International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease

Abstract

Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms (multicentric CD [MCD]). MCD includes human herpesvirus-8 (HHV-8)–associated MCD, POEMS-associated MCD, and HHV-8−/idiopathic MCD (iMCD). The first-ever diagnostic and treatment guidelines were recently developed for iMCD by an international expert consortium convened by the Castleman Disease Collaborative Network (CDCN). The focus of this report is to establish similar guidelines for the management of UCD. To this purpose, an international working group of 42 experts from 10 countries was convened to establish consensus recommendations based on review of treatment in published cases of UCD, the CDCN ACCELERATE registry, and expert opinion. Complete surgical resection is often curative and is therefore the preferred first-line therapy, if possible. The management of unresectable UCD is more challenging. Existing evidence supports that asymptomatic unresectable UCD may be observed. The anti–interleukin-6 monoclonal antibody siltuximab should be considered for unresectable UCD patients with an inflammatory syndrome. Unresectable UCD that is symptomatic as a result of compression of vital neighboring structures may be rendered amenable to resection by medical therapy (eg, rituximab, steroids), radiotherapy, or embolization. Further research is needed in UCD patients with persisting constitutional symptoms despite complete excision and normal laboratory markers. We hope that these guidelines will improve outcomes in UCD and help treating physicians decide the best therapeutic approach for their patients.

The role of IL-6 and IL-6 blockade in COVID-19

“…Both siltuximab and tocilizumab are approved for the treatment of multicentric Castleman disease [71,72] and are indicated for unresectable unicentric Castleman disease with inflammatory symptoms [73], although limited data exist for the unicentric form [74].…”

Timing and Outcomes of Intracranial Stenting in the Post-SAMMPRIS Era: A Systematic Review

“…Reversal of the stenosis with intracranial stenting had shown the potential to reduce the risk of recurrent stroke significantly, while its safety and efficacy has remained controversial since the publication of the SAMMPRIS trial in 2011, in which the 30-day stroke or death rate in the stenting arm were higher compared with aggressive medical management ( 1 ). Based on SAMMPRIS and VISSIT trial, medical management rather than stenting is recommended with symptomatic ICAS, while stenting is approved for patients who had ≥2 strokes despite medical treatment ( 2 , 3 ). However, with the proper patient selection and the best interventional techniques being revealed gradually, the intracranial stenting shows more promising efficacy, still being expected to improve the treatment of ICAS.…”

Combinatorial treatment for unresectable unicentric Castleman disease

“…The international consensus and treatment guidelines from the Castleman Disease Collaborative Network (CDCN) recommend complete surgical extirpation as the preferred approach 3 . The long‐term outcome is excellent with complete surgical resection, resulting in 3‐year disease‐free survival of 92% and an overall survival of 91% in one large series 4 .…”