What are the signs and symptoms of vasculitis?

Signs and Symptoms of Vasculitis

Vasculitis presents with diverse clinical manifestations depending on the vessel size affected and organ involvement, with common features including fever, fatigue, weight loss, and specific organ-related symptoms such as headache, skin lesions, and respiratory or renal abnormalities.

General Signs and Symptoms

• Constitutional symptoms are common in vasculitis and include:

  • Weight loss (>2 kg)
  • Low-grade fever
  • Fatigue
  • Night sweats 1

• Laboratory abnormalities frequently observed:

  • Elevated erythrocyte sedimentation rate (ESR)
  • Elevated C-reactive protein (CRP)
  • Presence of specific autoantibodies (e.g., ANCA) 1

Manifestations by Vessel Size

Large Vessel Vasculitis (Giant Cell Arteritis, Takayasu Arteritis)

• Giant Cell Arteritis:

  • New-onset persistent localized headache, often in temporal area
  • Jaw and/or tongue claudication
  • Acute visual symptoms (amaurosis fugax, visual loss, diplopia)
  • Tenderness/thickening of temporal arteries
  • Scalp tenderness 1

• Takayasu Arteritis:

  • New onset or worsening limb claudication
  • Bruits over major vessels
  • Reduced or absent pulses
  • Blood pressure discrepancies between limbs
  • Carotidynia (neck pain) 1

Medium and Small Vessel Vasculitis

• ANCA-Associated Vasculitis (Granulomatosis with Polyangiitis, Microscopic Polyangiitis, Eosinophilic Granulomatosis with Polyangiitis):

  • Pulmonary manifestations:

    • Hemoptysis
    • Pulmonary nodules or infiltrates
    • Diffuse alveolar hemorrhage 1, 2

  • Renal manifestations:

    • Microscopic hematuria with dysmorphic red blood cells
    • Red cell casts
    • Moderate proteinuria (1-3 g/day)
    • Rapidly declining kidney function 3

  • Ear, nose, and throat involvement:

    • Sinusitis
    • Nasal crusting
    • Epistaxis
    • Hearing loss 1

Cutaneous Manifestations

• Palpable purpura (most common skin manifestation, especially in small vessel vasculitis) 4

• Infiltrated erythema

• Nodular erythema

• Livedo racemosa

• Deep ulcers

• Digital gangrene 4, 5

• Specific patterns:

  • Subcutaneous nodules that ulcerate with surrounding livedo racemosa suggest polyarteritis nodosa
  • Palpable purpura on lower legs is characteristic of immune complex vasculitis
  • Hemorrhagic papules and necrotic plaques in acral areas after cooling indicate cryoglobulinemic vasculitis 5

Neurological Manifestations

• Central nervous system:

  • Headaches
  • Altered consciousness
  • Stroke
  • Seizures
  • Encephalopathy 1

• Peripheral nervous system:

  • Mononeuritis multiplex
  • Paresis of extremities 1

Diagnostic Considerations

• Cerebral vasculitis should be considered in patients with:

  • Ischemic or hemorrhagic stroke
  • Recurrent stroke
  • Stroke with encephalopathic changes
  • Stroke accompanied by fever
  • Multifocal neurological events
  • Unexplained skin lesions
  • Glomerulopathy 1

• Primary CNS vasculitis typically presents with:

  • Headaches
  • Altered consciousness
  • Small-vessel strokes occurring over weeks to months 1

Important Clinical Pitfalls

• Not all patients with vasculitis show laboratory signs of inflammation 1
• Classic angiographic findings (arterial beading, alternating areas of constriction and dilatation) are nonspecific 1
• Vasculitis can mimic other conditions like infections, malignancies, and thrombotic disorders 4
• Symptoms may overlap with connective tissue diseases, requiring careful differentiation 2
• Biopsy is often necessary for definitive diagnosis, but a negative biopsy does not exclude vasculitis 1

Urgent Evaluation

• Patients with suspected giant cell arteritis and visual symptoms require immediate evaluation and treatment to prevent permanent visual loss 1
• Diffuse alveolar hemorrhage with hypoxemia is a medical emergency requiring prompt intervention 1
• Rapidly declining kidney function suggests severe renal vasculitis requiring urgent assessment 3