Churg-Strauss Syndrome has been renamed Eosinophilic Granulomatosis with Polyangiitis (EGPA)

What’s in a name? New descriptive name may result in more timely diagnoses.

Churg-Strauss Syndrome is in the process of being renamed Eosinophilic Granulomatosis with Polyangiitis (EGPA). The changeover will be gradual and Churg Strauss Syndrome will follow the new name in parenthesis.- i.e.  Eosinophilic Granulomatosis with Polyangiitis (Churg- Strauss).

Several subspecialty organizations including the American College of Rheumatology (ACR), the American Society of Nephrology (ASN) and the European League Against Rheumatism (EULAR) have asked to remove eponyms from the nomenclature of vasculitis for a variety of reasons. Consequently, there has been a movement away from honorific eponyms to more descriptive or etiology (cause) based names for the vasculitic diseases.

In 2011 the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitis revised the classification of vasculitides and approved Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss) as the official name. As this was a consensus reached by vasculitis experts from all over the world, it is likely that this new nomenclature will also be supported by national and international subspecialty organizations and medical journals.

Churg-Strauss Syndrome was first described by Drs. Jacob Churg and Lotte Strauss at Mount Sinai Hospital in New York City in 1951 and was named after them. To further complicate the naming of this disease, Churg and Strauss termed the syndrome “allergic granulomatosis”, which it is still sometimes called.

Although some may find the new name, Eosinophilic Granulomatosis with Polyangiitis, difficult to pronounce, it will also be referred to as EGPA which is much easier to remember and pronounce. Broken down, the new name translates to: “Eosinophilic (a type of white blood cell that proliferates and cause damages in CSS), “Granulomatosis” (multi-focal necrotizing inflammation in which inflammatory cells are arranged in a granular fashion) and“polyangiitis” (inflammation involving multiple blood vessels of different size and type).

This choice of descriptive wording makes the nomenclature more consistent with new or established names of the two other related diseases, microscopic polyangiitis (MPA) and Granulomatosis with Polyangiitis (Wegener’s, GPA). The polyangiitis is similar in all three illnesses. GPA has the necrotizing granulomatosis, but eosinophils play no significant role. All three syndromes often have autoantibodies in the blood, called anti-neutrophil cytoplasmic antibodies (ANCA). Therefore, all three illnesses are often grouped together and also referred to as ANCA-associated vasculitis. However, the presence of ANCA is much less common in EGPA than in GPA and MPA.

The CSSA supports the name change with the belief that it will help with more timely diagnoses and with correctly categorizing the disease. It may also help in creating an ICD code specific to EGPA. A gradual changeover from one name to another and the combining of the two names will help greatly in the transition period.

May 2012
Reviewed by Dr. Ulrich Specks and Dr. Eric Matteson, Mayo Clinic. Rochester, MN

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