What is Churg Strauss Syndrome

Churg-Strauss Syndrome, now medically known as Eosinophilic Granulomatosis with Polyangiitis (EGPA), is a rare autoimmune disorder characterized by blood vessel inflammation (vasculitis) and a high count of eosinophils, a type of white blood cell. This condition typically progresses through three phases—allergic, eosinophilic, and vasculitic—and can affect multiple organ systems, most commonly the lungs, sinuses, and skin.

What are the symptoms and body systems affected by Churg-Strauss Syndrome?

Churg-Strauss Syndrome is a multisystem disease. The inflammation caused by Churg-Strauss Syndrome often leads to severe asthma, chronic rhinosinusitis, and nasal polyps. As the condition advances into the vasculitic phase, it may cause nerve damage (mononeuritis multiplex), skin rashes, joint pain, and potential damage to the heart, kidneys, and gastrointestinal tract due to restricted blood flow.

How common is Churg-Strauss Syndrome and who does it affect?

Churg-Strauss Syndrome is exceptionally rare, with an estimated prevalence of 10 to 14 cases per million adults. The average age of onset for Churg-Strauss Syndrome is between 38 and 50 years, though it can occur at any age. Current data suggests no strong gender bias, and it appears to affect individuals across various geographic and ethnic populations globally.

What causes Churg-Strauss Syndrome?

The exact cause of Churg-Strauss Syndrome remains unknown, but it is classified as an ANCA-associated vasculitis. The underlying mechanism involves an overactive immune system that triggers eosinophil infiltration into tissues, leading to inflammation and damage to small and medium-sized blood vessels. Unlike other forms of vasculitis, the high eosinophil count is a hallmark feature that helps clinicians differentiate Churg-Strauss Syndrome from similar inflammatory conditions.

Key clinical features of Churg-Strauss Syndrome

• Asthma: Present in over 95% of patients, often preceding other symptoms by years.


• Eosinophilia: Blood tests consistently show elevated levels of eosinophils (typically >10% of white blood cell count).


• Neuropathy: Peripheral nerve involvement occurs in approximately 75% of those diagnosed with Churg-Strauss Syndrome.


• Community Insight: Currently, 126 people with Churg-Strauss Syndrome are active members of the DiseaseMaps.org community, sharing lived experiences and management strategies.

Next steps

• Consult a rheumatologist or an immunologist specializing in vasculitis.


• Keep a detailed symptom diary to track asthma flares and skin changes.


• Join the DiseaseMaps.org community to connect with others navigating this rare diagnosis.


• Ask your doctor about the Vasculitis Foundation for specialized educational resources.

Medical disclaimer: This content is for informational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Eosinophilic granulomatosis with polyangiitis.


• Orphanet: Eosinophilic granulomatosis with polyangiitis (ORPHA:260).


• Vasculitis Foundation: Information on EGPA/Churg-Strauss Syndrome.


• OMIM (Online Mendelian Inheritance in Man): Entry #617300.