This can affect both genders and includes a variety of manifestations of varying severity. Type 3: Autoimmune manifestations primarily limited to the GI tract. This can affect both genders and may also be considered IPEX-like. There is considerable overlap in these disorders, and it is often unclear how to properly distinguish between them as the responsible genes are generally poorly understood at this time. Treatment
Medical therapy is commonly used, most typically with corticosteroids (budesonide and prednisone). However, some patients are refractory to corticosteroids, and in these patients immunosuppressive therapy with azathioprine, cyclophosphamide, tacrolimus, cyclosporine and infliximab has been described. An intravenous nutrition such as total parenteral nutrition and/or a special diet may be necessary. Hematopoietic stem cell transplantation may be curative. References
Further reading
Montalto, Massimo; DOnofrio, Ferruccio; Santoro, Luca; Gallo, Antonella; Gasbarrini, Antonio; Gasbarrini, Giovanni (January 2009). "Autoimmune enteropathy in children and adults". Scandinavian Journal of Gastroenterology. 44 (9): 1029–1036. doi:10.1080/00365520902783691. PMID 19255930. S2CID 33004674. "Autoimmune Enteropathy". Cincinnati Childrens.
Prevention
Inhalants
Disease caused by exposure to industrial inhalants and burn pits can be prevented with the use of engineering controls (e.g., exhaust hoods or closed systems), personal protective equipment, monitoring of potentially affected personnel, worker education and training. Transplant
The primary prevention of bronchiolitis obliterans in people who have received either lung transplant or HSCT therapy is immunosuppression. In regards to post lung transplantation, the combination of calcineurin inhibitor combined with a purine synthesis inhibitor and a glucocorticoid is the general regimen used. People also have a baseline post-transplant lung function testing done in order to determine if their lung function is declining over time. People who are post-HSCT their immunosuppressive regimen typically includes methotrexate in combination with a calcineurin inhibitor to prevent GVHD, a risk factor for developing bronchiolitis obliterans. Treatment
While the disease is not reversible, treatments can slow further worsening. This may include the use of corticosteroids or immunosuppressive medication which may have an effect on the ability to receive a lung transplant if offered. If patients have difficulty breathing (hypoxemia) oxygen can be supplemented. Routine vaccinations are recommended for patients with chronic lung disease to prevent complications from secondary infections due to pneumonia and influenza.Transplant recipients are at risk for re-developing the disease, as bronchiolitis obliterans is a form of chronic rejection. Evaluation of interventions for its prevention relies on early detection of abnormal spirometry results or unusual decreases in repeated measurements.