What are the symptoms and treatment options for vasculitis?

Vasculitis Symptoms and Treatment Options

Vasculitis presents with diverse symptoms depending on the type and affected vessels, typically requiring high-dose glucocorticoid therapy (40-60 mg/day prednisone) for induction of remission, followed by appropriate immunosuppressive agents based on disease severity and organ involvement. 1

Types and Symptoms of Vasculitis

Large Vessel Vasculitis (Giant Cell Arteritis and Takayasu Arteritis)

Giant Cell Arteritis (GCA) Symptoms:

• New-onset persistent localized headache (often temporal area)
• Constitutional symptoms (weight loss >2 kg, low-grade fever, fatigue, night sweats)
• Jaw and/or tongue claudication
• Acute visual symptoms (amaurosis fugax, acute visual loss, diplopia)
• Polymyalgia rheumatica symptoms
• Limb claudication 1

Clinical Examination Findings in GCA:

• Tenderness/thickening of temporal arteries with reduced pulsation
• Scalp tenderness
• Bruits (particularly in axilla)
• Reduced pulses/blood pressure in upper limbs
• Pathological ophthalmologic findings 1

Takayasu Arteritis Symptoms:

• New onset or worsening limb claudication
• Constitutional symptoms (weight loss, fever, fatigue, night sweats)
• Myalgia, arthralgia, arthritis
• Severe abdominal pain
• Neurological symptoms (stroke, seizures, syncope, dizziness)
• Extremity paresis
• Cardiovascular symptoms (myocardial infarct, angina)
• Visual symptoms 1

ANCA-Associated Vasculitis (AAV)

AAV can affect almost any organ system with manifestations including:

• Kidney involvement: hematuria, proteinuria, rapidly declining renal function
• Pulmonary involvement: hemorrhage (10% of patients), respiratory symptoms
• Skin manifestations: palpable purpura, nodules, livedo reticularis
• Neurological involvement: peripheral neuropathy, mononeuritis multiplex
• Constitutional symptoms: fever, weight loss, fatigue
• Eye and ENT involvement 1

Medium Vessel Vasculitis (Polyarteritis Nodosa)

Polyarteritis nodosa (PAN) commonly presents with:

• Systemic symptoms: fever, weight loss
• Neurological manifestations: mononeuritis multiplex, peripheral neuropathy
• Cutaneous manifestations: nodules, livedo reticularis
• Renal manifestations: hypertension
• Gastrointestinal manifestations: abdominal pain 1

Treatment Approach

Large Vessel Vasculitis Treatment

1. Induction therapy:

   • High-dose glucocorticoid therapy (40-60 mg/day prednisone-equivalent)
   • Rapid referral to specialized center for diagnostic work-up within 24 hours for suspected GCA
   • Pre-emptive treatment with glucocorticoids before appointment for patients with visual symptoms 1

2. Maintenance therapy:

   • Taper glucocorticoid dose to 15-20 mg/day within 2-3 months
   • After 1 year, taper to ≤5 mg/day for GCA and ≤10 mg/day for Takayasu arteritis 1

ANCA-Associated Vasculitis Treatment

1. For severe/organ-threatening disease:

   • Combination of cyclophosphamide and glucocorticoids for remission induction
   • Rituximab is an effective alternative to cyclophosphamide with potentially fewer side effects 1, 2

2. For non-organ threatening disease:

   • Methotrexate (oral or parenteral) with glucocorticoids 1

3. Maintenance therapy:

   • Options include azathioprine, methotrexate, or rituximab 1, 2

Polyarteritis Nodosa Treatment

1. For severe PAN:

   • Early treatment with cyclophosphamide and glucocorticoids
   • Limit long-term exposure to both treatments to minimize toxicity 1

2. For mild disease:

   • NSAIDs for self-limited conditions
   • Colchicine and dapsone for mild recurrent or persistent disease 3

Important Considerations

• Rapid diagnosis is critical: Delay in treatment when visual loss is present is the strongest risk factor for permanent blindness in GCA 1

• Monitoring: Regular assessment for drug toxicity, including blood counts, renal function, and blood sugar levels 1

• Long-term complications: Patients should be assessed for comorbidities after remission induction, as vasculitis can cause permanent damage to kidneys, lungs, heart, nervous system, and other organs 1

• Patient education: Patients should receive clear explanations about their disease, treatment options, side effects, and prognosis to improve outcomes and help recognize early signs of relapse 1

• Referral to specialized centers: Patients with AAV should be treated at centers with experience in vasculitis management 1

• Holistic approach: Consider the impact of vasculitis on quality of life, including fatigue, pain, and psychological effects 1

Treatment Algorithm

1. Assess disease severity and organ involvement
2. For life or organ-threatening disease:
   • Immediate high-dose glucocorticoids (40-60 mg/day prednisone)
   • Add cyclophosphamide or rituximab for remission induction
3. For non-organ threatening disease:
   • Glucocorticoids plus methotrexate
4. Once remission achieved:
   • Taper glucocorticoids according to disease type
   • Implement appropriate maintenance therapy
5. Monitor regularly for:
   • Disease activity
   • Treatment toxicity
   • Long-term complications

Remember that early diagnosis and prompt treatment are essential to prevent permanent organ damage and improve survival in patients with vasculitis.