Difference between revisions of "Vasculitides"

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m (→‎Overview: subdivide)
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==Overview==
==Overview==
Most common:<ref>TN05 RH3.</ref>
===Most common<ref>TN05 RH3.</ref>===
*Polyarteritis nodosa (PAN).
*Polyarteritis nodosa (PAN).
*Microscopic polyangiitis.
*Microscopic polyangiitis.
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===Grouping by size===
===Grouping by size===
Small vessel vasculitides:
====Small vessel vasculitides====
*Predominantly cutaneous vasculitis.
*Predominantly cutaneous vasculitis.
*Henoch-Schoenlein purpura.
*Henoch-Schoenlein purpura.
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**Churg-Strauss syndrome (50% ANCA +ve).
**Churg-Strauss syndrome (50% ANCA +ve).
**Microscopic polyangiitis (usually p-ANCA).
**Microscopic polyangiitis (usually p-ANCA).
Large vessel vasculitides:<ref>TN05 RH20.</ref>
*Giant cell arteritis (AKA ''temporal arteritis'').
*Takayasu's arteritis.


Notes:
Notes:
*ANCA = anti-neutrophil cytoplasmic antibodies.
*ANCA = anti-neutrophil cytoplasmic antibodies.
====Large vessel vasculitides<ref>TN05 RH20.</ref>====
*Giant cell arteritis (AKA ''temporal arteritis'').
*Takayasu's arteritis.


==Giant cell arteritis==
==Giant cell arteritis==

Revision as of 02:05, 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

  • Nasal cavity.
  • Renal.
  • Pulmonary hemorrhage

Notes:

  • Pulmonary hemorrhage syndromes:[6]
    • Goodpasture.
    • Idiopathic pulmonary hemosiderosis.
    • Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener's granulomatosis).
    • Systemic lupus erythematosus.

Microscopic

Features:

  • Granulomas typically poorly formed.[7]

References

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