Wegener's granulomatosis

Alternative eponyms

Related people

An uncommon disorder characterized by necrotizing vasculitis, granulomatous lesions of the entire respiratory tract, and glomerulonephritis.

Description

An uncommon disorder characterized by necrotizing vasculitis, granulomatous lesions of the entire respiratory tract, and glomerulonephritis. The lesions closely resemble those in polyarteritis nodosa. The symptoms are weakness, malaise, progressive weight loss, purulent rhinitis, sinusitis, polyarthralgia, ulcerations of the nasal septum, signs of severe progressive renal disease, and fever. The disease commonly occurs in mid-adult life. Either sex may be affected. If it is not treated early, death occurs in a matter of months, most often from kidney damage.

The condition was first described by Peter McBride (1854-1946) in 1897. The pathological-anatomical picture was described by Heinz Karl Ernst Klinger (born 1907), a German medical student, in 1931. Detailed description by Friedrich Wegener in 1936 and 1939.

A limited form has been known as Carrington-Liebow syndrome. See under C. B. Carrington.
A lymphoid variant has been known as Liebow-Carrington syndrome. See under Averill Abraham Liebow, American physician, 1911-1978.

Bibliography

  • P. McBride:
    Photographs of a case of rapid destruction of the nose and face.
    The Journal of Laryngology, Rhinology, and Otology, London, 1897, 12, 64-66.
  • H. Klinger:
    Grenzformen der Periarteriitis nodosa.
    Frankfurter Zeitschrift für Pathologie, Wiesbaden, 1931, 42: 455-480.

  • F. Wegener:
    Über generalisierte, septische Gefässerkrankungen.
    Verhandlungen der deutschen pathologischen Gesellschaft, Stuttgart, 1936, 29: 202. Report of 3 cases. Über eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren.
    Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1939, 102: 36-38.
  • G. C. Godman, Jacob Churg:
    Wegener's granulomatosis. Pathology and review of the literature.
    AMA. Archives of Pathology, Chicago, 1954, 58: 533.
    Godman and Churg introduced the term Wegener's granulomatosis.
  • J. Churg, L. Strauss:
    Allergic granulomatosis, allergic angiitis, and periarteriitis nodosa.
    American Journal of Pathology, Philadelphia, 1951, 27: 277-294.
    Churg-Strauss disease.
  • C. B. Carrington, A. A. Liebow:
    Limited forms of angiitis and granulomatosis of Wegener’s type.
    American Journal of Medicine, New York, 1966, 41: 497-527.
  • A.A. Liebow, C. B. Carrington:
    The lymphomatoid variant of limited Wegener’s granulomatosis (abstract).
    American Journal of Pathology, Philadelphia, 1969, 55: 78-83.
  • G. S. Hoffman:
    “Wegener’s granulomatosis”: the path traveled since 1931.
    Medicine, Baltimore, 1994, 73: 325-329.

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