Whipple's disease with normal duodenal histology diagnosed by ileal biopsy using balloon endoscopy.

A 34 year-old previously healthy Japanese woman presented with a 4 year history of migratory arthralgia, chronic diarrhea, mild fever, and 10 kg weight loss. She was initially misdiagnosed with seronegative arthritis, followed by eosinophilic gastroenteritis. Oral prednisolone was found to be ineffective. Upper endoscopy revealed normal duodenal mucosa. Duodenal biopsy specimen indicated no abnormalities. However, balloon endoscopy revealed white shaggy villi in the ileum, and a biopsy specimen from which revealed diffuse infiltration of foamy macrophages in the lamina propria. These macrophages contained diastase-periodic acid-Schiff positive granules, consistent with Whipple's disease. Polymerase chain reaction analysis of DNA extracted from the ileum was compatible with Whipple's disease. In most previously reported cases of Whipple's disease, the duodenum was affected and duodenal biopsy specimens led to a diagnosis of Whipple's disease. Whipple's disease with normal duodenal histology is extremely rare and balloon endoscopy might be a useful diagnostic tool in such a case. The patient was treated with intravenous ceftriaxone and oral trimethoprim-sulfamethoxazole, and her symptoms improved one month after treatment. Balloon endoscopy performed 32 months later revealed complete improvement in lesions of the ileum.

An inverse relationship between autoimmune liver diseases and Strongyloides stercoralis infection.

A case-control study was undertaken to describe the prevalence of Strongyloides stercoralis infection among patients with autoimmune liver diseases, such as primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH), and primary sclerosing cholangitis (PSC). This study covered 4,117 patients who were admitted to hospitals in Okinawa, Japan, between 1988 and 2006. During this period, 538 patients had the following chronic liver diseases: PBC, AIH, PSC, chronic viral hepatitis group, and alcoholic liver disease. The other 3,579 patients who were hospitalized and underwent parasitologic tests served as controls. The frequency of S. stercoralis infection in the autoimmune liver diseases group (1.0%) was lower than that found in the control group (7.0%; P = 0.0063). None of the female patients with PBC born before 1955 had S. stercoralis infection, which was also statistically significant (P = 0.045). We hypothesized that immunomodulation by S. stercoralis infection may lower the incidence of autoimmune liver disease.

[Recurrent esophageal cancer with complete response to TS-1 chemotherapy].

An 80-year-old man was admitted to our hospital for treatment of recurrent esophageal cancer in December, 2004. He was diagnosed as having esophageal cancer of stage IVa (T2N4M0) in October, 2002, and he received chemoradiotherapy (nedaplatin (CDGP)/5-fluorouracil (5-FU) total 6 course+60 Gy). Afterwards, lymph nodes recurred, and two courses of CDGP/vindesine were given. Then, the primary lesion showed a complete response (CR), and lymph nodes a partial response (PR). In December, 2004, paraesophageal lymph nodes were enlarged to the size of 7 cm. On admission, because of renal disturbance and dementia with advanced age, we chose chemotherapy with TS-1 (100 mg/body/day, three weeks of administration, then two weeks of withdrawal). He had adverse effects of hematotoxicity of grade 3, and non-hematotoxicity of grade 1. He received 6 courses of this regimen and eventually showed CR. Serum SCC was decreased from 4.7 ng/mL to 0.9 ng/mL. At present,the lesions have not recurred during the follow-up for 18 months.

A rare association of primary biliary cirrhosis and pernicious anemia.

Primary biliary cirrhosis is often associated with autoimmune diseases. However, an association between primary biliary cirrhosis and pernicious anemia has rarely been reported. We report a patient with primary biliary cirrhosis associated with pernicious anemia and autoimmune gastritis. The patient was a 64-year-old Japanese woman who had been diagnosed as having primary biliary cirrhosis 5 years previously. She was readmitted with jaundice and macrocytic anemia. The diagnosis of pernicious anemia was confirmed by the low level of serum vitamin B12 and the presence of anti-parietal cell antibody and anti-intrinsic factor antibody. Pernicious anemia should be regarded as a possible complication of primary biliary cirrhosis.