Clinical Presentation of Vasculitis
Vasculitis presents with highly variable clinical manifestations determined primarily by the size of affected blood vessels and the specific organs involved, ranging from constitutional symptoms (fever, weight loss, fatigue) to life-threatening organ damage including glomerulonephritis, pulmonary hemorrhage, and neurologic deficits.
Presentation by Vessel Size
Large Vessel Vasculitis (Giant Cell Arteritis & Takayasu Arteritis)
Giant Cell Arteritis 1:
- Cranial manifestations: New-onset persistent headache (often temporal), scalp tenderness, jaw/tongue claudication
- Visual symptoms: Amaurosis fugax, acute visual loss, diplopia (20% of patients) - can progress to permanent blindness 2
- Vascular findings: Tender/thickened temporal arteries with reduced pulsation, bruits (particularly axillary), reduced pulses or blood pressure asymmetry in upper limbs 3
- Systemic symptoms: Weight loss >2 kg, low-grade fever, fatigue, night sweats 3
- Associated: Polymyalgia rheumatica symptoms in two-thirds of patients 2
Takayasu Arteritis 1:
- Vascular manifestations: Limb claudication, pulse loss or inequality, bruits, carotidynia 3
- Hypertension: >140/90 mm Hg is a key finding 3
- Neurologic: Stroke, seizures (non-hypertensive), syncope, dizziness 3
- Cardiac: Myocardial infarction, angina 3
- Constitutional: Weight loss >2 kg, fever, fatigue, night sweats, myalgia, arthralgia 3
Medium Vessel Vasculitis (Polyarteritis Nodosa)
Polyarteritis Nodosa 4:
- Neurologic: Mononeuritis multiplex and peripheral neuropathy (most common) 4
- Cutaneous: Nodules, livedo reticularis 4
- Renal: Hypertension (not glomerulonephritis) 4
- Gastrointestinal: Abdominal pain 4
- Systemic: Fever, weight loss 4
Small Vessel Vasculitis (ANCA-Associated Vasculitis)
Common Organ Involvement 5:
- Renal: Rapidly progressive glomerulonephritis with microscopic hematuria, dysmorphic red blood cells, red cell casts, moderate proteinuria (1-3 g/day), rapidly declining GFR over days to weeks 5
- Pulmonary: Upper and lower respiratory tract involvement; pulmonary hemorrhage in 10% (associated with increased mortality) 5
- Skin: Palpable purpura (pathognomonic for small vessel vasculitis) 6, 7
- Eyes: Ocular involvement 5
- Nervous system: Peripheral and central nervous system manifestations 5
Pulmonary-Renal Syndrome: Simultaneous lung and kidney injury with alveolar hemorrhage and acute kidney injury - should immediately raise concern for vasculitis 5
Granulomatosis with Polyangiitis (GPA) 8:
- Upper respiratory tract disease (chronic rhinosinusitis)
- Lower respiratory tract involvement
- Glomerulonephritis
- 90% ANCA-positive (primarily PR3-ANCA) 5
Eosinophilic Granulomatosis with Polyangiitis (EGPA) 8:
- Respiratory: Asthma (required for diagnosis), chronic rhinosinusitis with nasal polyps 8
- Cardiac: Eosinophilic cardiomyopathy (critical complication) 8
- Neurologic: Mononeuritis multiplex, motor neuropathy 8
- Gastrointestinal: Eosinophilic gastroenteritis 8
- Laboratory: Eosinophilia ≥1 × 10⁹/L 8
- Skin: Palpable purpura, nodules 8
Cutaneous Manifestations Across Vasculitis Types
Key skin findings by vessel size 7:
- Small vessel: Palpable purpura (lower legs predominantly) 6
- Medium vessel: Livedo racemosa, subcutaneous nodules 6, 7
- All sizes: Skin ulcers, necrosis, blisters 9, 7
Critical Pitfalls
ANCA-negativity does not exclude vasculitis: 10% of small-vessel vasculitis patients are ANCA-negative 5
Rapidly progressive presentations require immediate action: In pulmonary-renal syndrome with compatible clinical presentation and positive ANCA serology, do not delay immunosuppressive therapy waiting for biopsy confirmation, especially in rapidly deteriorating patients 5
Visual symptoms in GCA are emergencies: Acute visual disturbance with elevated inflammatory markers requires immediate glucocorticoid therapy before diagnostic confirmation to prevent permanent blindness 3
Constitutional symptoms are common but nonspecific: Fever, weight loss, fatigue, and night sweats occur across all vasculitis types and should prompt thorough vascular examination 4, 3, 1
Multisystem involvement is the rule: Clinically recognized disease in multiple organ systems may not always be present initially, but systemic evaluation is mandatory 10, 11