Case Presentation: A 27-year-old male with no significant medical history presented with a 3-day history of peri-umbilical pain localized to the right iliac fossa. Pain was sharp in nature, constant and aggravated by movement. No bowel or urinary symptoms and no previous abdominal surgeries. No history of inflammatory bowel disease or sarcoidosis. Upon admission, had a low-grade pyrexia. Examination findings revealed a soft abdomen with tenderness and guarding in the right iliac fossa. There appeared to be a fullness over the tender area, but there was no rebound tenderness and Rovsing’s sign negative. Urinalysis revealed no abnormality and routine blood tests showed a mild leukocytosis of 12.5 × 109/l (range, 4.0–11.0 × 109/l) with a neutrophilia of 10.5 × 109/l (range. 2.0–7.5 × 109/l). CT showed inflammation and thickening of the distal appendix with faint adjacent stranding, decision was made to perform a laparoscopic appendicectomy. At laparoscopy, the appendix appeared inflamed. Histology confirmed granulomatous appendicitis. He had an uneventful postoperative recovery and 2 months further remains in good health with no new systemic symptoms with appropriate gastroenterology follow up for screening for inflammatory bowel disease.

Discussion: Granulomatous inflammation of the appendix is very uncommon. It can be caused by a variety of conditions, including systemic disorders such as Crohn’s disease and sarcoidosis, and infections such as mycobacterium tuberculosis, yersinia pseudotuberculosis, parasites and fungi. We present a young 27 year old male without any previous history nor family history of inflammatory bowel disease who was found to have granulomatous appendicitis.

Conclusions: Granulomatous appendicitis as an isolated pathological entity unassociated with systemic disease is rare. It accounts for less than 2% of all cases of appendicitis. Isolated granulomatous inflammation of the appendix of unknown etiology, otherwise known as idiopathic granulomatous appendicitis is extremely rare. Patients with this condition present with the typical signs and symptoms of acute appendicitis. The initial belief that it represented a manifestation of Crohn’s disease is incorrect in the great majority of cases, as only 5–10% of patients with granulomatous appendicitis develop Crohn’s disease elsewhere in their gastrointestinal tract. The remaining etiologies are diverse. Unusual causes include sarcoidosis, foreign body reaction, or infections such as mycobacterium, fungal, or parasitic. These conditions combined explain less than 10% of cases. More recently, two etiologies have been recognized that potentially account for most of the previous “idiopathic” cases of granulomatous appendicitis. The first is infection by pathogenic Yersinia species, demonstrated in approximately 25% of cases. The second cause may be the most common of all, namely subacute or recurrent appendicitis with interval appendectomy. The majority of patients are cured by appendectomy alone. Interestingly, appendectomy has been shown to provide some protection against developing inflammatory bowel disease and in reducing its severity if performed before the onset of disease. Granulomatous appendicitis should be considered in young healthy persons and appropriate gastroenterology follow up for screening for associated autoimmune diseases must be considered.