immunoflurocense
• 2 types according to the pattern of staining

11. Cont….
• C-ANCA
• Named for granular
cytoplasmic staining
pattern
• Antibodies directed against
myeloperoxidase (MPO)
• Associated with
GPA/granulomatosis with
polyangitis/wegener’s
granulomatosis

12. Cont…
• P-ANCA
• Named for their perinuclear
staining pattern
• Antibody directed against
proteinase-3 (PR3)
• Associated with Microscopic
polyangitis & EGPA/eosinophilic
granulomatosis with polyangitis

14. Interesting fact…
• Skin lesion more common in small vessel and
medium vessel vasculitis
• Large vessel vasculitis associated with absence
of pulse, claudication, pain etc…

15. ANCA associated vasculitis…scenerio
• A patient presented with (Fever, Wt Loss, Fatigue), skin
lesion, (haemoptysis, hematuria), renal impairment
• A patient presents with (Fever, Wt Loss, Fatigue), skin
lesion, (haemoptysis, but no hematuria),
ENT+ophthalmological features (eye- proptosis,
ophthalmoplegia, diplopia, Ear- deafness,Nose- crusting,
bleeding, sinusitis, collapse of nose, oral ulcer, throat
nodule) less severe renal impairment
• A patient presents with (Fever, Wt Loss, Fatigue), skin
lesion, H/O allergic rhinitis, nasal polyp, asthma, cbc
reveals eosinophilia

16. MICROSCOPIC
POLYANGITIS
P-ANCA
GPA
(WEGENER’S)
C-ANCA
EGPA
(CHURG STRAUSS)
P-ANCA
Prodrome Nasal polyp, allergic
rhinitis, late onset
asthma ***
Acute
presentation
Haematuria ***,
haemoptysis,
(neuropathy), skin
lesion
Mucosal ulcer,
haemoptysis,
proptosis, nasal
problem, skin lesion
Purpuric/nodular skin
lesion, (mono
neuritis multiplex)
Haemoptysis Present present ?
haematuria Present Absent (uncommon) Absent
Glomerulone
phritis
Rapid proliferating
GN
uncommon
GI
involvement
common 50% (messenteric
vasculitis)
Antibody MPO PR3 Both + Eosinophilia

17. Answers…
• 1. A patient presented with (Fever, Wt Loss,
Fatigue), skin lesion, (haemoptysis,
hematuria), renal impairment
• =>>> Microscopic polyangitis

19. Cont…
• 2. A patient presents with (Fever, Wt Loss,
Fatigue), skin lesion, (haemoptysis, but no
hematuria), ENT+ophthalmological features
(eye- proptosis, ophthalmoplegia, diplopia,
Ear- deafness,Nose- crusting, bleeding,
sinusitis, collapse of nose, oral ulcer, throat
nodule) less severe renal impairment
• =>>> wegener’s granulomatosis/ GPA/
granulomatosis with polyangitis

23. Cont…
• A patient presents with (Fever, Wt Loss,
Fatigue), skin lesion, H/O allergic rhinitis,
nasal polyp, asthma, cbc reveals eosinophilia
• =>>> churg strauss syndrome/EGPA/
eosinophilic granulomatosis with polyangitis

24. Work up…investigations
• Routine
– CBC (anemia, eosinophilia- EGPA)
– ESR ↑
– CRP ↑
– Urine R/E (hematuria in Microscopic polyangitis)
– S. Creatinine (microscopic polyangitis, GPA)
– CXR (pulmonary infiltrate/alveolar hemorrhage, effusion)

25. Cont…
• MRI Chest
– Migratory infiltrate in case of GPA***, other
nodules in airway
• Serological
– MPO/P-ANCA↑ (M.P)
– PR3/C-ANCA ↑ (GPA)
– Both+eosinophilia (EGPA)

26. Biopsy…
• Specimen- skin with muscle
• Microscopic polyangitis=necrotising vasculitis,
nongranulomatous inflammation
• GPA= necrotising vasculitis, with
granulomatous inflammation
• EGPA=small vessel vasculitis with eosinophilic
infiltration

27. treatment
• Organ threatening/acute severe disease
– High dose steroid such as pulse I/V methy
prednisolone .5-1g for 3 day then oral .5mg/kg and IV
cyclophosphamide .5-1g 2wkly 3month
– Followed by maintenance with lower dose steroid
and azathiprine/MTX/MMF
– Plasmapheresis in case of fulminant lung involvement
– Oral Cyclophosphamide can be substituted by
Rituximab, equally effective
– Steroid+MTX effective in limited AAV (anca associated
vasculitis) such as indolent skin, lung, sinus disease

