What are the symptoms of autoimmune vasculitis?

Symptoms of Autoimmune Vasculitis

Autoimmune vasculitis presents with a wide spectrum of symptoms depending on the size of blood vessels affected, with common manifestations including constitutional symptoms, organ-specific damage, and laboratory abnormalities reflecting systemic inflammation. 1

General Constitutional Symptoms

• Fever (low-grade or high)
• Fatigue
• Weight loss (>2 kg)
• Night sweats
• Myalgia and arthralgia

Symptoms by Vessel Size

Large Vessel Vasculitis

Giant Cell Arteritis (GCA)

• Cranial symptoms:
  • New-onset persistent localized headache (often temporal)
  • Scalp tenderness
  • Jaw and/or tongue claudication
  • Acute visual symptoms (amaurosis fugax, visual loss, diplopia)
  • Tenderness/thickening of temporal arteries
  • Reduced pulsation of temporal arteries
• Extracranial manifestations:
  • Symptoms of polymyalgia rheumatica
  • Limb claudication
  • Bruits (particularly in axilla)
  • Reduced pulses/blood pressure in upper limbs 1

Takayasu Arteritis (TAK)

• New onset or worsening limb claudication
• Severe abdominal pain
• Stroke, seizures (non-hypertensive)
• Syncope and dizziness
• Paresis of extremities
• Myocardial infarction and angina
• Acute visual symptoms
• Hypertension (>140/90 mmHg)
• New loss of pulses, pulse inequality
• Bruits
• Carotidynia (neck pain) 1

Medium and Small Vessel Vasculitis

ANCA-Associated Vasculitis (AAV)

• Respiratory symptoms:

  • Pulmonary nodules
  • Infiltrates and cavities
  • Diffuse alveolar hemorrhage
  • Cough and dyspnea 2, 3

• Renal manifestations:

  • Hematuria
  • Proteinuria
  • Rapidly progressive glomerulonephritis
  • Hypertension 2

• ENT involvement:

  • Sinusitis
  • Nasal crusting
  • Epistaxis
  • Hearing loss
  • Subglottic stenosis 2

• Skin manifestations:

  • Purpura
  • Nodules
  • Digital necrosis
  • Ulcers 1

• Neurological manifestations:

  • Mononeuritis multiplex
  • Peripheral neuropathy
  • CNS involvement 1

Laboratory Findings

• Elevated inflammatory markers (ESR and CRP) in >95% of cases
• Positive ANCA in AAV
• Anemia
• Thrombocytosis
• Elevated liver enzymes
• Hematuria and proteinuria 1, 2

Diagnostic Considerations

• Symptoms may vary widely depending on the specific type of vasculitis and organs involved
• Diagnosis often requires a combination of clinical features, laboratory findings, imaging, and sometimes biopsy
• Temporal artery biopsy for GCA showing histopathological features of arteritis
• Vascular imaging (ultrasound, MRI, PET-CT) showing arterial wall thickening or "halo" sign 1

Warning Signs Requiring Urgent Attention

• Acute visual disturbance (risk of permanent blindness)
• Stroke symptoms
• Severe abdominal pain
• Respiratory distress/hemoptysis
• Acute limb ischemia
• Rapidly declining kidney function 1, 2

Early recognition of these symptoms is crucial as prompt treatment with high-dose glucocorticoids (40-60 mg/day prednisone-equivalent) can prevent irreversible organ damage, particularly vision loss in GCA 1. Patients with suspected vasculitis should be referred urgently to specialized centers for comprehensive evaluation and management.