Which type of physicians manage leukocytoclastic vasculitis?

Management of Leukocytoclastic Vasculitis: Specialist Care Requirements

Leukocytoclastic vasculitis (LCV) should be managed by a multidisciplinary team led by rheumatologists and dermatologists, with additional specialists involved based on organ involvement. This approach is essential for optimal patient outcomes as LCV can range from skin-limited disease to life-threatening systemic vasculitis.

Primary Specialists for LCV Management

Rheumatologists

• Serve as the primary specialists for systemic LCV cases
• Lead multidisciplinary care coordination
• Manage immunosuppressive therapy
• Monitor disease activity and treatment response
• Particularly important for ANCA-associated vasculitis forms of LCV 1

Dermatologists

• Often the first to diagnose LCV through skin biopsy
• Manage skin-limited LCV cases
• Provide ongoing skin care for all LCV patients
• Administer topical treatments when appropriate 2, 3

Additional Specialists Based on Organ Involvement

The European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) guidelines emphasize that patients with LCV should be managed in close collaboration with, or at, centers of expertise 1, 4. This typically involves:

1. Nephrologists: For patients with renal involvement (glomerulonephritis)
2. Pulmonologists: For respiratory manifestations (pulmonary hemorrhage, nodules)
3. Otorhinolaryngologists: For sinonasal, subglottic, and airway involvement
4. Ophthalmologists: For ocular manifestations (scleritis, episcleritis, retro-orbital disease)
5. Neurologists: For central or peripheral nervous system involvement
6. Gastroenterologists: For gastrointestinal manifestations

Specialist Involvement Based on Disease Severity

Skin-Limited LCV

• Primary management: Dermatologists
• Treatment approach: Often conservative with colchicine, dapsone, or low-dose corticosteroids 2, 5

Systemic LCV

• Primary management: Rheumatologists coordinating with other specialists
• Treatment approach: More aggressive immunosuppression with glucocorticoids plus cyclophosphamide or rituximab 1, 4

Multidisciplinary Team Composition

The EULAR task force for ANCA-associated vasculitis management included a diverse group of specialists, highlighting the multidisciplinary nature of care required 1:

• Rheumatologists
• Renal physicians (nephrologists)
• General internists
• Pulmonologists
• Pathologists
• Otorhinolaryngologists
• Immunologists
• Ophthalmologists

Referral Considerations

The ACR/Vasculitis Foundation guideline specifically recommends:

1. Referral to specialized centers for refractory disease
2. Collaboration with specialists for specific organ manifestations
3. Consideration of clinical trial enrollment for complex cases 1, 4

Practical Management Algorithm

1. Initial diagnosis: Dermatologist (skin biopsy) or Rheumatologist
2. Disease assessment: Determine if skin-limited or systemic
   • Skin-limited: Primary dermatology management
   • Systemic: Primary rheumatology management with multidisciplinary approach
3. Organ-specific involvement: Add relevant specialists to the care team
4. Treatment initiation: Based on disease severity
   • Non-organ threatening: Dermatologist or Rheumatologist (colchicine, dapsone, low-dose steroids)
   • Organ/life-threatening: Rheumatologist-led team (high-dose steroids plus cyclophosphamide or rituximab)
5. Maintenance therapy: Typically managed by Rheumatologist for at least 24 months 1
6. Long-term monitoring: Shared between specialists based on organ involvement

The EULAR recommendations specifically state that "AAV are rare, heterogeneous, and potentially life-threatening and organ-threatening diseases and thus require multidisciplinary management by centres with, or with ready access to, expertise in vasculitis" 1, underscoring the importance of specialist care for these complex conditions.