Clinical Scenario

Refractory coeliac disease type 2 (RCD-II) is a specific presentation of celiac disease in which an aberrant intraepithelial lymphocyte (IEL) population is identified in the small bowel despite at least 12 months on a strict gluten-free diet (GFD).

Defining Characteristics of RCD-II

Aberrant IEL population in the small bowel with loss of surface CD3 and CD8, but with intracytoplasmic CD3 expression retained.
Flow cytometry typically demonstrates ≥ 20% aberrant IELs in the small bowel mucosa.
Clonality confirmed by T-cell receptor (TCR) gene rearrangement analysis.
Present despite at least 12 months on a strict gluten-free diet (GFD).

Treatment Approach — Partial Overview

In selected patients with RCD-II, management may involve purine nucleoside analogue-based therapy, with or without autologous haematopoietic stem cell transplantation, or an alternative targeted approach.

Full regimen, sequencing, and dosing details are available in the complete protocol ↓

References

DOI: 10.1002/ueg2.70195

RCD‑II: Is defined by the presence of an aberrant IEL population characterised by loss of surface CD3 and CD8, with expression of intracytoplasmic CD3.
Flow cytometry typically demonstrates ≥ 20% aberrant IELs, and clonality is confirmed by T‑cell receptor (TCR) gene rearrangement analysis.
In selected patients, cladribine (or fludarabine), with or without autologous haematopoietic stem cell transplantation (auto‑HSCT), or JAK inhibitors may be considered on an individual basis.

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Treatment of Celiac Disease in Refractory Coeliac Disease Type 2 (RCD-II) with Aberrant Intraepithelial Lymphocytes

References