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Important Points
  1. Definition of vasculitis (Ref. Hari. 18th ed., Pg - 2786)
    Vasculitis is a clinicopathologic process characterized by inflammation of and damage to blood vessels. The vessel lumen is usually compromised, and this is associated with ischemia of the tissues supplied by the involved vessel.

Clinical manifestations include

  1. Constitutional signs and symptoms such as fever, myalgias, arthralgias, and malaise.
  2. Local manifestations of downstream tissue ischemia.
  3. Systemic necrotizing vasculitides, several types affects the aorta and medium sized vessels, but most affect small vessels, such as arterioles, venules, and capillaries (designated small vessel vasculitis).

Mechanisms of vasculitis are the direct invasion of vascular walls by infectious pathogens and immune-mediated mechanisms.

Table: Chapel Hill Consensus Classification and Characteristics of Selected Vasculitis
  1. Causes of Large Vessel Vasculitis
    1. Giant cell (temporal) arteritis
    2. Takayasu arteritis (AIIMS Nov 12)
  2. Causes of Medium Vessel Vasculitis
    1. Polyarteritis nodosa (classic polyarteritis nodosa)
    2. Kawasaki disease (AIIMS Nov 12)
  3. Causes of Small Vessel Vasculitis
    1. Wegener granulomatosis (AIIMS Nov 12)
    2. Churg-Strauss syndrome
    3. Microscopic polyangiitis (microscopic polyarteritis)
    4. HSP
Table 326-2 Potential Mechanisms of Vessel Damage in Vasculitis Syndromes (Ref. Hari. 18th ed., Pg- 2786, table 326.2)
Pathogenic immune complex formation and/or deposition
  1. Henoch-Schönlein purpura
  2. Vasculitis associated with collagen vascular diseases
  3. Serum sickness and cutaneous vasculitis syndromes
  4. Hepatitis C–associated cryoglobulinemia vasculitis
  5. Polyarteritis nodosa–like vasculitis associated with hepatitis B
Production of antineutrophilic cytoplasmic antibodies
  1. Granulomatosis with polyangiitis (Wegener's)
  2. Churg-Strauss syndrome
  3. Microscopic polyangiitis
Pathogenic T lymphocyte responses and granuloma formation
  1. Giant cell arteritis
  2. Takayasu's arteritis
  3. Granulomatosis with polyangiitis (Wegener's)
  4. Churg-Strauss syndrome

Antineutrophil Cytoplasmic Antibodies (ANCA).