28. Follow up
• Chance of relapse
• So regular followup for long term
– CBC
– Urine R/E
– S. Creatinine
– ESR
– CRP
– Lung Function
– C-ANCA, P-ANCA

29. Immune complex mediated vasculitis…
• SLE
• Systemic Sclerosis
• Cryoglobulinemia (hepatitis c associated)
• They are also considered secondary vasculitis

30. SLE
• Incidence from 10% upto 40%
• Risk factor
– Young onset, long term disease, male
• Features
– Usully small, but may involve large & medium sized vessel
– Usually cutaneous (punctate lesion and palpable purpura)
– Mononeuritis multiplex
– Messenteric ischaemia
– Infarction

31. SLE
• Other features
– Raynaud’s phenomenon
– Neuropsychiatric
– Myocarditis
– Serositis
– Leukopenia
– Anti phospholipid syndrome

32. SLE
• Investigation
– Biopsy and/or arteriography
• Treatment
– High dose steroid
– Cyclophosphamide (Oral/pulse; pulse safer)
– MMF
– Rituximab

33. Systemic sclerosis
• Occlusive vasculopathy is common feature
• Vasculitis is less common
– Hard to confirm clinically
– Histopathology required
• Features
– Telangiectasias
– digital ulcers
– tissue ischemia
• Rx- immunosupressant

34. Cryoglobulinemia
• 3 types, but only type II and III associated with
vasculitis
• Immunoglobulin precipitate in cold
• Associated with hepatitis C virus
• Maybe associated with other autoimmune
disease

35. Cryoglobulinemia
• Features
– Rash
– Raynaud’s phenomenon
– Arthalgia
– Neuropathy
• Investigations
– Screening hepatitis B,C.
– Histopathology

36. Cont…
• Treatment
– No consensus
– Treatment of hepatitis c may produce good
outcome
– Use of immunosuppressents maybe associated
with poor outcome

37. Other, antibody associated vasculitis…
• Goodpasture syndrome
– Due to antibody(IgG type) directed against
glomerular basement membrane
• Risk factor
– Exclusively in smoker

38. Cont…
• Feature
– Renal
• Haematuria, glomerulonephritis, HTN
– Lung
• Haemoptysis, tachypnea, hypoxia
– Rash
• DD
– Microscopic polyangitis, GPA/wegener’s
– SLE

39. Cont…
• Investigation
– Biopsy
– Anti GBM antibody
– Maybe ANCA positive
• Treatment
– Plasmapheresis
– Steroid
– immunosupressent

40. Other Small vessel vasculitis
• Leukocytoclastic vasculitis
– Synonym=hypersensitivoty vasculitis
– Cause= idiopathic (50%), collagen disease, drug,
food, malignancy
• Feature
– Papable purpura, urticarial plaque, vesicle, bullae
– Rash are painful/burning
– arthalgia

41. Cont…LCV
• *****HSP/IgA vasculitis is a distinct entity of
LCV
• Investigation
– Biopsy with immune flurocense
– Leucocytoclasis (perivascular nuclear debris of
neutrophil)
– In case of HSP, IgA deposition , not in others

42. Cont…
• Treatment
– Remove inciting agent
– Pain management
– In severe case prednisolone 1mg/kg for 4wk
– 15mg/kg single pulse can be given

43. Other…..behcet’s disease…
• Epidemiology
– Common in silk route countries
– Male predominant
– HLA B51 associated
• Clinical feature
– Recurrent oral ulcer (atleast 3 times in 12 months)
• universal
• Multiple
• Deeper
• Lasts 10-30 days

44. Clinical features…..
• Plus any of the two
– Recurrent genital ulcer
– Eye lesion (uveitis, retinal vasculitis, cells in
vitreous etc)
– Skin lesion ( erythema nodosum,
oseudofolliculitis, acenform nodules etc)
– Positive pathergy test (pustule formation after
prick)

45. Cont…
• Treatment
– Steroid
– Colchicine in case of erythema nodosum
– Thalidomide in case of resistant oral-genital ulcer

46. Changes between 23rd and 22nd
davidson
• ANCA associated vasculitis were advised to be
managed like SLE, but in 23rd they have
specifically stated management

47. source
• Davidson 23rd
• NCBI
• internet