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Related Experiment Videos

[The Churg-Strauss syndrome].

Tatjana Ilić, Dragana Popović-Kuzmanović, Anka Milicić

    Vojnosanitetski Pregled
    |June 11, 2002
    PubMed
    Summary

    Churg-Strauss syndrome (CSS) is a vasculitis often linked to asthma and positive ANCA tests. This case highlights a patient diagnosed with CSS despite negative ANCA, emphasizing the need for clinical criteria in diagnosis.

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    Area of Science:

    • Rheumatology
    • Pulmonology
    • Immunology

    Background:

    • Churg-Strauss syndrome (CSS), a form of medium vessel vasculitis, typically affects the lungs and is associated with bronchial asthma, eosinophilia, and positive antineutrophil cytoplasmic antibodies (ANCA) in 55-67% of cases.
    • Diagnosis often relies on a combination of clinical symptoms and serological markers, particularly ANCA positivity.

    Observation:

    • A 60-year-old female patient presented with bronchial asthma, peripheral pulmonary infiltrates, blood eosinophilia, xerophthalmia, tachycardia, chronic rhinosinusitis, and polyneuropathy.
    • Despite fulfilling five clinical diagnostic criteria for CSS, all immunological tests, including various ANCA assays (IIF, ELISA for proteinase 3, lactoferrin, myeloperoxidase, elastase, cathepsin G), were negative.
    • Biopsies of skin and salivary glands did not reveal eosinophilic infiltrates.

    Findings:

    • The patient's presentation met the clinical criteria for CSS, even in the absence of ANCA.
    • Eosinophilic infiltrates were not detected in the biopsied tissues, suggesting a potential variant or subtype of CSS.

    Implications:

    • This case underscores the importance of considering clinical diagnostic criteria for CSS even when serological markers like ANCA are negative.
    • It suggests the existence of ANCA-negative forms of CSS, expanding the diagnostic spectrum of this condition.
    • The successful response to immunosuppressive therapy further supports the classification of this case as ANCA-negative CSS.

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