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Inflammatory Disease - Vasculitides


  1. Classification (Based on Pathogenesis)
    i. Direct Infection Q
  • Bacterial (e.g., Neisseria)
  • Rickettsial (e.g., Rocky mountain spotted fever)
  • Fungal (e.g., Aspergillosis, Mucormycosis)
  • Viral (e.g., Herpes Zoster - varicella)
  • Spirochetal (eg syphilis) 
  1. Immunologic
    Immune complex mediated Q
  • Infection (HBV, HCV)                   
  • Henoch - Schönleinpurpura
  • SLE & Rheumatoid arthritis           
  • Drug induced - Penicillin, Streptokinase
  • Serum sickness 
  1. Anti neutrophil cytoplasmic Antibody (ANCA) mediated Q
    1. Wegner granulomatosis - CANCA (PR-3) → Anti proteinase 3
    2. Microscopic polyangiitis P- ANCA (MPO) → Anti myeloproximdase
    3. Churg Strauss syndrome 
  2. Direct antibody attack mediated: Q
    1. Good pasture syndrome (anti GBM antibodies)
    2. Kawasaki disease (antiendothelial antibodies)
  3. Cell Mediated Q
    1. Allograft organ rejection    ­       
    2. Inflammatory bowel disease   
    3. Paraneoplasticvasculitis
      1. Giant cell (temporal) arteritis       
      2. Takayasu arteritis   
      3. Polyarteritisnodosa (classic PAN) 
  4. Classification on size of vessel involved
    1. Large Vessel Vasculitis
      1. Giant cell (temporal) arteritis           
      2. Takayasu arteritis 
    2. Medium sized vessel vasculitis:
      1. Classic PAN  
      2. Kawasaki disease
    3. Small vessel vasculitisQ
      1. Wegener granulomatosis              
      2. Churg Strauss syndrome
      3. Microscopic polyangiitis        
      4. Henoch. Schönleinpurpura
      5. Essential Cryoglobulinemia vasculitis    
      6. Cutaneous Leukocytoclasticangiitis
Individual Diseases
  1. Giant cell (temporal) arteritis.
  1. Most common systemic vasculitides
  2. Predilection for extracranial branches of carotid artery: Q often involved temporal, facial also vertebral and opthalmic (blinders).
  3. Giant cell arteritis (temporal arteritis), present with migratory muscular and back pains (polymyalgia rheumatica), dizziness, visual disturbances, headaches, weight loss, anorexia, and tenderness over one or both of the temporal arteries.
  4. The cause of the arteritis (which may include giant cells, neutrophils, and chronic inflammatory cells) is unknown, but the dramatic response to corticosteroids suggests an immunogenic origin.
  5. The disease may involve any artery within the body, but involvement of the ophthalmic artery or arteries may lead to blindness unless steroid therapy is begun.
  6. The erythrocyte sedimentation rate (ESR) is markedly elevated to values of 90 or greater.
  1. Granulomatous - inflammation Q with multinucleated giant cells, fragmentation of internal elastic lamina
  2. Non specific panarteritis
  3. Later: Collagenous thickening of vessel well: organization of thrombus
  4. Cause: NK T Cell mediated injury: immunologic reaction against elastin Segmental nature: Adequate biopsy requires 2-3 mm length of artery, negative biopsy doesn't rule out Q
  1. Takayasu Arteritis:
  1. Female < 40 years
  2. Ocular disturbances, weak pulse of upper limb (pulseless disease) Q
  1. Narrowing of aortic arch at origin of vessels Q
  2. Gross: Irregular thickening with intimal wrinkling
  3. Inv. coronary, pulmonary, Renal vessels         .
  4. Granulomatous change with giant cells
  5. Long-term survival, quiescence in 1-2 years
  6. C/F - Low B. P and weaker pulses in upper extremities
  1. Coldness and numbness of fingers.
  2. Ocular disturbances Q
  3. H. T.
  1. Polyarteritis Nodosa (Classic)
  • Classic polyarteritis nodosa (PAN) is a systemic disease characterized by necrotizing inflammation of small or medium-sized muscular arteries, typically involving the visceral vessels but sparing the small blood vessels of the lungs and kidneys.
  • Histologically, there is intense localized acute inflammation and necrosis of vessel walls with fibrinoid necrosis, and often thrombosis of the vessel with ischemic infarcts of the affected organ.
  • Healed lesions display fibrosis in the walls of affected blood vessels with focal aneurysmal dilations.
  • Clinically, polyarteritis is a protracted, recurring disease that affects young adults.
  • It is a multisystem disease affecting many organs of the body, and this makes it difficult to diagnose unless the vasculitis is recognized by biopsy. 
Symptoms include
a. Fever, b. Weight loss, c. Malaise,
d. Abdominal pain, e. Headache f. Myalgia.