Two main patterns are recognized:
  1. c-ANCA – it is directed against proteinase-3 (AIIMS Nov 2010) (AIIMS Nov 12) found in Wegener granulomatosis (AIIMS May 2010)
  2. p-ANCA is specific for myeloperoxidase (MPO). It is found in
    1. Microscopic polyangiitis,
    2. Churg-Strauss syndrome
    3. Goodpasture syndrome.
I. Large Vessel Vasculitis
  1. Giant cell (temporal) arteritis (GCA) (Ref. Hari. 18th ed., Pg-2795)
    1. Occurs in patients older than age 50.
    2. Females >> males
    3. Granulomatous arteritis of the aorta and its major branches, with a predilection for the extracranial branches of the carotid artery.
    4. Often involves the temporal artery.
    5. Headache is the most common presenting feature. (AIIMS Nov 2012)
    6. Fever (AIIMS Nov 2012) jaw claudication, weight loss, sweats, anemia can occur.
    7. Often is associated with polymyalgia rheumatica (Pain in the shoulder and pelvic joints).
    8. Vision may be involved due to involvement of retinal artery which is a branch of internal crated artery.
    9. Sudden blindness can occur.
    10. Other cranial ischemic complication include strokes, scalp or tongue infarction.
    11. Raised ESR is a characteristic feature.
    12. Diagnosis is by temporal artery biopsy (Gold Standard Test)
    13. Treatment - Steroids
  2. Takayasu Arteritis (AIIMS Nov 12) (Ref. Hari. 18th ed., Pg-2796)
    1. Granulomatous inflammation of the aorta and its major branches.
    2. Usually occurs in patients younger than age 50, mainly Asian females.
    3. Asymmetric radial pulse and BP difference in the two arms is a feature.
    4. Renal Artery Stenosis is a feature. (AIPG 2010)​
II. Medium – Sized Vessel Vasculitis
  1. Polyarteritis nodosa (classic polyarteritis nodosa) (Ref. Hari. 18th ed., Pg-2794)
    1. Necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules
    2. They are features of Churg Strauss disease.
    3. Aneurysmal dilatations along the involved arteries are characterstic of PAN.
    4. RAS is not a feature. (AIPG 2010)
    5. Lungs and Spleen vessels are not involved.
    6. Numbness of multiple limbs indicates peripheral neuropathy / mononeuritis multiplex.
    7. Digital gangrene forms part of the typical skin findings in PAN.
    8. hypertension is a common manifestation of renal involvement in PAN.
    9. Renal failure can occur.
    10. Diseases associated in PAN (a) Hepatitis B (b) Hairy cell leukemia (Ref. Hari. 18th ed., Pg- 2794)
Clinical manifestations related to organ system involvement in classic polyarteritis nodosa (Ref. Hari. 18th ed., Pg- 2794, table 326.6)
System % Features
Renal 60 Hypertension, Renal failure
Musculoskeletal 64 Arthritis, arthralgia, myalgia
PNS 51 Peripheral neuropathy, mononeuritis multiplex
GIT 44 Abdominal pain, nausea and vomiting, bleeding, bowel infarction and perforation, cholecystitis, hepatic infarction, pancreatic infarction.
Skin 43 Rash, purpura, nodules, cutaneous infarcts, livedo reticularis, Raynaud phenomenon
Cardiac 36 Congestive heart failure, myocardial infarction, pericarditis
Genitourinary 25 Testicular, ovarian, or epididymal pain
CNS 23 Cerebral vascular accident, altered mental status, seizure.
  1. Kawasaki disease (AIIMS Nov 12)
    1. It’s a disease of children below 5 years. (LQ 2012)
    2. Arteritis involving, medium-sized, or small arteries and associated with mucocutaneous lymph node syndrome.
    3. Coronary arteries (LQ 2012) are often involved. Aorta and veins may be involved.
    4. Kawasaki disease is an acute febrile multisystem disease of children
    5. Prolonged fever over 5 days that is unresponsive to antibiotics is seen.
    6. Although the disease is generally benign & self limiting it is associated with coronary artery aneurysm in 25% of cases.
    7. Non suppurative cervical lymphadenopathy is characteristic. (LQ 2012)
    8. Strawberry tongue, skin rash & conjunctivitis are the features.
Treatment of Kawasaki disease.
  1. Acute phase: As soon as diagnosis is made, ideally <10days of disease onset.
    Intravenous immunoglobulin + High dose aspirin.
  2. Subacute phase: After day 14 or once the patient is afebrile for 48 – 72 hours
    1. Low dose aspirin
    2. Corticosteroids are only recommended for patients who have persistent fever despite at least 2 dose of I/V immunoglobulin.
Important Points

Kawasaki disease

  1. Characteristic laboratory findings include:
    1. Increased ESR
    2. Thrombocytosis
  2. Treatment of choice- High dose intravenous immunoglobulins
  3. Prognosis for uneventful recovery is excellent.

Indications of IV Immunoglobulin therapy

  1. Immune deficiencies
    1. X-linked agammaglobulinemia
    2. Hypogammaglobulinemia (primary immune deficiencies)
    3. Acquired compromised immunity conditions (secondary immune deficiencies)
  2. Inflammatory and autoimmune diseases.
    1. Guillain–Barré syndrome.
    2. Dermatomyositisc.
    3. Multiple Sclerosis (MS)
    4. Myasthenia Gravis,
    5. Pemphigus
    6. Wegener's granulomatosis (WG)
    7. Churg-Strauss syndrome
    8. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
  3. Acute infections.
    1. Pediatric HIV
    2. Kawasaki's Disease


