History of Wegener’s

Wegener’s granulomatosis (GPA or Granulomatosis with polyangiitis) was thought to have been first described in 1897 by Scottish otolaryngologist (ENT specialist) Peter McBride, who wrote an article for the British Medical Journal about mid-facial granuloma syndrome. We now recognise this disease as a separate distinct entity to GPA, called Stewart’s Midline Granuloma or lethal midline granuloma. In 1931, German physician Heinz Klinger reported a patient with upper airways inflammation, joint pains, kidney disease and a deformity of the nose. A few years later, in 1936 Freidrich Wegner and his medical student noted three patients with similar findings and recognised this as a unique clinical syndrome. He titled his paper “Rhinogenic (relating to the nose and sinuses) granulomatosis with special involvement of the arterial system and kidneys”.

Before the 1950s there were no truly effective therapies for GPA. Unfortunately mortality rates were high. In the sixties, corticosteroid use revolutionised treatment of many conditions and the survival rate of those suffering from GPA improved dramatically, but complication rates were still high. Disease remission only became possible in the 1970s, when the National Institute for Health (NIH) trialed Cyclophosphamide. Since then, various other treatments have emerged but Cyclophosphamide has been the cornerstone of therapy for many decades. However research continues to better understand this complex vasculitic syndrome and how we can achieve a lasting cure.