Idiopathic Multicentric Castleman Disease (iMCD) Diagnostic Criteria
Diagnoses idiopathic multicentric Castleman disease (iMCD) and iMCD subtypes.
Idiopathic Multicentric Castleman Disease is a rare disease process, it is imperative to make the diagnosis with a combination of clinical and pathologic findings.
Be sure to review the exclusion criteria outlined in the Evidence section.
Advice
Castleman disease is a rare and complex condition. Clinicians should be aware of changing evidence and available clinical trials which may impact treatment decisions.
Management
- Both nonsevere and severe iMCD are generally treated with immunotherapy and steroids.
- First line immunotherapy is with siltuximab, although tocilizumab may be used if first-line therapy is not approved or unavailable.
- Those with severe iMCD should also be referred to a Center of Excellence and/or a CD expert.
Critical Actions
- 10%-20% of patients with severe iMCD may require critical care. These patients should be considered for high dose steroid therapy, along with weekly dosing of siltuximab for up to four weeks.
- A CD expert is highly recommended in the care of these patients.