Difference between revisions of "Vasculitides"

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==Wegener's granulomatosis==
==Wegener's granulomatosis==
===Clinical====
===Clinical===
*Epistasis.
*Epistasis.
*Renal failure - present as ''nephritic syndrome''.
*Renal failure - present as ''nephritic syndrome''.
Line 92: Line 92:
Features:
Features:
*Granulomas typically poorly formed.<ref>PBoD p.747.</ref>
*Granulomas typically poorly formed.<ref>PBoD p.747.</ref>
Image: [http://www.flickr.com/photos/pulmonary_pathology/3734403695/ Wegener's granulomatosis (flickr.com)].


==References==
==References==

Revision as of 02:46, 22 May 2010

This article deals with the vasculitides (singular vasculitis).

Overview

Most common[1]

  • Polyarteritis nodosa (PAN).
  • Microscopic polyangiitis.
  • Wegener's granulomatosis.
  • Predominantly cutaneous vasculitis.
  • Giant cell arteritis (GCA).

Grouping by size

Small vessel vasculitides

  • Predominantly cutaneous vasculitis.
  • Henoch-Schoenlein purpura.
  • Essential cryoglobulinemic vasculitis.
  • ANCA-associated:
    • Wegener's granulomatosis (c-ANCA > p-ANCA).
    • Churg-Strauss syndrome (50% ANCA +ve).
    • Microscopic polyangiitis (usually p-ANCA).

Notes:

  • ANCA = anti-neutrophil cytoplasmic antibodies.

Large vessel vasculitides[2]

  • Giant cell arteritis (AKA temporal arteritis).
  • Takayasu's arteritis.

Giant cell arteritis

  • AKA temporal arteritis.

Clinical

Features:

  • Classic finding: jaw claudication, in a patient older than 50 years.
  • Other findings: headache, vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.

Work-up:

  • CRP, ESR, temporal artery biopsy.

Treatment:

  • Treat right away with high dose steroids.

Micrograph

Features:

  • Classical: granulomas.

Takayasu's arteritis

General:[3]

  • Disease of medium/large arteries.
    • Classically involves the aortic arch (leading to decreased pulses in the upper limbs).
  • Typically in patients <40 yrs old.
  • Usually asian.

Microscopic

Features:[4]

  • Adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum.
  • Mononuclear inflammation in media.
  • Granulomas, giant cells.
  • +/-Patchy necrosis of media.

Polyarteritis nodosa

  • Abbreviated PAN
  • Involves small and medium sized vessels.
  • Often - renal vessels, mesenteric vessels.[5]
  • Strong association with hepatitis B (see medical liver diseases); ~1/3 of patients with PAN have HBV.

Serology:

  • ANCA is usually negative.

Microscopic

Features:

  • Inflammatory cells (neutrophils, lymphocytes) within the tunica media.
  • Necrosis: dead vessel wall - pink anucleate stuff, nuclear debris (black specks of nuclear material).

Wegener's granulomatosis

Clinical

  • Epistasis.
  • Renal failure - present as nephritic syndrome.
    • Renal biopsy: crescentic glomerulonephritis (AKA rapidly progressive glomerulonephritis).
  • Pulmonary hemorrhage.

Serology:

Notes:

  • Pulmonary hemorrhage syndromes:[7]
    • Goodpasture syndrome.
    • Idiopathic pulmonary hemosiderosis.
    • Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener's granulomatosis).
    • Systemic lupus erythematosus.

Microscopic

Features:

  • Granulomas typically poorly formed.[8]

Image: Wegener's granulomatosis (flickr.com).

References

  1. TN05 RH3.
  2. TN05 RH20.
  3. PBoD P.538.
  4. PBoD P.538.
  5. Klatt. AOP P.14.
  6. TN05 RH6.
  7. PBoD p.745.
  8. PBoD p.747.