What is the treatment for patients with Takayasu arteritis, Polyarteritis Nodosa, Behcet disease, or Henoch-Schönlein Purpura (HSP)?

Treatment for Takayasu Arteritis, Polyarteritis Nodosa, Behcet Disease, and Henoch-Schönlein Purpura

Takayasu Arteritis Treatment

For patients with active Takayasu arteritis, high-dose glucocorticoids (prednisone 40-60 mg daily) plus a non-glucocorticoid immunosuppressive agent is the recommended initial therapy to induce remission and minimize glucocorticoid toxicity. 1

Initial Treatment Approach:

• High-dose glucocorticoids (prednisone 40-60 mg daily) should be started promptly upon diagnosis to control inflammation 1
• Add a non-glucocorticoid immunosuppressive agent as adjunctive therapy to reduce glucocorticoid exposure 1
• Methotrexate (20-25 mg/week) is often used as the initial non-glucocorticoid agent, especially in children due to better tolerability 1
• Alternative first-line immunosuppressants include azathioprine (2 mg/kg/day) and TNF inhibitors 1
• Tocilizumab is not recommended as initial therapy but may be considered for refractory disease 1

Monitoring and Disease Assessment:

• Regular clinical assessment combined with inflammatory markers (ESR, CRP) to monitor disease activity 1
• Scheduled non-invasive imaging (MRI, CT angiography, or FDG-PET) to detect subclinical disease activity 1
• Imaging interval may be shorter early in disease (every 3-6 months) and longer with established quiescent disease 1
• New vascular lesions on imaging warrant immunosuppressive therapy even if clinically asymptomatic 1

Refractory Disease Management:

• For disease refractory to initial therapy, TNF inhibitors are conditionally recommended over tocilizumab 1
• Tocilizumab may be considered when TNF inhibitors are contraindicated or ineffective 1, 2
• Leflunomide is another option for refractory cases 3
• Abatacept is not recommended as it has been shown to be ineffective in a randomized controlled trial 1

Surgical Considerations:

• Surgical intervention should be delayed until disease is quiescent 1
• Medical management is preferred over surgical intervention for renovascular hypertension and renal artery stenosis 1
• For worsening limb/organ ischemia while on immunosuppressive therapy, escalate immunosuppression before considering surgery 1
• When surgery is necessary during active disease, high-dose glucocorticoids should be used perioperatively 1
• Surgical decisions should be made collaboratively between rheumatologist and vascular surgeon 1

Additional Recommendations:

• Low-dose aspirin or antiplatelet therapy should be added for patients with critical cranial or vertebrobasilar involvement 1
• Long-term clinical monitoring is strongly recommended for all patients, even those in remission 1

Polyarteritis Nodosa Treatment

High-dose glucocorticoids combined with cyclophosphamide is the standard treatment for severe polyarteritis nodosa, with antiviral therapy added for HBV-associated cases. 1

Treatment Approach:

• High-dose glucocorticoids (1 mg/kg/day of prednisone) for initial control of inflammation 1
• Cyclophosphamide for severe disease with major organ involvement 1
• For HBV-associated PAN, antiviral therapy is essential alongside immunosuppression 1
• Less severe disease may be managed with methotrexate or azathioprine plus glucocorticoids 1

Behcet Disease Treatment

Treatment for Behcet disease must be tailored to organ involvement, with colchicine for mucocutaneous manifestations and immunosuppressants for severe organ involvement. 1

Treatment Based on Manifestations:

• Mucocutaneous lesions: Colchicine, topical steroids, and short courses of oral glucocorticoids 1
• Ocular involvement: Azathioprine, cyclosporine, or TNF inhibitors 1
• Vascular involvement: High-dose glucocorticoids plus cyclophosphamide or TNF inhibitors 1
• Neurological involvement: High-dose glucocorticoids followed by maintenance with azathioprine or mycophenolate mofetil 1

Henoch-Schönlein Purpura (HSP) Treatment

For uncomplicated HSP with primarily cutaneous and joint manifestations, supportive care is sufficient, while glucocorticoids are indicated for gastrointestinal or renal involvement. 1

Treatment Approach:

• Mild disease (skin and joint involvement only): Supportive care with adequate hydration and pain management 1
• Gastrointestinal involvement: Short course of glucocorticoids (1-2 mg/kg/day) 1
• Renal involvement: High-dose glucocorticoids; add cyclophosphamide, azathioprine, or mycophenolate mofetil for severe nephritis 1
• Monitor urinalysis regularly for at least 6 months after diagnosis to detect delayed renal involvement 1

Monitoring:

• Regular blood pressure measurements and urinalysis to detect renal involvement 1
• Follow-up with nephrology for patients with renal manifestations 1

Treatment Pitfalls and Caveats

• Disease activity assessment in Takayasu arteritis is challenging as clinical symptoms may not correlate with vascular inflammation 3, 4
• Reliance solely on inflammatory markers is insufficient; imaging is essential for monitoring disease activity 1, 3
• Surgical interventions performed during active disease have higher failure rates 1
• Tocilizumab affects acute phase reactants, which may mask ongoing inflammation while not controlling disease 1, 2
• Long-term immunosuppression increases infection risk; appropriate prophylaxis should be considered 2
• Pregnancy planning is important as many immunosuppressants are contraindicated during pregnancy 1