Kidney nontumor
Vasculitis
Churg-Strauss syndrome


Topic Completed: 28 October 2019

Revised: 28 October 2019

Copyright: 2019, PathologyOutlines.com, Inc.

PubMed Search: Churg-Strauss syndrome[TI] kidney

Aubre Gilbert, B.A.
Nicole K. Andeen, M.D.
Page views in 2019 to date: 439
Cite this page: Gilbert A, Andeen NK. Churg-Strauss syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneychurgstrauss.html. Accessed December 10th, 2019.
Definition / general
  • Systemic, small to medium vessel, eosinophil rich granulomatous vasculitis
Essential features
  • Rare, systemic, small to medium vessel granulomatous vasculitis (Allergy 2013;68:261)
  • Diagnosed clinically by the presence of
    • Asthma
    • Blood eosinophilia exceeding 1,500/mm3
    • Evidence of vasculitis involving 2 or more organs
  • Renal manifestations occur in 45% of those with the diagnosis and include
    • Pauci-immune crescentic glomerulonephritis
    • Renal arteritis, arteriolitis and medullary angiitis
  • Microscopic features include
    • Glomerular crescents
    • Fibrinoid necrosis
    • Necrotizing vasculitis
    • Extravascular eosinophil rich granulomatous inflammation
  • Associated with HLA-DRB4 and antineutrophil cytoplasmic antibodies (ANCA)
Terminology
  • Eosinophilic granulomatosis with polyangiitis (EGPA), Churg-Strauss syndrome
ICD coding
  • ICD-10: M30.1 - polyarteritis with lung involvement (Churg-Strauss)
Epidemiology
Sites
  • Upper airway tract and lungs
  • Kidneys
  • Peripheral nervous system
  • Gastrointestinal tract
  • Heart
  • Skin
Pathophysiology
  • Immune dysregulation
  • Eosinophil infiltration (Ann N Y Acad Sci 2005;1051:121, Rheumatology (Oxford) 2008;47:804)
    • Activated Th2 cells release cytokines IL4, IL13 and IL5
    • IL4, IL13 and IL5 promote eotaxin3 release from endothelial and epithelial cells
    • Eotaxin3 brings eosinophils from the blood stream into tissue
    • Activated eosinophils secrete granules containing eosinophil basic protein and eosinophil derived neurotoxin, damaging tissue
    • Activated eosinophils secrete IL25, which activates Th2
    • Dysregulation of the following immune cells are also implicated: Th1, Th17, IL17A, B cells
  • ANCA induced endothelial damage: ANCA leads to neutrophil degranulation and tissue damage (Ann N Y Acad Sci 2005;1051:121, Rheumatology (Oxford) 2008;47:804)
Etiology
Clinical features
Diagnosis
  • Clinical diagnosis
  • Biopsy of an affected organ with microscopic confirmation: characteristic pathological changes are helpful but not essential to make the diagnosis
  • In 1984, new clinical diagnostic criteria were proposed (Medicine (Baltimore) 1984;63:65)
    • 3 diagnostic criteria, all must be present
      • Asthma
      • Blood eosinophilia exceeding 1,500/mm3 or > 10%
      • Evidence of vasculitis involving 2 or more organs
Laboratory
  • Peripheral eosinophilia (usually > 1,500 cells/μl or > 10%) is present in active disease
  • Perinuclear / antimyeloperoxidase ANCA
  • Renal involvement: hematuria, proteinuria, elevated serum creatinine
  • Emerging biomarkers: eotaxin3 (at cutoff level of 80 pg/ml, the sensitivity and specificity of eotaxin3 for the diagnosis was 87.5% and 98.6%, respectively) (Rheumatology (Oxford) 2011;50:1737)
Prognostic factors
  • Overall 5 year survival rate is 97% (Medicine (Baltimore) 2011;90:19)
  • The following criteria are associated with a higher 5 year mortality (Medicine (Baltimore) 2011;90:19)
    • Age > 65
    • Cardiac symptoms
    • Gastrointestinal involvement
    • Renal insufficiency (serum creatinine > 150 μmol/l)
    • Lack of ear, nose and throat manifestations
Case reports
  • 54 year old man with pauci-immune necrotizing and crescentic glomerulonephritis, accompanied by acute coronary syndrome due to vasospasm (Am J Kidney Dis 2010;56:e5)
  • 58 year old man with polyneuropathy, peripheral eosinophilia, pauci-immune necrotizing and crescentic glomerulonephritis, small bowel eosinophilic vasculitis, without asthma (Saudi J Kidney Dis Transpl 2014;25:402)
  • 59 year old woman with Churg-Strauss syndrome and eosinophilic tubulointerstitial nephritis (Intern Med 2012;51:1555)
  • 67 year old woman with pauci-immune necrotizing and crescentic glomerulonephritis, tubulointerstitial nephritis with eosinophils and peripheral blood eosinophilia without asthma (Am J Kidney Dis 1998;31:1032)
Treatment
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Nicole K. Andeen, M.D.

Crescent

Arteritis

Medullary angiitis

Immunofluorescence description
Immunofluorescence images

Contributed by Nicole K. Andeen, M.D.

Fibrin / fibrinogen

Positive stains
Negative stains
  • Not applicable
Electron microscopy description
Electron microscopy images

Contributed by Nicole K. Andeen, M.D.

Fibrin tactoids

Sample pathology report
  • Kidney, biopsy:
    • Focally necrotizing and crescentic glomerulonephritis, pauci-immune complex type (see comment)
    • Comment: Consistent with this patient's positive ANCA, the acute renal insufficiency and hematuria are due to a focally necrotizing and crescentic glomerulonephritis, pauci-immune complex (vasculitic) type. Active injury is manifest as segmental necrosis or cellular crescents in 36% of glomeruli. Chronic injury is manifest as segmental glomerulosclerosis / scarring in 9% of glomeruli, global glomerulosclerosis in 9% and approximately 10% tubular atrophy and interstitial fibrosis. Extraglomerular vasculitis is not identified.
Differential diagnosis
Additional references
Board review question #1
A 56 year old man presents with acute kidney injury and positive antineutrophil cytoplasmic antibodies (ANCA). Kidney biopsy demonstrates the below finding in 30% of glomeruli, with trace granular mesangial staining for IgA by immunofluorescence microscopy and no deposits by electron microscopy. What is the best diagnosis?



  1. ANCA associated glomerulonephritis superimposed on IgA nephropathy
  2. Antiglomerular basement membrane antibody (anti-GBM) disease
  3. Crescentic IgA nephropathy
  4. Focally necrotizing and crescentic glomerulonephritis, pauci-immune complex type, consistent with ANCA associated disease
Board review answer #1
D. Focally necrotizing and crescentic glomerulonephritis, pauci-immune complex type, consistent with ANCA associated disease

Reference: Churg-Strauss syndrome

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Board review question #2
Criteria for the diagnosis of eosinophilic granulomatosis with polyangiitis / Churg-Strauss syndrome include

  1. Asthma, peripheral eosinophilia, vasculitis
  2. Elevated eotaxin levels
  3. Kidney biopsy demonstrating pauci-immune complex mediated necrotizing and crescentic glomerulonephritis
  4. Positive antimyeloperoxidase antineutrophil cytoplasmic antibodies (MPO ANCA)
Board review answer #2
A. Asthma, peripheral eosinophilia, vasculitis

Reference: Churg-Strauss syndrome

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