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Related Experiment Videos

[Churg-Strauss syndrome].

N Stübiger1, T Schlote, I Kötter

  • 1Abteilung für allgemeine Augenheilkunde, Universitäts-Augenklinik Tübingen.

Klinische Monatsblatter Fur Augenheilkunde
|April 30, 1999
PubMed
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Churg-Strauss syndrome, a rare vasculitis, can present with asthma, skin lesions, and vision loss due to central retinal artery occlusion. Diagnosis requires characteristic clinical and lab findings, differentiating it from other vasculitides.

Area of Science:

  • Rheumatology
  • Ophthalmology
  • Pathology

Background:

  • Churg-Strauss syndrome (CSS), or allergic granulomatosis with angiitis, is a rare necrotizing vasculitis of unknown cause.
  • It affects small to medium vessels, characterized by eosinophilic granulomas and symptoms like asthma, hypereosinophilia, and fever.
  • Ocular manifestations in CSS are infrequently documented.

Observation:

  • A 53-year-old woman with a history of asthma, lung infiltrates, and sinusitis developed skin vasculitis and mononeuritis.
  • She experienced decreased visual acuity and transient vision loss, with funduscopy revealing central retinal artery occlusion.
  • These symptoms emerged alongside her existing conditions, indicating a potential systemic involvement.

Findings:

  • Laboratory results showed significant blood eosinophilia (20%) and elevated IgE levels (396 kU/l).

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  • Antineutrophil cytoplasmic antibody (ANCA) tests were negative for both p-ANCA and c-ANCA.
  • These findings, combined with clinical presentation, are characteristic of CSS.

    • Implications:

    • The case highlights the importance of recognizing ocular complications, such as central retinal artery occlusion, in CSS.
    • Accurate diagnosis, supported by clinical and laboratory data, is crucial for differentiating CSS from other vasculitides like PAN or Wegener's.
    • Histological examination remains vital for definitive diagnosis when typical manifestations are absent.