24- S. CELL VASCULITIS(arterioles, venules and capillaries)

• Medium cell vasculitis


  Affects main visceral vessels, e.g. renal, coronary
• Polyarteritis nodosa (PAN)


  • Predominantly affects middle-aged men
  • Associated with chronic hep B and C (esp. hepatitis B)
  • Due to immune complex deposition & necrotizing arteritis associated with microaneurysms, thrombosis, and infarction
  • Spares the lungs
  • Neurological: foot drop, mononeuritis multiplex, stroke in the young
  • Skin: digital and lower limb ulcers, livedo reticularis, purpura
  • GI: GI bleed, abdominal pain worse with food, N & V
  • Renal: glomerulonephritis, hypertension
• Diagnosis of PAN


  1. Angiography (microaneurysms [beading] in hepatic, intestinal, renal vessels)

  2. Biopsy of an affected organ, often the kidney

  3. High ESR/CRP, P-ANCA in 20%

  4. Screen all for Hep B and C
• Treatment of PAN


  Prednisone +/- immunosuppressive drugs (cyclophosphamide)
• Kawasaki disease


  • Affects children <5 years
• Small cell vasculitis


  Affects small arteries, arterioles, venules and capillaries
• ANCA positive small cell vasculitis


  • Microscopic Polyangiitis
  • Wegner's Granulomatosis
  • Churg Strauss syndrome
• All ANCA positive small cell vasculitis are pauci-immune (normal complement, no or little IF)
• Microscopic Polyangiitis


  • Pulmonary fibrosis
  • Purpuric rash
• Wegner's Granulomatosis


  • Cavity lesions in lungs (necrotizing non-caseating granuloma) à hemoptysis
  • Chest x-ray: Multiple migratory nodular infiltrates with frequent caseation, Ground glass opacities of pulmonary hemorrhage, Reticulo-nodular infiltrates, Airway stenosis / ulceration
  • Sinusitis
  • Otitis media
  • Mastoiditis
  • Oral / gingival
  • Depressed nasal septum
  • Nose bleeds
• Churg Strauss syndrome


  • Asthma
  • Eosinophilia
  • Purpuric rash
• All ANCA positive small cell vasculitis have glomerulonephritis & peripheral neuropathy
• Treatment of ANCA positive small cell vasculitis


  Prednisone & cyclophosphamide
• Behçet's disease


  • A rare multisystem chronic disease of unknown cause and most common in Turkey, Iran and Japan
  • Characterized by recurrent oral ulceration
  • Diagnosis is clinical and requires the presence of oral ulceration and any two of: Genital ulcers, Eye lesions (uveitis, retinal vascular lesions), Skin lesions (erythema nodosum, papulopustular lesions), Or a positive skin pathergy test (skin injury, e.g. Needle prick, leads to pustule formation within 48 hours)
  • Other features: Arthritis, Gastrointestinal ulceration with pain and diarrhea, Pulmonary and renal lesions, Meningoencephalitis, and organic confusional states
• Treatment of Behçet's disease


  Immunosuppressive therapy (steroids, azathioprine, ciclosporin) or occasionally thalidomide
• Henoch–Schoenlein purpura


  • Most commonly seen in children
  • Presents as a purpuric rash, mainly on the legs and buttocks
  • Abdominal pain & bleeding, arthritis, hematuria, nephritis
  • Vascular deposition of IgA immune complexes
  • Onset is preceded by an acute upper respiratory tract infection
  • Recovery is usually spontaneous
  • Give steroids if severe abdominal pain or progressive renal insufficiency
• IgA nephropathy


  Localized form (only in the kidney) of Henoch–Schoenlein purpura
• Cryoglobulinemia


  • Cryoglobulins are immunoglobulins and complement components that precipitate reversibly in the cold
  • May be essential cryoglobulinemia (no underlying disease), associated with infection (ex: hepatitis B and C, HIV), or an autoimmune disease (Sjogren)
  • Mostly associated with Hep B
  • There is involvement of the skin (purpura, arthralgia, and leg & digital ulcers), kidneys (glomerulonephritis, hematuria) and polyneuropathy (peripheral neuropathy, mono-neuritis multiplex)
• Treatment of cryoglobulinemia


  • If due to Hep. C à HCV treatment
  • If essential à steroids + cyclophosphamide
• Don't confuse cryoglobulinemia with cold agglutins (both IgM)
• Both cryoglobulinemia and cold agglutins are immune-complex mediated glomerulonephritis with a granular IF pattern and non-blanching purpuric rash