RESUMO
Nonresponsive celiac disease (CeD) is relatively common. It is generally attributed to persistent gluten exposure and resolves after correction of diet errors. However, other complications of CeD and disorders clinically mimicking CeD need to be excluded. Novel therapies are being evaluated to facilitate mucosal recovery, which might benefit patients with nonresponsive CeD. Refractory CeD (RCeD) is rare and is divided into 2 types. The etiology of type I RCeD is unclear. A switch to gluten-independent autoimmunity is suspected in some patients. In contrast, type II RCeD represents a low-grade intraepithelial lymphoma. Type I RCeD remains a diagnosis of exclusion, requiring ruling out gluten intake and other nonmalignant causes of villous atrophy. Diagnosis of type II RCeD relies on the demonstration of a clonal population of neoplastic intraepithelial lymphocytes with an atypical immunophenotype. Type I RCeD and type II RCeD generally respond to open-capsule budesonide, but the latter has a dismal prognosis due to severe malnutrition and frequent progression to enteropathy-associated T-cell lymphoma; more efficient therapy is needed.
Assuntos
Doença Celíaca , Doença Celíaca/diagnóstico , Doença Celíaca/terapia , Doença Celíaca/imunologia , Doença Celíaca/dietoterapia , Humanos , Dieta Livre de Glúten , Mucosa Intestinal/patologia , Mucosa Intestinal/imunologia , Mucosa Intestinal/efeitos dos fármacos , Glutens/imunologia , Glutens/efeitos adversos , Resultado do Tratamento , Budesonida/uso terapêuticoRESUMO
Following the introduction of tacrolimus, intestinal transplantation is now a valid option for patients with chronic intestinalfailure. However, its outcome is undermined by the abundant lymphoid component of the graft and the septic nature of the procedure. The heavy immunosuppression necessitated by this transplant, and its non specific nature, creates a risk of infectious and tumoral complications. Several approaches are being tested to improve the immune tolerance of intestinal grafts, both in animals models and in the clinic. The most promising seek to induce specific tolerance while sparing antimicrobial and antitumoral immunity.