Churg-Strauss Syndrome Workup

  • Author: Mehran Farid-Moayer, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Apr 13, 2011
 

Laboratory Studies

  • Hematology
    • Eosinophilia, usually at least 10% eosinophils (or 5000-9000 eosinophils/µL)
    • Anemia
  • Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels
  • Renal tests
    • Elevated serum BUN and creatinine levels in cases of renal involvement
    • Abnormal urine sediment, proteinuria, microscopic hematuria, and RBC casts
  • Antineutrophil cytoplasmic antibodies: ANCA is present in approximately 40% of patients with Churg-Strauss syndrome (CSS). Most of these patients are perinuclear-ANCA (p-ANCA)–positive (antimyeloperoxidase antibodies).[18]
  • Elevated serum IgE levels
  • Hypergammaglobulinemia
  • Positive results for rheumatoid factor at low titer
  • Other immunologic tests
    • Levels of eosinophil cationic protein (ECP), soluble interleukin-2 receptor (sIL-2R), and soluble thrombomodulin (sTM), which is a marker of endothelial cell damage, are elevated.
    • Elevated sIL-2R and ECP levels in Churg-Strauss syndrome indicate an immunoregulatory defect associated with vasculitis and eosinophilia.
  • Eosinophilia in bronchioalveolar lavage (BAL), evident in 33% of cases
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Imaging Studies

  • Chest radiography[19, 20]
    • Pulmonary opacities can be found in 26%-77% of cases of Churg-Strauss syndrome, and films demonstrate no abnormalities in approximately 25% of patients.[20]
    • Localized parenchymal opacities usually are bilateral, peripheral, and patchy.
    • Cavitation is rare.
    • Pulmonary infiltrates may be transient. Occasionally, infiltrates are similar to those observed in patients with chronic eosinophilic pneumonia, or they may be nodular. See the image below. Transient pulmonary infiltrates in a patient with Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS).
    • Extensive air-space opacities in the setting of a drop in hemoglobin levels suggest massive intra-alveolar hemorrhage as a result of pulmonary alveolar capillaritis. Hemoptysis is present in only 45%-66% of cases.
    • Pleural effusions are observed in 5%-30% of cases and can be eosinophilic.
    • Hilar nodal enlargement has occasionally been reported.
  • CT scanning[21, 22]
    • In the limited number of patients with Churg-Strauss syndrome studied with CT scanning, findings include peripheral areas of parenchymal consolidation with ground-glass attenuation similar to that of chronic eosinophilic pneumonia.
    • Much less commonly, parenchymal nodules (from 5 mm to 3.5 cm), with cavitation or air bronchograms, can be observed.
    • Bronchial dilatation and bronchial wall thickening may also be visible.
    • High-resolution CT scanning of chest produces findings that include significant enlargement of peripheral pulmonary arteries with stellate and irregular configuration—a vasculitis pattern.
  • Other imaging studies are indicated for the complications of the disease and specific organ-system involvement, including abdominal CT scanning for pancreatitis, coronary angiography for myocardial ischemia and infarction, and echocardiography for congestive heart failure (CHF).
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Other Tests

  • The following tests are for specific organ-system involvement:
    • ECG for cardiac manifestations
    • Gastrointestinal endoscopy for GI bleeding
    • Electromyelography (EMG) and nerve conduction studies for peripheral neuropathies
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Procedures

  • Biopsy: If local organ involvement exists, obtaining a biopsy of that organ is most helpful in confirming the diagnosis. However, if no localizing finding exists, obtaining nerve or muscle biopsy may be considered. Sural nerve biopsy is the most feasible procedure. In the case of renal involvement, kidney biopsy results may show focal or crescentic glomerulonephritis; however, this pathological change is consistent with, but not diagnostic of, Churg-Strauss syndrome.
    • Skin
    • Lung - Open or video-assisted thoracoscopic biopsy is preferred over transbronchial
    • Renal
    • Nerve
    • Muscle - Muscle biopsy has a sensitivity of around 67% in detecting systemic vasculitis.[23]
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Histologic Findings

The characteristic pathologic changes in Churg-Strauss syndrome, found especially in the lung,[24, 25] include small necrotizing granulomas, as well as necrotizing vasculitis involving small arteries and venules. The granulomas are composed of a central eosinophilic core surrounded radially by macrophages and epithelioid giant cells. Churg-Strauss syndrome affects medium- and small-sized vessels.

Glomerulonephritis is not as common or severe as in Wegener granulomatosis, but, when present, it is usually focal and segmental and indistinguishable from other forms of so-called pauci-immune (without significant tissue deposition of immune complexes) glomerulonephritis.[26, 27]

Eosinophilic granuloma in a patient with Churg-StrEosinophilic granuloma in a patient with Churg-Strauss syndrome (CSS).
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Contributor Information and Disclosures
Author

Mehran Farid-Moayer, MD  Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center

Mehran Farid-Moayer, MD is a member of the following medical societies: American Academy of Sleep Medicine, American Medical Association, and American Thoracic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Spencer T Lowe, MD  Rheumatologist, Private Practice, Peninsula Medical Group, Burlingame, CA

Spencer T Lowe, MD is a member of the following medical societies: American College of Rheumatology, California Medical Association, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

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Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS).
Eosinophilic granuloma in a patient with Churg-Strauss syndrome (CSS).
The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.
 
 
 
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