Rapid formation of aortoesophageal fistula complicated by mycotic thoracic aortic aneurysm secondary to infective endocarditis.

A 73-year-old man was admitted with four weeks of intermittent fever. He had a history of total aortic arch replacement for aortic arch aneurysm four years prior. CT scans showed no abnormalities before admission. Repeated blood cultures yielded Streptococcus anginosus and Prevotella melaninogenica, suggesting infective endocarditis (IE). Transesophageal echocardiography revealed a vegetation on the aortic valve, confirming IE. He suddenly presented with massive hematemesis and hypotension. Endoscopy revealed an elevated lesion with a laceration but no active bleeding in the esophagus. CT scans showed a thoracic aneurysm involving the esophagus. A diagnosis of aortoesophageal fistula (AEF) complicated by mycotic thoracic aortic aneurysm (MTAA) was made, and he underwent stent graft interpolation followed by minimally invasive esophagectomy. MTAAs are more prone to rupture than arteriosclerotic aneurysms as they are usually not true but pseudoaneurysms. Antecedent infection, including endocarditis, sepsis, predisposes to MTAA. AEF is a rare but life-threatening cause of gastrointestinal bleeding characterized by Chiari's triad. There have been no reports of such rapid formation of AEF after the graft replacement, as shown here. A recent article reported a rapid formation (16 days) of AEF after thoracic endovascular aortic repair, emphasizing prosthetic infection as the most important risk factor. Our case underscores the importance of suspecting AEF and conducting repeated appropriate examinations even if initial examinations do not reveal any aneurysms.

Ulcerative colitis exacerbated by strongyloidiasis.

We herein report a case of ulcerative colitis (UC) exacerbated by strongyloidiasis. Parasites including Strongyloides stercoralis and Entamoeba histolytica can cause chronic gastrointestinal inflammation and long-lasting symptoms resembling UC. On the other hand, it is not well-known that such organisms can trigger the exacerbation of pre-existing UC. We would like to highlight the importance of recognition of strongyloidiasis in the management of UC patients who have lived in or migrated from endemic regions, such as Asia, Africa, and South America.

A case of abdominal tuberculosis lymphadenitis extending into the gallbladder and duodenum in a human T-cell lymphotropic virus type 1 carrier.

A 69-year-old man with a history of pulmonary tuberculosis presented with fever. He tested positive for the QuantiFERON TB-2G and human T-cell lymphotropic virus type 1 antibodies. Imaging revealed a mass in the neck of the gallbladder, with periportal lymph node enlargement and penetration into the duodenum. A definite diagnosis could not be made, even with a subsequent detailed examination. Finally, cholecystectomy and a lymph node biopsy were performed. Histopathology revealed a caseating granuloma in the lymph nodes and in the serosa of the gallbladder, and polymerase chain reaction was positive for tuberculosis. Therefore, the patient was diagnosed with abdominal tuberculosis lymphadenitis extending into the gallbladder and duodenum.

Whipple's disease: the first Japanese case diagnosed by electron microscopy and polymerase chain reaction.

A 52-year-old man presented with diarrhea and 20 kg weight loss in one year. Enteroscopy showed diffuse yellow-white shaggy mucosa in the duodenum and jejunum. Biopsies of the duodenal mucosa revealed massive infiltration within the lamina propria by foamy macrophages strongly positive for periodic acid-Schiff stain. Electron microscopy demonstrated numerous bacilli within macrophages of the lamina propria. Tropheryma whipplei DNA was detected by polymerase chain reaction. The definitive diagnosis of Whipple's disease was made. Antibiotic therapy dramatically improved his clinical picture. This is the first Japanese case with Whipple's disease diagnosed by electron microscopy and polymerase chain reaction.

Successful long-term treatment with cyclosporin A in protein losing gastroenteropathy.

Protein-losing gastroenteropathy (PLG) can occur as a manifestation of various diseases including autoimmune disorders, and optimal therapy of these underlying diseases may be the only effective remedy for PLG. In the present report, we describe a case of a 54-year-old woman with PLG associated with an autoimmune disease, presumably CREST syndrome. She failed to respond to steroid treatment. Subsequently, cyclosporine was initiated, which resulted in a rapid recovery. The patient was successfully treated with low-dose cyclosporine for five years. There has not been, to our knowledge, any report of PLG successfully treated with cyclosporine. Cyclosporine therapy may be effective not only in inducing but also in maintaining complete remission in patients with autoimmune-associated PLG, especially refractory or intolerable to steroids and/or immunosuppressive therapies.

Pulseless hematochezia: Takayasu's arteritis associated with ulcerative colitis.

A 36-year-old woman with ulcerative colitis presented with fever, chest and back pain, and fatigue sensation of the arm. Her upper limb pulses were absent. Angiography showed multiple aneurysms of the aorta and its branches, consistent with Takayasu's arteritis. She showed HLA-B35 but no B52, which is the typical haplotype among the coexistence cases of both diseases. Prednisolone was effective. The possible pathogenic association of the disorders is discussed.

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.