Link between CF and Sjögren disease?

Question

Is there a link between CF and Sjögren disease?

Answer

Dear,
Thank you for this question.
Until present there is no clear link between cystic fibrosis and Sjögren disease. In the literature there is however one case report of a 47-year old woman who had pancreatitis AND Sjögren disease and in whom 2 mutations in the CFTR gene had been detected. There was however no clear connection between both diseases.
Sjögren disease is as you know an auto-immune disorder. In case of auto-immune disorders, antibodies are made against substances in one’s own body. This means that the immune system attacks the own body rather than foreign material such as bacteria or viruses. Examples of immune disorders are inflammatory bowel disease (Crohn’s disease, colitis ulcerosa), auto-immune disorders of the thyroid (Hashimoto thyroiditis, Graves disease), diseases of the joints (rheumatoid arthritis), diabetes mellitus type I.
Also in patients with CF, raised titres of antibodies against ‘self’ can be found. The significance of these antibodies and the connection with the severity of cystic fibrosis are unclear. There is a suspicion that the production of auto-antibodies is a consequence of the chronic infection and stimulation of the immune system. Others state that making auto-antibodies is only seen in a subgroup of patients with cystic fibrosis in whom the immune system functions differently. The influence of autoantibodies on the course of cystic fibrosis is yet unclear.
Patients with cystic fibrosis can develop auto-immune disease. Most frequent are joint problems. Diabetes type I is also an auto-immune disease, but there is at present no evidence that diabetes type I is more frequent in patients with CF. When they develop diabetes it is a consequence of destruction of the pancreas and insensitivity of the body to insulin.
In general one can state that there is a link between cystic fibrosis and auto-immune disorders, but many facts in this link are incompletely understood.
We hope that this answers your question.
Prof. Kris De Boeck

Pancreas. 2007 Jul;35(1):94-5. Coincidental CFTR mutation and Sjögren syndrome revealing a pancreatic disease. Frossard JL, Dumonceau JM, Spahr L, Giostra E, Hadengue A.
Eur Respir J. 2009 Nov;34(5):1079-85. Epub 2009 May 14. Prevalence and clinical significance of auto-antibodies in adults with cystic fibrosis. Lachenal F, Nkana K, Nove-Josserand R, Fabien N, Durieu I.

03.05.2012