• Clinical science

Vasculitides

Abstract


Vasculitides are a heterogeneous group of autoimmune diseases, all characterized by inflammation of blood vessels (vasculitis) and subsequent ischemia and damage to the organs supplied by these vessels. Vasculitis may occur as a primary disease (idiopathic) or as a secondary response to an underlying disease (e.g., hepatitis B infection). While the inflammatory process may be confined to one organ, it may also involve several organ systems. Vasculitis should be considered in patients presenting with palpable purpura, pulmonary infiltrates, unexplained ischemic events, and multisystem disease. The detection of antineutrophil cytoplasmic antibodies (ANCA) in the blood is an important diagnostic marker; however, there are also ANCA-negative vasculitis syndromes. Immunosuppressive treatment is implemented to stop vascular inflammation. Specific (e.g., antiviral drugs) or symptomatic (e.g., NSAID) management may be necessary. If the vasculitis is secondary to an underlying disease, appropriate treatment of the underlying disease should be initiated.

Overview

Large vessel vasculitis

Giant cell arteritis (cranial arteritis)

See giant cell arteritis for more information.

Takayasu arteritis (aortic arch syndrome)

References:[1][2][3][4]

Medium-sized vessel vasculitis

Kawasaki disease

See Kawasaki disease for more information.

Polyarteritis nodosa (PAN)

PAN should be considered in young adults presenting with stroke or myocardial infarction! The diagnosis may be confirmed with a biopsy of involved tissue!

References:[2][5]

Thromboangiitis obliterans

See thromboangiitis obliterans for more information.

ANCA-associated small vessel vasculitis

Granulomatosis with polyangiitis

See granulomatosis with polyangiitis for more information.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Microscopic polyangiitis

References:[1][6][7][8]

Leukocytoclastic vasculitis of small vessels (non-ANCA-associated)

The following leukocytoclastic vasculitis syndromes exhibit a characteristic histolopathologic feature of nuclear debris from neutrophil leukocytes that have infiltrated blood vessel walls.

Henoch-Schönlein purpura (Immunoglobulin A (IgA) vasculitis)

See Henoch-Schönlein purpura for more information.

Cryoglobulinemic vasculitis

Cutaneous leukocytoclastic vasculitis

  • Definition: necrotizing vasculitis of cutaneous small vessels
  • Etiology: drug-induced; infections
  • Clinical features:
  • Diagnostics:
    • Skin biopsy
    • Investigation of underlying cause
    • Systemic vasculitis should be ruled out.
  • Treatment: symptomatic; discontinue drug intake; immunosuppression may be required

References:[1][9][10]

Behçet disease

  • Definition: a systemic vasculitis that can affect arteries and veins of all sizes
  • Epidemiology [10]
    • Most common from the Mediterranean region to eastern Asia, with the highest prevalence observed in Turkey and Japan
    • Strong HLA-B51 association
    • Peak incidence: 20–40 years
  • Pathophysiology: not completely understood
  • Clinical features [11][12][13][10]
    • Recurrent painful oral ulceration (95–100%): multiple minor aphthous ulcers, major aphthous ulcers, and/or herpetiform ulcers
      • Typically the initial presenting symptom
    • Recurrent genital ulceration
      • Single or multiple ulcers that resemble oral aphthous ulcers and heal with scarring
      • Location
        • : vulva (most common), vagina
        • : scrotum (most common), penis
    • Ocular disease (50–80%): uveitis (iridocyclitis, chorioretinitis), conjunctivitis, keratitis, papillitis, and/or retinal vasculitis
      • Typically bilateral
      • More common and more severe among men
      • Usually occurs 2–3 years after the onset of oral and/or genital ulceration
      • Initial presenting feature of Behçet disease in 10–20% of cases
    • Skin lesions (35–85%): erythema nodosum, pyoderma gangrenosum, and/or papulopustular lesions (pseudofolliculitis or acneiform eruptions)
    • Arthritis (40–50%): non-erosive, non-deforming, asymmetric mono-/oligoarthritis of knees, ankles, hands, and/or wrists
    • Gastrointestinal disease (< 25%): ileocecal ulceration → abdominal pain, anorexia, diarrhea, lower GI bleeding, nausea, vomiting
    • Vasculopathy
    • Neuro-Behçet syndrome (5–10%)

Behçet disease triad: oral ulcers, genital ulcers, and uveitis!

Differential diagnosis of vasculitis

Clinical presentation Diagnostic clues

Giant cell arteritis

  • Elderly women, typically > 50 years
  • Visual impairment → may result in blindness
  • Temporal headaches
  • ↑↑ ESR
  • Autoantibodies absent
  • Halo sign around the vessel on duplex sonography
  • Temporal artery biopsy (gold standard) shows granulomatous inflammation with giant cells, and intima proliferation that results in stenosis.
Takayasu arteritis
  • Angiography shows stenosis (gold standard)
  • Biopsy shows granulomatous inflammation of aorta and its major branches.
Kawasaki syndrome
Polyarteritis nodosa
  • >
  • Very heterogeneous: renal, skin, gastrointestinal, and neuromuscular involvement
  • Angina pectoris
Thromboangiitis obliterans
  • Men
  • Strong association with tobacco use!
  • Severe limb ischemia → acute necrosis of toes and fingers often requires amputation
  • Angiography (confirmatory test): non-atherosclerotic segmental occlusion of arteries
Granulomatosis with polyangiitis
Churg-Strauss syndrome
  • Severe allergic asthma
  • Skin manifestations (e.g., tender nodules)
  • Gastrointestinal, cardiac, renal involvement possible
Microscopic polyangiitis
Henoch-Schönlein purpura
  • Children; 90% < 10 years
  • Palpable purpura on lower limbs
  • Intestinal colic
  • Renal involvement
  • Often secondary to upper respiratory tract infections
  • IgA in serum
Cryoglobulinemic vasculitis
  • 40–50 years; > 3:1
  • Skin manifestations (100% of cases)
  • Arthralgia
  • Vasomotor symptoms (Raynaud phenomenon)
Cutaneous leukocytoclastic vasculitis
  • Drug-induced
Behçet disease
  • Most common in Turkey, the Middle East, and Japan
  • Oral and genital ulcers
  • Uveitis

References:[15][16][17][8]