Vasculitides

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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.

Image(s):

Takayasu's arteritis

General

Features:[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:[3]

  • 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.[4]
  • 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.
  • Fibroid necrosis: dead vessel wall - pink anucleate stuff, nuclear debris (black specks of nuclear material).

Image: PAN (immunologyclinic.com).

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

Microscopic

Features:

  • Granulomas typically poorly formed.[7]

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

See also

References

  1. TN05 RH3.
  2. TN05 RH20.
  3. 3.0 3.1 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. 538. ISBN 0-7216-0187-1.
  4. Klatt. AOP P.14.
  5. TN05 RH6.
  6. 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.
  7. 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.