The cause is probably a bacterial infection. The disease affects primarily middle-aged men, and the onset of symptoms is usually slow. Without treatment it is usually fatal.
Risk factors are unknown.
The incidence is 1 out of 100,000 people. The reappearance of symptoms may indicate drug-resistant organisms, requiring a change of antibiotics. Correction of nutritional deficiencies caused by the malabsorption are made with dietary supplements. Without treatment, the condition is usually fatal. Treatment improves the chance of a good outcome.