What is the historical background of granulomatosis with polyangiitis (GPA)?

Updated: Aug 31, 2021
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

In 1897, Peter McBride likely gave the first written description of a patient with granulomatosis with polyangiitis (GPA). In 1931, Klinger described a 70-year-old physician with constitutional symptoms, joint symptoms, proptosis, widespread upper respiratory tract inflammation leading to saddle nose deformity, glomerulonephritis, and pulmonary lesions. (See Presentation.)

In 1936, Dr. Frederich Wegener reported three patients with similar clinical features and published his findings on their distinct clinical and histopathologic findings, leading to the eponymous designation of the disease.

In 1954, Goodman and Churg provided the definitive description of GPA upon their identification of a triad of pathological features that characterize the disease, including (1) systemic necrotizing angiitis, (2) necrotizing granulomatous inflammation of the respiratory tract, and (3) necrotizing glomerulonephritis. (See Etiology, Presentation, and Workup.)

The American College of Rheumatology, European League Against Rheumatism, and American Society of Nephrology have recommended a gradual shift from honorific eponyms to disease-descriptive or etiology-based nomenclature. In the case of GPA, the change was triggered by evidence that Wegener was a member of the Nazi party before and during World War II. The recommended alternative name was already being used in the medical literature. [10]

Before the institution of effective therapy, the mean survival of adults with untreated GPA was only 5 months, with 82% of patients dying within the first year and 90% of patients dying within the second year. Despite improvement with the use of corticosteroids, the mean survival time was increased only to 12.5 months.

With the advent of cytotoxic therapy for GPA, patient survival markedly improved. In 1983, Fauci et al reported a 93% complete remission rate in 85 patients (mean age 43.6 y, range 14-75 y) treated with prednisone and oral cyclophosphamide. (See Treatment and Medication.) [2]


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