Churg-Strauss Syndrome

Churg-Strauss Syndrome

Surya V. Seshan, MD

Renal biopsy from a patient with CSS shows circumferential, transmural necrotizing arteritis surrounded by numerous eosinophils and by lymphocytes. Eosinophils invade the media image.

Periodic acid-Schiff stain of a glomerulus in CSS reveals segmental necrosis image, a cellular crescent in Bowman space, and rupture of Bowman capsule image with periglomerular inflammation.



  • Churg-Strauss syndrome (CSS)


  • Allergic granulomatosis and angiitis

    • Defined by autopsy study in 1951 by Churg and Strauss


  • American College of Rheumatology (ACR) criteria differentiate various forms of vasculitides once diagnosis of vasculitis is made

    • CSS diagnosis requires 4 of 6 following features

      • Asthma

      • Eosinophilia > 10%

      • Extravascular eosinophil infiltration

      • Pulmonary infiltrates

      • Paranasal sinus changes

      • Peripheral neuropathy

  • Chapel Hill Consensus Conference (pathology)

    • Criteria are diagnostic and generally exclude other forms of systemic vasculitides

    • Eosinophil-rich inflammatory infiltrate and granulomatous inflammation involving respiratory tract

    • Eosinophil-rich necrotizing vasculitis affecting small to medium-sized vessels

    • Associated with asthma and peripheral eosinophilia

  • Classified with antineutrophilic cytoplasmic antibody (ANCA) diseases


Environmental Exposure

  • Trigger agents: Allergens, vaccinations, or drugs


  • Associated with HLA-DRB4 gene, risk factor


  • Primarily cell-mediated tissue and vascular injury

    • CD4(+) T cells secrete interferon-gamma (Th-1 cytokine), promoting granulomatous inflammation

    • Eosinophil activation via IL-4, IL-5, and IL-13 secretion and CD95-CD95L pathway

    • Tissue damage by eosinophils due to release of cytotoxic eosinophilic cationic and major basic protein

    • Eotaxin-3 from endothelial cells chemotactic for eosinophils

  • ANCA probably contributes to pathogenesis

    • ANCA titers correlate with disease activity



  • Incidence

    • Rare: 3-11/1,000,000 worldwide

    • 16-27% have renal involvement from 3 large series

    • Lesser frequency and milder disease than other small vessel vasculitides involving kidney

  • Age

    • Peak age: 40-60 years; mean: ˜ 48 years

    • Younger adults may also be affected

  • Gender

    • No gender predilection

  • Ethnicity

    • No ethnic predisposition


  • Multisystemic disease

  • 4 phases

    • Allergic

      • Constitutional symptoms

      • Asthma

      • Rhinitis

    • Eosinophilic

      • Peripheral eosinophilia

      • Tissue eosinophil infiltration (e.g., gastrointestinal, sinusitis)

      • Heart

    • Vasculitic

      • Skin purpuric rash

      • Peripheral neuropathy

      • Cerebral vessels

      • Lung

      • Kidney

    • Post vasculitic

      • Sequelae related to major organ damage and hypertension

  • Renal disease

    • Higher incidence in ANCA(+) cases

    • Progressive renal insufficiency

    • Acute renal failure (less frequent)

    • Hematuria (mild to severe)

    • Subnephrotic proteinuria, usually < 1.0 g/d

    • Asymptomatic (isolated) urinary abnormalities

    • Obstructive uropathy caused by ureteral stenosis

Laboratory Tests

  • Anemia, leukocytosis

  • Peripheral eosinophilia (10-20%)

    • May be lower or normal in patients previously treated with steroids for asthma

  • Elevated erythrocyte sedimentation rate

  • ANCA (40-70% positive)

    • Mainly p-ANCA (antimyeloperoxidase antibodies [MPO]); rarely c-ANCA or atypical

  • Rheumatoid factor (25% positive)

  • Elevated serum IgE levels and immune complexes containing IgE

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Churg-Strauss Syndrome
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