Table 1.

Management of CD: an overview.

	Treatment	Prognosis
Unicentric (orbital) CD	Surgery (cornerstone)±neoadjuvant chemotherapy alternative: radiotherapy (i) If associated ocular involvement—trial of steroids (unless contraindicated) followed by radiotherapy	Surgery: excellent prognosis with 10-year survival rate > 95% Radiotherapy alone: 2- year survival rate—approximately 80% [7]
Multicentric CD	(i) Cytotoxic chemotherapy (CHOP/etoposide/CVAD/COP) (ii) Monoclonal antibody—rituximab (+CHOP) (iii) Corticosteroids (limited role) (iv) Emergent therapies: IL-6 receptor antagonist (tocilizumab, siltuximab), anakinra (IL-1 receptor antagonist), and autologous stem cell transplantation (v) Antiviral agents (in combination/maintenance therapy)	Multicentric CD has a long-term poor prognosis (worst in idiopathic MCD) Emergent therapies including rituximab show a better survival rate, but larger multicenter studies are required

CHOP: cyclophosphamide, doxorubicin, vincristine, and prednisolone; CVAD: cyclophosphamide, vincristine, doxorubicin, dexamethasone; COP: cyclophosphamide, vincristine, prednisolone.