What is the origin of the names Wegener granulomatosis (Granulomatosis with Polyangiitis (GPA)) and Churg Strauss syndrome (Eosinophilic Granulomatosis with Polyangiitis (EGPA))?

Origin of Disease Names: Wegener Granulomatosis and Churg-Strauss Syndrome

Historical Eponyms and Current Nomenclature

These diseases were named after the physicians who first described them, but both have been renamed to descriptive terminology: Wegener's granulomatosis is now called Granulomatosis with Polyangiitis (GPA), and Churg-Strauss syndrome is now called Eosinophilic Granulomatosis with Polyangiitis (EGPA). 1

Wegener Granulomatosis (Now GPA)

Original Description and Naming

• Friedrich Wegener provided a comprehensive description of this disease in the 1930s-1940s, which led to the condition being named "Wegener's granulomatosis" 2
• The disease is characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tract, glomerulonephritis, and variable degrees of small-vessel vasculitis 1

Name Change and Rationale

• In 2010, the name was officially changed from Wegener's granulomatosis to Granulomatosis with Polyangiitis (GPA) due to revelations about Friedrich Wegener's extensive links to Nazism 2
• Wegener and his wife were members of numerous Nazi organizations, and he was rewarded by the German Reich for his stance 2
• He described himself as a frontline combatant in the Nazi invasion of Poland and received numerous accolades from the German Reich that provided professional, material, and personal benefits 2
• His association with Nazism helped him advance his career and attain high social status during and before World War II 2

Current Terminology

• The disease is now referred to as Granulomatosis with Polyangiitis (GPA) in all modern guidelines and medical literature 1, 3

Churg-Strauss Syndrome (Now EGPA)

Original Description and Naming

• Churg-Strauss syndrome was first described in 1951 by Jacob Churg and Lotte Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia 4
• The original description identified the key features of asthma, eosinophilia, and vasculitis that define this condition 4, 5

Evolution of Understanding

• The understanding of this disease has continued to evolve since its earliest documentation in 1951 5
• Key historical advances in diagnosis, classification, and nomenclature have shaped our understanding of this protean disorder over time 5

Current Terminology

• The disease is now called Eosinophilic Granulomatosis with Polyangiitis (EGPA) in contemporary medical literature and guidelines 1
• This descriptive name better reflects the pathological features: eosinophilic infiltration, granulomatous inflammation, and polyangiitis (inflammation of multiple vessels) 1

Clinical Distinction Between the Two Entities

Key Differentiating Features

• GPA is characterized by necrotizing granulomatous respiratory tract lesions and is strongly associated with PR3-ANCA (positive in 80-90% of cases) 3, 6
• EGPA is distinguished by clinical features of atopy, peripheral blood eosinophilia, and tissue eosinophilia, with ANCA positivity in only ~40% of cases (usually MPO-ANCA) 1, 6

Rare Overlap

• While both are distinct entities, genuine overlap between GPA and EGPA has been reported but is exceedingly rare, with only isolated case reports describing simultaneous presentation 7, 8