III. Small Vessel Vasculitis (Ref. HIIIari. 18th ed., Pg-2789)
  1. Wegener granulomatosis (AIIMS Nov 12)
    1. Granulomatous inflammation (LQ 2012) involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels (e.g, capillaries, venules, arterioles, and arteries).
    2. Necrotizing glomerulonephritis is common.
    3. Triad of upper respiratory tract involvement, lower respiratory involvement and renal involvement. (LQ 2012)
    4. Kidney has Crescentic glomerulo nephritis (AIIMS Nov 12)
    5. Cavity formation in the lungs is a feature (Ref. Hari. 18th ed., Pg-2789)
    6. Eye involvement is not a important feature. (LQ 2012)
  2. Churg-Strauss Syndrome (Ref. Hari. 18th ed., Pg-2793)
    1. Eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels associated with asthma and blood eosinophilia.
    2. Granuloma, & Eosinophilia are the features of Churg-Strauss Syndrome (LQ 2012)
  3. Microscopic polyangiitis (microscopic polyarteritis) (Ref. Hari. 18th ed., Pg-2793)
    1. Necrotizing vasculitis with no immune deposits affecting small vessels (i.e. capillaries, venules, or arterioles).
    2. Necrotizing arteritis involving small and medium-sized arteries may be present.
    3. Mecrotizing glomerulonephritis is common.
    4. Pulmonary capillaritis often occurs.
Renal involvement in microscopic polyangiitis
  1. Pathology in kidney is that of glomerulonephritis
  2. Rapid renal impairment is characteristic
  3. Hypertension is infrequent.

Extra Edge: Differences between PAN & MPA

Clinical features PAN MPA
Kidney involvement
1. Renal vasculitis with infarcts and microaneurysms




Lung involvement
1. Alveolar hemorrhage


Laboratory data
1. HBV – infection

Yes (10%)

Positive (p-ANCA)
1. Abnormal angiogram with microaneurysms
2. Histology
3. Relapses

Necrotizing vasculitis
Necrotizing vasculitis (No granulomas)
  1. Henoch Schönlein purpura (Ref. Hari. 18th ed., Pg-2797)
    1. It is generalized vasculitis causing GN, purpura, arthralgias and abdominal pain, occurring mainly in children.
    2. Small Vasculitis, palpable purpura (AIPG 10)
    3. Skin biopsy specimen can be useful in confirming leukocytoclastic vasculitis with IgA and C3 deposition by immunofluorescence.
    4. Tetrad –
      1. Palpable purpura
      2. Arthritis (Migratory)
      3. Glomerulo nephritis
      4. Abdominal pain
    5. Renal involvement is manifested by hematuria and proteinuria.
    6. Serum IgA is normal, platelet count is normal. (FAQ)
    7. Platelet count is normal.
    8. Serum compliment levels are normal.
    9. Treatment is symptomatic.
    10. Glucocorticoid is used in its treatment.
    11. The prognosis of HSP is excellent.
Important Points
  1. Recurrent episodes of painless gross hematuria represent the classic clinical presentation of IgA nephropathy,
  2. The most common systemic vasculitis in children is HSP, and the most common primary glomerular disease is IgA Nephropathy. H.S purpura and IgA nephropathy may actually represent spectra of same disease and both are associated with elevated levels of IgA.
  3. ANA and ANCA are characteristically Negative and serum complement levels are typically normal.


Extra Edge: Causes of palpable purpura
  1. Emboli (bacterial fungal or parasitic)
    1. Acute meningococcemia
    2. Disseminated gonococcal infection
    3. Rocky mountain spotted fever
    4. Ecthyma gangrenosum
  2. Vasculitis
    1. Leukocytoclastic vasculitis (predominantly cutaneous vasculitis)
    2. Henoch - Schönlein purpura
    3. Polyarteritis nodosa


