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Primary intestinal autoimmune disease as a cause of chronic intestinal pseudo-obstruction

The purpose of this letter is to elucidate on the pathophysiology of a disease that is often considered to be idiopathic. Chronic intestinal pseudo-obstruction (CIPO) is a clinical syndrome characterised by ineffective intestinal propulsion in the absence of organic intestinal obstruction. It is a common cause of intestinal failure requiring total parenteral nutrition (TPN). It can be either a primary/idiopathic (neurogenic or myogenic) disorder or secondary to another recognised underlying disease. Most cases of childhood CIPO are congenital enteral neuromuscular diseases; however, neuropathy due to Hirschprung’s disease, Chagas disease, infections, and toxins occur in later childhood. In adults, most cases of CIPO are secondary to progressive systemic sclerosis, dermatomyositis, systemic lupus erythematosus, rheumatoid arthritis, and Sjogren’s syndrome.1,2

We report a case of an adult onset of CIPO secondary to an autoimmune process affecting exclusively …