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Churg-Strauss syndrome: diagnostic difficulties and pathogenesis
Erdal Diri1, Dolores M Buscemi, Kenneth M Nugent
1Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock 79430, USA.
The American Journal of the Medical Sciences
|February 18, 2003
Summary
Churg-Strauss syndrome can mimic rheumatoid arthritis, presenting with joint pain and a rash. Early diagnosis and treatment are crucial for managing this rare vasculitic condition.
Area of Science:
- Rheumatology
- Neurology
- Dermatology
Background:
- Churg-Strauss syndrome (CSS), also known as eosinophilic granulomatosis with polyangiitis, is a rare systemic vasculitis.
- It is characterized by asthma, eosinophilia, and systemic vasculitis, often affecting small to medium-sized vessels.
- CSS can present with diverse clinical manifestations, making diagnosis challenging.
Observation:
- A 52-year-old woman with chronic obstructive pulmonary disease presented with polyarthritis and a purpuric rash.
- She had a history of recurrent ear infections, leukocytosis with eosinophilia, elevated rheumatoid factor, and positive antineutrophil cytoplasmic antibodies.
- Skin and sural nerve biopsies confirmed leukocytoclastic vasculitis and vasa nervosum vasculitis, respectively.
Findings:
- The patient's presentation mimicked rheumatoid arthritis due to symmetrical polyarthritis.
- Recurrent staphylococcal infections were suspected as a potential trigger for the vasculitic process.
- The diagnosis of Churg-Strauss syndrome was established based on clinical, laboratory, and biopsy findings.
Implications:
- This case highlights that Churg-Strauss syndrome can present with symptoms suggestive of rheumatoid arthritis.
- It underscores the importance of considering CSS in patients with unexplained arthritis, rash, and eosinophilia, especially with a history of infections.
- Timely diagnosis and appropriate immunosuppressive therapy, including corticosteroids and azathioprine, are vital for managing CSS and preventing neurological complications like foot drop.