What is the treatment for Castleman’s ileitis?

Treatment of Castleman's Disease Involving the Ileum

Surgical resection is the definitive treatment for unicentric Castleman's disease affecting the ileum, while multicentric disease requires systemic therapy with siltuximab (or tocilizumab if unavailable) as first-line treatment. 1, 2

Initial Assessment and Disease Classification

The critical first step is determining whether the disease is unicentric (UCD) or multicentric (MCD), as this fundamentally changes management:

• Unicentric disease is localized and carries an excellent prognosis, while multicentric disease is systemic with considerable morbidity and mortality 1
• Imaging should identify whether lymphadenopathy is confined to a single anatomic region (unicentric) or involves multiple nodal stations (multicentric) 3
• The ileum may be secondarily involved in Castleman's disease and can represent the first manifestation 4

Treatment Algorithm for Unicentric Castleman's Disease

Surgical resection remains the standard and curative therapy for UCD 1, 2:

• Complete surgical excision of the affected lymphoid tissue is often curative for unicentric hyaline vascular Castleman disease 3
• Local approaches are appropriate given the indolent nature of UCD 5
• Prognosis after complete resection is excellent 1

Treatment Algorithm for Multicentric Castleman's Disease

Systemic therapies are required for MCD, with treatment intensity based on disease severity 1, 2:

First-Line Therapy

Siltuximab (anti-IL-6 monoclonal antibody) with or without corticosteroids is the preferred first-line therapy 2:

• Tocilizumab should be used if siltuximab is unavailable 2
• IL-6 is a key therapeutic target given its central role in disease pathogenesis 5
• Rituximab monotherapy is also considered a mainstay of therapy, particularly in HHV-8 associated cases 1

Severe Disease Requiring Intensive Therapy

For the most severe cases, adjuvant combination chemotherapy is recommended in addition to targeted therapy 2:

• Single-agent and combination chemotherapy with glucocorticoids are established systemic approaches 1
• Antiviral strategies should be considered, particularly in HIV-positive patients or those with HHV-8 association 1

Second and Third-Line Options

Additional agents tailored by disease severity should be used for treatment failures 2:

• Rituximab can be used as an alternative if not employed first-line 1
• Cytotoxic chemotherapy carries attendant toxicity risks but has variable efficacy 5

Critical Monitoring Considerations

Careful attention must be paid to potential concomitant conditions 1:

• Screen for associated infections, particularly HHV-8 and HIV 1, 3
• Monitor for development of lymphoma, which is associated with Castleman's disease 3
• Evaluate for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) 3
• Assess for plasma cell dyscrasias and paraneoplastic pemphigus 3

Common Pitfalls to Avoid

• Do not treat unicentric disease with systemic chemotherapy when surgical resection is feasible - surgery is curative and avoids unnecessary toxicity 1, 3
• Do not assume all cases are benign - multicentric disease has considerable morbidity and mortality requiring aggressive systemic therapy 1
• Do not overlook gastrointestinal involvement as a presenting manifestation - the digestive tract may give the first clinical signs of disease 4
• Do not use outdated chemotherapy-first approaches - targeted therapies like siltuximab have improved outcomes for newly diagnosed MCD patients 1