Cases of Shanghai fever were subsequently reported in children without pre-existing conditions, primarily from Asian countries [4,5,6]. However, to the best of our knowledge, this is the first report of Shanghai fever that occurred in an adult patient with immunodeficiency.
In the present case, the patient presented diarrhea with fever and septic shock with Pseudomonas aeruginosa infection, proven by the two sets of blood culture. No other species were proven from blood cultures. Hence our case met the criteria of Shanghai fever . Furthermore, widespread patchy necrotizing intestinal lesions with multiple bowel perforations were found during both first and second operation, which was similar to the reported case of Shanghai fever . Actually, pathological examination of the perforated bowel lesions showed proliferation of Gram-positive and negative rods and Gram-positive cocci near the perforation site. These bacteria are suggested to be both causative agent and indigenous bacteria of intestine. We could not perform autopsy because we respected the intention of his family, so that we could not examine the lesions in further detail. Therefore, we were unable to identify the species of rods under this limitation. We clinically diagnosed the case as Shanghai fever.
Among patients receiving chemotherapy, neutropenia is known to be a major risk factor for Pseudomonas aeruginosa infection. The bloodstream, respiratory, and urinary tracts are common infection sites . Persistent lung infection is a known severe condition . In the present case, neutropenia caused by treatment-related MDS may be a risk factor of infection. Furthermore, the patient was administered long-term steroid therapy. Steroid use may cause immunosuppression and is reported to be a risk factor of urinary tract infection caused by Pseudomonas aeruginosa . In the present case, prednisolone use may be a risk factor of infection and neutropenia.
There are some diseases with a similar clinical course. Notably, gastrointestinal Behçet’s disease involves the entire small bowel, and especially manifests with round-shaped deep ulcers in the ileocecal region [9, 10]. Moreover, some studies have shown a relationship between MDS and Behçet’s disease ; however, in these studies most of the patients had coexistent intestinal Behçet’s disease with ulcers of the ileum without eye lesions [12, 13]. Recently, Oka et al. reported that acquisition of trisomy 8 was associated with Behçet’s disease in MDS . The present patient had no other symptoms that met the international criteria for Behçet’s disease , and he was negative for trisomy 8.
Pathological examination showed multiple ulcers and perforation with proliferation of rod-shaped bacilli. The rod was negative for periodic and Schiff stain. Gram staining of the specimen showed Gram-positive and negative rods and Gram-positive cocci near the perforated area. The corresponding clinical manifestations may be present in abdominal tuberculosis, histoplasmosis, sarcoidosis, eosinophilic gastroenteritis, and systemic mastocytosis ; however, pathological examinations showed no characteristic signs of such diseases. Pseudomonas aeruginosa infection was proven by blood culture; thus, we suspected that the pathogen was the cause.
We performed small bowel resection and right hemicolectomy including all of the lesions. Though both high dose corticosteroid use and septic shock are risk factors of anastomotic failure . In this case, avoiding anastomosis would have resulted in a double stoma with jejunostomy and colostomy, which would have significantly decreased his quality of life. Therefore, we performed intestinal anastomosis in the 1st operation. Indeed, in high-risk cases of anastomotic failure, we always should consider the need for colostomy.
The mortality rate of Shanghai fever is relatively high, as it is associated with multiple organ failure. Halder et al. reported that the mortality rate is 23–89% and that the key treatment of Shanghai fever is general intensive care and antipseudomonal agents . We continued intensive care with antibiotics (tazobactam/piperacillin 13.5 g/day) for 12 days, but the patient succumbed to multiple organ failure.