small vessel vasculitis

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