Isolated giant-cell arteritis in small bowel. Case report and review of the literature.
Isolated giant cell arteritis of the small bowel is an infrecuent condition of unknown etiology, with only a few cases described in the literature.
Materiales y métodos
We report a case and made a medline search in order to review the English literature about this entity, by the key words: Isolated, giant-cell arteritis, vasculitis and small bowel.
A 68 years-old white female with presented ischemic signs in a prolapsed jejunostomy (18cm). This jejunostomy was a result of a complicated Nissen fundoplication 6 years before. She didn´t present any symptom related to sistemic disease, like asthenia, fever, arhtralgia or myalgia. Laboratory studies and ESR were normal. Pathology findings showed ischemic necrosis and giant-cell arteritis in the small arteries of the submucosa, just in the healthy area. As a result of pathologic findings, she was carefully explored, without any evidence of systemic vasculitis. Our patient has remained asymptomatic and ESR has remained normal until now.
It was concluded that the diagnosis must be ischemic necrosis due to mechanical prolapse of jejunostomy. Giant cell-arteritis was considered an incidental discovery, as it was only found in the healthy area. Polyarteritis nodosa, Behçet disease, rheumatoid arthritis, and systemic lupus erythematosus are systemic vasculitic processes which frecuently produce a mesenteric ischemia. There is no reference of Wegener vasculitis manifestated as isolated vasculitis in this location and very infrequently, Churg-Strauss syndrome presented as isolated vasculitis in small bowel. Giant-cell arteritis involving large arteries of mesenteric vasculature associated with infarction or ischemia has been reported as manifestation of giant-cell (temporal) arteritis syndrome in 12 cases. There are only a few cases of isolated giant-cell vasculitis in the literature, located among others in breast (8 cases), lung (2 cases), sinovium (1), genital tract (18 cases) and small bowel (1 case). Cases, as our patient, with isolated giant-cell vasculitis affecting only small arteries, are very rare and most of them have been described in the female genital tract as an incidental discovery without systemic involvement after long-term follow up. One case located exclusively in small bowel developed systemic disease in a follow-up of 5 years. Therefore, the significance of this finding in small bowel is controversial, thus it is mandatory a complete exploration of the patient and strict follow-up to rule out systemic disease.