Granulomatosis with polyangiitis

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

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

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

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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.