Very under-diagnosed entity, covers the doubtful grey areas as well.

1. Vasculitis- Classification,
secondary forms, mimickers
Dr. Shinjan Patra

2. Introduction
• Vasculitis- A clinico-pathologic process
characterized by inflammation of and damage
to blood vessels.
• Vessel lumen is usually compromised and
associated with ischemia of the tissues.
• May be the sole manifestation of a disease or
may be a secondary component.

3. When to Suspect a Vasculitis
• Unexplained ischemia:
–Claudication, limb ischemia, angina, TIA,
stroke, mesenteric ischemia, cutaneous
ischemia
–Especially in a young individual

4. Multi-organ dysfunction
• Suggestive features:
–Glomerulonephritis
–Peripheral neuropathy
–Pulmonary hemorrhage
–Sub-acute endocarditis
–Skin manifestations- purpura, urticaria,
livedo reticularis, vesiculo-bullous lesions.

5. Classification
• Primary Vasculitis syndromes- Vasculitis is the
principal feature of the disease.
• Secondary vasculitis syndromes- Vasculitis is a
secondary manifestation of a connective
tissue disorder.

6. Primary Vasculitis syndromes
• Predominantly Large Vessel (Aorta and its
major branches & corresponding vessel
in venous circulation)-
–Giant cell arteritis
–Takayasu’s arteritis
–Cogan’s syndrome
–Behcet’s disease

7. • Predominantly Medium Vessel vasculitis-
smaller than major aortic branches, yet
enough to contain four elements (intima,
elastic lamina, media, adventitia)
–Polyarteritis Nodosa
–Kawasaki’s disease
–Buerger’s disease
–Primary angitis of CNS

8. • Predominantly Small Vessel vasculitis
(includes capillaries, post-capillary
venules, arterioles- less than 500 μ in
outer diameter)
 Immune Complex mediated- Good-
pasture’s disease, HSP, cutaneous
leucocytoclastic angitis, essential
cryoglobulinemia.
 ANCA-associated disorders- Granulomatosis
with polyangitis, Microscopic polyangitis,
Churg-Strauss syndrome.

9. Primary Vasculitis syndromes

10. Comparison of predominant C/F in
these groups-
Large-vessel vasculitis Medium-vessel vasculitis Small-vessel vasculitis
Limb Claudication Cutaneous nodules + ulcers Purpura + urticaria
Asymmetric blood
pressures
Digital gangrene Glomerulonephritis
Absence/ Inequality of
pulses
Mononeuritis multiplex Alveolar hemorrhage
Bruits Renovascular hypertension Uveitis/ scleritis/ splinter
hemorrhages

11. Pathophysiologic mechanisms
• Pathogenic immune complex deposition-
Lupus vasculitis, Hep-C/hep-B asso vasculitis.
• Production of anti-neutrophilic cytoplasmic
antibodies- Granulomatosis with polyangitis,
microscopic polyangitis, churg-strauss syn.
• Pathogenic T-lymphocyte responses and
granuloma formation- Giant cell arteritis,
Takayasu’s arteritis.

12. ANCA- Anti-Neutrophilic
Cytoplasmic Antibodies
• Antibodies directed against neutrophil granule
constituents
• c-ANCA
– Stains cytoplasm (hence “c”)
– Main target antigen: proteinase-3
– Highly specific (>90%) for Wegener’s
• p-ANCA
– Stains perinuclear (hence “p”)
– Main target antigen: myeloperoxidase
– A/w MPA and Churg-Strauss

13. Secondary Vasculitis syndromes
• Specific etiology-
- Drug-induced
- Hep-C associated cryoglobulinemic vasculitis
- Hep-B associated vasculitis
- Malignancy associated
• Associated with systemic disease-
- Lupus vasculitis
- Rheumatoid vasculitis
- Sarcoid vasculitis

14. Drug Induced vasculitis
• Examples- Hydralazine, propyl-thiouracil,
Allopurinol, Thiazides, Gold, Sulfonamides,
Phenytoin, Penicillin.
• Palpable purpura- generalized/ limited to
lower extremities/ urticarial lesions/
hemorrhagic blisters.
• Glomerulonephritis/ pulmonary hemorrhage.
• T/t- Glucocorticoids/ Cyclophosphamide

15. Hep-C associated cryoglobulinemic
vasculitis
• Type 2 & 3 cryoglobulinemia-
monoclonal/polyclonal IgG and IgM.
• C/f- fatigue, arthralgia’s, purpura, raynaud’s
phenomenon, peripheral neuropathy &
nephropathy.
• T/t- Antivirals + Rituximab + low-dose IL-2

16. Hep-B associated Vasculitis
• Associated with polyarteritis nodosa
• Type 2 & 3 cryoglobulinemic vasculitis-
fatigue, arthralgia’s, purpura, raynaud’s
phenomenon, peripheral neuropathy &
nephropathy.
• T/t- Tenofovir/ Entacavir.

17. RA associated vasculitis
• Incidence- less than 1% +
hypocomplementemia
• C/f- petechiae, purpura, digital infarcts,
gangrene, livedo reticularis, painful lower
extremity ulcers, sensorimotor
polyneuropathy.
• Bywater’s lesion- Isolated digital vasculitis
with splinter like lesions in peri-ungual region.
• T/t- Usual DMARD’s + cyclophosphamide + CS

18. Lupus associated vasculitis
• Vasculitic rash in 20% of cases.
• Ischemic events- TIA, CVA, AMI.
• Associated atherosclerosis and dyslipidemia.
• T/t- Immunosuppressive + statins.

19. Paraneoplastic peripheral neuropathies
• Painful symmetric or asymmetric distal axonal
sensorimotor neuropathy.
• SCLC & lymphoma- commonest.
• T/t- CS + cyclophosphamide.

20. Vasculitis Mimickers
Infectious disease-
 Bacterial endocarditis- Janeway lesions, septic
emboli with hemorrhage & infarction.
 Disseminated Gonococcal infection- Petechial
lesions/ papular lesions/ pustular lesions.
 Pulmonary Histoplasmosis
 Coccidioidomycosis
 Whipple’s disease

21.  Syphilis-
 Lyme disease
 Rocky mountain spotted fever
Neoplasms-
• Atrial myxomas
• Lymphoma
• Carcinomatosis

22. Coagulopathies-
• Antiphospholipid syndrome
• Thrombotic/ Immune thrombocytopenic purpura
• Warfarin induced skin necrosis
• DIC
Drug toxicities-
• Cocaine
• Levamisole
• Amphetamines
Others- Sarcoidosis, Amyloidosis, Migraine.

23. THANK YOU