Granulomatosis with polyangiitis

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Granulomatosis with polyangiitis
Diagnosis in short

Granulomatosis with polyangiitis. H&E stain.

Synonyms Wegener's granulomatosis (old term)
LM DDx small vessel vasculitis (inflammatory cells within the vessel wall, vessel wall injury - such as fibrinoid necrosis), granulomas - typically poorly formed
Site blood vessels - see vasculitides

Associated Dx renal failure (due to rapidly progressive glomerulonephritis), pulmonary hemorrhage
Signs epistasis
Prevalence uncommon
Blood work PR3-ANCA (c-ANCA) +ve
Clin. DDx dependent on presentation - nasal lesion: cocaine use; other causes of pulmonary hemorrhage; other causes of rapidly progressive glomerulonephritis

Granulomatosis with polyangiitis, abbreviated GPA, is a type of vasculitis that typically afflicts the lungs and kidneys.

It was previously known as Wegener's granulomatosis, abbreviated WG.

It should not be confused with eosinophilic granulomatosis with polyangiitis, previously known as Churg-Strauss syndrome.

General

  • Autoimmune.

Clinical

Serology:

  • PR3-ANCA (c-ANCA) +ve.[1]

Notes:

Microscopic

Features:

  • Small vessel vasculitis:
    1. Inflammatory cells within the vessel wall.
      • Granulomas - typically poorly formed.[3]
        • Multinucleated giant cells - common. (???)
    2. Vessel wall injury.

Notes:

  • In the lung, the granulomas tend to be centrilobular, as the artery travels with the airway and is centrilobular.
    • It may difficult to find small blood vessels in affected portions of lung.

Images

www

See also

References

  1. Yeung, J.C.; Leonard, Blair J. N. (2005). The Toronto Notes 2005 - Review for the MCCQE and Comprehensive Medical Reference (2005 ed.). The Toronto Notes Inc. for Medical Students Inc.. pp. RH6. ISBN 978-0968592854.
  2. Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 745. ISBN 0-7216-0187-1.
  3. Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 747. ISBN 0-7216-0187-1.