What are the symptoms of systemic vasculitis?

From the Guidelines

Systemic vasculitis symptoms can be diverse and affect multiple organ systems, but common manifestations include fever, weight loss, fatigue, and organ-specific symptoms such as headaches, scalp tenderness, vision loss, jaw claudication, limb claudication, and abdominal pain.

Common Symptoms

• Fever and weight loss are common systemic symptoms of vasculitis, as seen in polyarteritis nodosa (PAN) and giant cell arteritis (GCA) 1
• Organ-specific symptoms vary depending on the type of vasculitis and the organs involved, but can include:
  • Respiratory symptoms such as cough, dyspnea, and pulmonary hemorrhage in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis 1
  • Neurologic symptoms such as mononeuritis multiplex, peripheral neuropathy, and stroke in PAN and GCA 1
  • Cutaneous symptoms such as nodules, livedo reticularis, and purpura in PAN and GCA 1
  • Renal symptoms such as hypertension, hematuria, and proteinuria in ANCA-associated vasculitis and PAN 1
  • Gastrointestinal symptoms such as abdominal pain, diarrhea, and bleeding in PAN and GCA 1

Specific Vasculitis Types

• Giant cell arteritis (GCA): characterized by headaches, scalp tenderness, vision loss, jaw claudication, and limb claudication 1
• Takayasu arteritis (TAK): characterized by limb claudication, constitutional symptoms, and arterial stenosis or aneurysms 1
• Polyarteritis nodosa (PAN): characterized by neurologic, cutaneous, renal, and gastrointestinal symptoms 1
• ANCA-associated vasculitis: characterized by respiratory, renal, and systemic symptoms, including pulmonary hemorrhage and glomerulonephritis 1 These symptoms can vary in severity and combination, and a thorough diagnostic evaluation is necessary to determine the specific type of vasculitis and guide treatment. The use of disease activity scores, such as the Birmingham Vasculitis Activity Score, and biomarkers, such as ANCA and erythrocyte sedimentation rate (ESR), can aid in diagnosis and monitoring of disease activity 1.

From the Research

Symptoms of Systemic Vasculitis

The symptoms of systemic vasculitis can vary depending on the size of the affected blood vessels and the organs involved. Some common symptoms include:

• Musculoskeletal affections, such as joint pain and muscle weakness, which were present in 65% of patients in one study 2
• Cutaneous symptoms, such as:
  • Palpable purpura, which is a nearly pathognomonic feature of immune complex vasculitis 3
  • Livedo racemosa and subcutaneous nodules, which are common in medium-vessel vasculitis 3
  • Erythema elevatum diutinum, which begins with brightly red to violaceous plaques at extensor sites, followed by fibrotic nodules 3
• Gastrointestinal symptoms, which were recorded in 56.3% of patients in one study 2
• Cardiovascular symptoms, such as coronary arteritis and myocarditis, which can be life-threatening 4, 5
• Neurological symptoms, such as nervous system affections, which were present in 10% of patients in one study 2
• Systemic inflammation, which can manifest as fever, fatigue, and weight loss 4, 5

Organ Involvement

Systemic vasculitis can affect various organ systems, including:

• Skin, which is one of the most frequently involved organs 3
• Joints and muscles, which can cause musculoskeletal symptoms 2
• Gastrointestinal tract, which can cause abdominal pain, diarrhea, and bleeding 2
• Cardiovascular system, which can cause coronary arteritis, myocarditis, and heart failure 4, 5
• Nervous system, which can cause neurological symptoms such as numbness, tingling, and weakness 2

Laboratory and Imaging Tests

Laboratory and imaging tests can help diagnose systemic vasculitis, including:

• Antineutrophilic cytoplasmic antibody (ANCA) testing, which can help define a subgroup of small-vessel vasculitides 5
• Imaging tests, such as computed tomographic angiography, magnetic resonance angiography, and positron emission tomography/computed tomography, which can help visualize the affected blood vessels and organs 4