Important Points:
Comparison of major types of vasculitis
Vasculitis Affected organs Histopathology
1. Cutaneous small-vessel vasculitis Skin, kidneys Neutrophils, fibrinoid necrosis
2. Wegener's granulomatosis Nose, lungs, kidneys Neutrophils, giant cells
3. Churg–Strauss syndrome Lungs, kidneys, heart, skin Histiocytes, eosinophils
4. Kawasaki disease Skin, heart, mouth, eyes Lymphocytes, endothelial necrosis
5. Buerger's disease Leg arteries and veins (gangrene) Neutrophils, granulomas
Important Points:
Disease Entity Associated specific antibody against :
1. Systemic sclerosis DNA Topo-isomerase I {Anti Scl 70 Ab}
2. CREST SYNDROME Anti Centromere
3. Sjogren syndrome Anti SSa and Anti SSb and Anti Fodrin antibody
4. Inflammatory myopathies Anti Jo1 antibody
4. Cold AIHA asso with Mycoplasma pneumonia Anti I-antibody
5. Cold AIHA asso with Infectious mononucleosis Anti i-antibody
6. Behcets syndrome Anti alpha Enolase of endothelial cells and ASCA in late cases
7. Chrohns disease ASCA
8. Rheumatic fever Serum 883- A B-cell alloantigen and
Antibody labelled D8/17
9. Type II MPGN and Partial Lipodystrophy C3 nephritic factor-which binds to alternate complement system C3 convertase and stabilizes it
10. Rheumatoid arthritis Anti CCP antibody
11. Mixed connective tissue disease Anti RNP antibodies
12. HCV infection Anti LKM 1 antibody
13. Autoimmune hepatitis Type 1 ANA
14. Autoimmune hepatitis Type 2a High titre anti LKM
15. Autoimmune hepatitis Type 2b Low titre anti LKM
16. Autoimmune hepatitis Type 3 Antibody to Soluble liver antigen { SLA}
17. Primary biliary cirrhosis Anti mitochondrial-E2 subunit of pyruvate dehydrogenase complex
18. Celiac sprue IgA anti-gliadin
IgA anti endomysial
IgA anti tissue transglutaminase
IgA anti reticulin antibody
19. Myasthenia gravis Anti Ach receptor antibody
Anti Muscle specific kinase antibody {MUSK}
20. Lambert Eaton myasthenic syndrome Anti P/Q type calcium channels
21. Fischer syndrome Anti ganglioside antibody {GQIb}
22. Chronic Hepatitis D Anti LKM III
23. Heparin induced thrombocytopenia Antibody against complex of heparin with Platelet factor 4 membrane protein
24. Polyarticular JRA Rheumatoid factor and Antinuclear antibodies
25. Pauciarticular JRA Antinuclear antibodies only
26. Systemic onset JRA No antibodies
Buerger's disease
Buerger's disease (also known as thromboangiitis obliterans) is a recurring inflammation and thrombosis of small and medium arteries and veins of the hands and feet. It is strongly associated with smoking,

Cutaneous vasculitis is defined as inflammation of the blood vessels of the dermis.
  1. Histopathologic feature is the presence of vasculitis of small vessels.
  2. Postcapillary venules are the most commonly involved vessels;
  3. Capillaries and arterioles may be involved less frequently.
  4. This vasculitis is characterized by a leukocytoclasis, It refers to the nuclear debris remaining from the neutrophils that have infiltrated in and around the vessels during the acute stages.
  5. In the subacute or chronic stages, mononuclear cells predominate; in certain subgroups, eosinophilic infiltration is seen.
  6. Erythrocytes often extravasate from the involved vessels, leading to palpable purpura.
Clinical and Laboratory Manifestations
  1. The hallmark of idiopathic cutaneous vasculitis is the predominance of skin involvement.
  2. Skin lesions may appear typically as palpable purpura;
  3. Other cutaneous manifestations of the vasculitis including macules, papules, vesicles, bullae, subcutaneous nodules, ulcers, and recurrent or chronic urticaria.
  4. The skin lesions may be pruritic or even quite painful, with a burning or stinging sensation. Lesions most commonly occur in the lower extremities in ambulatory patients or in the sacral area in bedridden patients due to the effects of hydrostatic forces on the postcapillary venules.
  5. Edema may accompany certain lesions, and hyperpigmentation often occurs in areas of recurrent or chronic lesions.
  6. There are no specific laboratory tests diagnostic of idiopathic cutaneous vasculitis. A mild leukocytosis with or without eosinophilia is characteristic, as is an elevated ESR.
The diagnosis by the demonstration of vasculitis on biopsy.

  1. Precipitating factor in the cutaneous vasculitis
  2. Glucocorticoids are often used in the treatment of idiopathic cutaneous vasculitis.
  3. Methotrexate and azathioprine

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