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Vasculitis (Pathoma) (Medium Vessel vasculitis (Polyarteritis Nodosa…

- - - - fibrinoid necrosis
    - - clinical signs(different to different organs)
        
        Renal a.
        
        HTN
        
        Mesenteric A.
        
        Abn pain w/ melena
        
        CNS
        
        neurological disturbances
        
        skin lesions
        
        Assd. w/ serum HBsAg
      - Lesions of varying stage are present
        
        string of pearls appearance on imaging
        
        long bv w/ lesions of varying stage
        
        early lesion w/ downstream fibrosis.
        
        possible aneurysm in early lesion secondary to fibrosis weakening
        
        Early lesion: transmural inflammation w/ characteristic fibrinoid necrosis
        
        heals
        
        Massive fibrosis: area of bumpy mass on blood vessel (nodes)
    - - Corticosteroids
      - Cyclophosphamide
      - fatal if not treated
  - - - Clinical presentation: Nonspecific vague symptoms
        
        fever
        
        conjunctivitis
        
        erythematous rash of palms and soles
        
        mnemonic: a kid on a Kawasaki motorcycle touches his palms and soles to a bike, and HR goes up (Coronary involvement)
        
        Enlarged cervical lymph nodes
      - May seem like a viral disorder bc of nonspecific symptoms
        
        Ddx: involvement of coronary A.
        
        Thrombosis w/ MI
        
        Aneurysm w/ rupture
        
        inflamed blood vessel wall causing dilatation and aneurysm
        
        aneurysm incr stress on wall and risk of rupture
      - Treatment:
        
        ASA
        
        interesting since kids w/ viral infections should NOT be given ASA due to risk of Reyes syndrome
        
        how ASA works: inhibit platelet aggregation. Decreasing COX and TXA2
        
        IVIG
        
        Disease is self limited
  - - - vasospasm: discoloration
        
        white to blue to red
    - - damage is irreversible
- - - - mnemonic: WeCener's Granulomatosis
        
        distribution looks like a C
        
        again: nasopharynx, lungs, kidneys
        
        C-ANCA
        
        Key treatment: Cyclophosphamide
    - - Sinusitis or nasopharyngeal ulceration
      - Hemoptysis w/ bilateral nodular lung infiltrates
      - Kidney: Rapidly Progressive GN: Hematuria
        
        elevated serum c-ANCA
        
        correlative to disease activity
        
        Slide has test neutrophil: introduce pt serum
        
        if pt has Ab against neutrophils then see anti-neutrophil cytoplasmic antibodies (present adjacent to nucleus: Perinuclear ANCA)
        
        Ab react in the periphery (cytoplasmic)
        -ANCA)
    - - see characteristic features of granuloma
        
        see epithelioid histiocytes
  - - - want to distinguish this from microscopic polyangeriitis
  - - - Clinical Signs:
        
        Palpable puerperal on buttock and legs
        
        inflammation (palpable) accompanies vasculitis
        
        involvement of GI tract
        
        GI pain and bleeding
        
        involvement of kidneys
        
        Hematuria (IgA nephropathy)
        
        deposition of IgA w/in the mesangium, results in glomerular bleeding
        
        Interesting: Usually occurs following an upper respiratory tract infection
        
        body generates IgA protecting mucosa
        
        IgA deposition drives vasculitis
      - Self limited w/ some recurrence
      - Treatment: steroids
- - - - classic location: Branches of Carotid artery
        
        Temporal a.
        
        headache
        
        Ophthalmic a.
        
        visual disturbances
        
        arteries feeding jaw
        
        jaw claudication
      - Clinical signs
        
        Flu-like symptoms w joint and muscle pain
        
        called polymyalgia rheumatica
        
        ESR is elevated
        
        100
      - Biopsy: Inflamed vessel wall w/ giant cells and intimal fibrosis
        
        Granulomatous Vasculitis
        
        Lesions are segmental
        
        only one area of a long vessel may be involved
        
        consequence: neg biopsy in pt may still have disease
        
        large area of fibrosis in intima
        
        decreased lumen size
        
        Inflammation in vessel wall
        
        Giant cells: Large cell w/ multiple nuclei
      - Treatment: Corticosteroids
        
        high risk of blindness w/o treatment
        
        inflammation of ophthalmic artery
        
        risk of thrombosis in ophthalmic artery
        
        treat if suspected
  - - - same disease as Temporal vasculitis w/ some exceptions
    - - classic: young asian female
    - - Visual and neuro symptoms
      - weak or absent pulse in an upper extremity (pulseless disease)
      - ESR elevated
      - Treatment: Corticosteroids
- - - - disruption of a bv damages endothelial cells
        
        exposure of sub endothelial collagen
        
        activation of coag cascade
        
        thrombus
        
        ischemia
      - damage leading to inflammation
        
        healing
        
        fibrosis
        
        narrowing lumen decreases blood flow
        
        organ ischemia