Update on the Diagnosis and Management of Refractory Coeliac Disease

Table 1

Summary of treatment modalities evaluated in RCD.

Therapy	No. of patients	Therapeutic effect	Notes and side effects	Reference
Thioguanine	12 RCDI	10 patients tolerated TG. clinical and histological response was observed in 83% and 78% respectively	1 patient died within 4 months of therapy due to progression of RCDI. Side effects: muscle spasms, elevation of biochemical liver tests	[44]
Azathioprine and Prednisone	10 RCDI; 8 RCDII	Clinical improvement in all patients in both groups. 8 RCD type I patients responded histologically, complete histological normalization in 4 patients	7 RCDII patients died from EATL	[41]
Mesalamine and Budesonide	10 RCDI	5 patients had complete symptom relief. No conclusion on histological improvement	4 patients had concomitant microscopic colitis. Side effect: headaches	[40]
Budesonide	23 RCDI; 5 RCDII	Overall, 76% of the patients had a clinical response to budesonide, considered as complete response in 55%. No histological improvement in any patient. RCDII patients had persistent clonal proliferation of IELs	1 patient with RCDII died of sepsis and malnutrition. 7 patients had concomitant microscopic colitis. There were no serious adverse events reported	[39]
Infliximab	1 RCDI	Excellent clinical results. Treatment was continued over the following 2 years with a return to near normal histology	No serious adverse events reported	[43]
Infliximab	1 RCDI	Complete clinical improvement. Marked histological improvement	No serious adverse events reported	[42]
Cyclosporin	13; (no differentiation)	Clinical response in 8 patients. Normalisation of histology in 5 patients	No serious adverse events reported	[54]
Cladribine	32 RCDII	Clinical response was observed in 81%, complete histological response in 47% and immunological response in 41%. 5 year survival in those who responded was 83% compared to 22% in those who did not	In total, 12 of 32 patients died of whom 42% died of EATL	[47]
Autologous stem cell transplantation	18 RCDII	13 patients were feasible for auto-SCT and transplanted successfully. Majority showed clinical improvement. 5 patients showed compete histological remission. 4-year survival rate was 66%	In 5 patients, auto-SCT could not be performed; they all died with a median survival of 5.5 months. 1 patient died because of transplant-related complications. EATL was observed in one transplanted patient, after 4 years of follow-up	[53]