Skin involvement results in palpable purpura.
The etiology is not known, but 30% of patients with classic PAN have circulating hepatitis B antigen in their serum.

  1. Fibrinoid necrosis & inflammatory infiltrate.
  2. Later: Fibrotic thickening
  3. H/P- Transmural inflammation of vessel wall
Later stages
  1. Fibrosis & thickening
  2. Aneurysms
All stage of activity coexist in different vessels or even within the same vessel.
  1. Kawasaki Disease: (Muco-cutaneous Lymph Node Syndrome) Q
  1. Arteritis of large, medium & small arteries (often coronary arteries)
  1. Children & infants « 4 years); Japan Q
  2. Fever, conjunctival & oral erythema & erosion, cervical lymphadenopathy: self limited
  1. 20% CVS sequelae (coronary artery aneurysm, Q thrombosis, myocardial infarction).
  2. Leading cause of acquired heart disease in children in USA
  1. Microscopic Polyangiitis
  1. Microscopic PAN
  2. Hypersensitivity or leukocytoclastic vasculitis
  3. Affects arterioles, capillaries & venules
  4. All lesions of same age
  5. ANCA in majority of cases (p-ANCA) unlike classical PAN
  6. Necrotizing glomerulonephritis & pulmonary capillaritis - common
    Limited to skin - Cutaneous leukocytoclastic vasculitis
  7. Histology: similar to PAN -segmental necrotizing vasculitis.
      Other small vessel vasculitis
    H-S purpura, cryoglobulinemia; similar picture: No ANCA association
  1. Henoch -Schönlein purpura -
  1. Vasculitis with IgA dominant immune deposits affecting vessels (e.g., capillaries, venules arterioles)
  2. Involves skin, gut, glomeruli: associated with arthralgias or arthritis
  1. Churg Strauss syndrome
  1. P-ANCA association 50% pts  Q
  2. Allergic granulomatosis and angiitis  Q
  3. Eosinophil rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis of small to medium sized vessels
  4. Associated with asthma, allergic rhinitis & blood eosinophilia
  5. Coronary arteritis & myocarditis - Mostly cause of disease & death Q
  1. Cutaneous leukocytoclastic vasculitis:
    Isolated cutaneous leukocytoclastic angiitis without systemic vasculitis or glomerulonephritis; no association with p-ANCA Q
  1. Wegener granulomatosis:
    40 yrs. male> female;
Pulmonary infiltrates, renal lesion, chronic sinusitis.
  1. Necrotizing vasculitis characterized by triad of
    1. Acute necrotizing granulomas of URT, LRT or both Q
    2. Focal necrotizing or granulomatous vasculitis
    3. Renal disease Q in form of focal necrotizing, GN or crescentic GN
    4. Necrotizing granulomatous arteritis affecting the upper and lower respiratory tracts and the kidneys is seen in patients with Wegeners granulomatosis.
    5. These areas of necrosis are characteristically large and serpiginous, and exhibit peripheral palisading of macrophages with presence of giant cells. In patients with Wegener’s granulomatosis, the nose, sinus, antrum, and trachea often exhibit ulcerations.
Limited Wegener granulomatosis:
No renal involvement, only respiratory tract involved cANCA:
+ve in 90%: marker of disease activity
H/P - acute and chronic’ inflammation of vessels well
  1. Organization, fibrosis
  2. Few microabscesses
  1. Thromboangiitis obliterans / Buerger disease
    Segmental, thrombosis, acute & chronic inflammation of med. & small sized arteries ( - tibial and radial) may secondarily extend to veins, and nerves of the extremities
    Male, Smokers, < 35 years
    1. Clinical: Intermittent claudication
      1. Gangrene
  1. Abstinence from smoking Relief   Q
  1. Goodpastures syndrome
  • It is another pulmonary hemorrhagic syndrome that may produce hematuria.
  • This syndrome is characterized by the development of a necrotizing hemorrhagic interstitial pneumonitis and rapidly progressing glomerulonephritis because of antibodies directed against the capillary basement membrane in alveolar septa and glomeruli.
  • A linear IgG immunofluorescence pattern is present, which is characteristic of a type II hypersensitivity reaction.
  1. Raynaud disease:
  1. Primary Raynaud’s Phenomenon
  1. Young healthy female, median age - 14 yrs, family history may be positive
  2. Intense vasospasm of small arteries or arterioles digits of hands and feet induced by cold.
  3. Colour: White Blue Red  Q
  4. Exagg. Vasomotor response to emotion or cold
  5. Benign; ulcer & gangrene - Rare
  1. Secondary Raynaud Phenomenon:
  1. Arterial insufficiency of extremities secondary to arteriolar narrowing –
  2. + SLE, scleredema, atherosclerosis, Burger disease.
  3. Age >30 yrs, severe disease.

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