Search

A 59-year-old man was treated for rheumatoid arthritis (RA) for 12 years with methotrexate (MTX) and prednisolone. After MTX-associated interstitial pneumonia developed, he was treated with cyclophosphamide and prednisolone for 7 months. Arthritis worsened, and tacrolimus was added to the treatment regimen. One month later, he had fever, loss of appetite, and dyspnea on exertion. Blood tests showed pancytopenia with large, atypical lymphocytes. Computed tomography showed mild splenomegaly. Bone marrow examination demonstrated CD20-positive, EBER-positive atypical lymphocytes, and hemophagocytosis. Random skin biopsy led to the diagnosis of intravascular large B-cell lymphoma (IVLBCL). The final diagnosis was a hemophagocytic syndrome-associated variant of IVLBCL. Complete remission was achieved after seven courses of R-CHOP. However, within a month, he complained of dizziness. Magnetic resonance imaging revealed focal infarctions in the cerebellum and around the left lateral ventricle. Central nervous system relapse was suspected. Although salvage chemotherapy (CHASER), whole brain irradiation, and intrathecal injection of cytarabine and prednisolone were temporarily effective, he died. Autopsy revealed infiltration of lymphoma cells in the brain and adrenal glands. To the best of our knowledge, this is the sixth case of IVLBCL and the first case of the hemophagocytic syndrome-associated variant of IVLBCL in RA patients in the literature.

A 75-year-old, non-smoker female, with a 31-year history of rheumatoid arthritis (RA) was referred to our hospital for examination of progressive interstitial lung disease (ILD). She had been diagn...

Background Epithelioid sarcoma most frequently occurs in the dermal or subcutaneous area of the distal extremities. To date, there have been three cases of primary pulmonary epithelioid sarcoma reported. We report a case of epithelioid sarcoma that is considered a primary lung tumor. Case presentation A 65-year-old asymptomatic Asian male patient underwent chest radiography during a routine health examination, and an abnormal mass was detected. His past medical history was unremarkable. He smoked 40 cigarettes every day and had slightly obstructive impairment on spirometry. He worked as an employee of a company and had no history of asbestos exposure. He underwent partial resection of the right lung by thoracoscopy. A histological examination of the tumor revealed a cellular nodule of epithelioid and spindle-shaped cells. Some of the tumor cells displayed rhabdoid features and reticular arrangement in a myxomatous stroma. Immunohistochemically, the tumor cells were positive for vimentin, smooth muscle actin (SMA), CD34, and epithelial membrane antigen (EMA); loss of the BAF47/INI1 protein in the tumor cells was also confirmed. A diagnosis of epithelioid sarcoma was established. Careful screening by whole-body positron emission tomography for another primary lesion after surgery did not detect any possible lesion. He had no cutaneous disease. Conclusion To our knowledge, this is the fourth case of a proximal-type epithelioid sarcoma considered as a primary lung tumor.

The patient was a 62-year-old man with anticitrullinated protein antibody-positive rheumatoid arthritis (RA) and a history of repeated respiratory tract infections and organizing pneumonia for eigh...

An epidemic outbreak of tuberculosis resulting from exposure at an autopsy occurred at the National Defense Medical College Hospital. It consisted of 3 tuberculosis patients and 68 infected staff members over a 6-year observation period from 1997 to 2002 after exposure to an index patient. We report the incident and the background of the tuberculosis infection in our hospital. A 72-year-old man with myelodysplastic syndrome was admitted to our hospital and later died. Miliary tuberculosis was diagnosed at autopsy. The first non-periodic health examination was performed on the hospital staff who had contact with the patient. Tuberculosis infection was defined as an area of erythema not less than 30 mm in diameter in the tuberculin skin test. 13 of the 39 hospital staff members who had had contact with the patient were infected, and 3 developed tuberculosis. Of the latter 3 secondary tuberculosis patients, the only contact point two had had with the index case was in the autopsy room, and the other had had contact both in the autopsy room and on the ward. The incidence of tuberculosis infection among the staff who had had contact in the autopsy room alone was higher than among the staff who had had contact on the ward alone (odds ratio = 5.04; 1.08-23.42: 95% confidence interval). Because one of the secondary tuberculosis patients had bronchial tuberculosis, which is a strong source of infection, the second non-periodic health examination was performed on the staff who had had contact with the secondary tuberculosis patient, and 69 inpatients who had had contact with the staff were carefully observed over a two-year period. 58 of the 171 staff members were infected, and none developed tuberculosis. However, 23 patients were diagnosed with active tuberculosis after admission to our hospital during the 6 years from 1997 to 2002. Many of them were elderly patients with underlying diseases. An autopsy was performed on 6 of those who died, but only one was diagnosed with active tuberculosis before death. Five of the six autopsied patients had old, healed tuberculous lesions. This outbreak underlines the need for standard precautions, including anti-air droplet infection at autopsy, because antemortem diagnosis of tuberculosis is not always possible, and there is a risk of elderly hospitalized patients developing tuberculosis if they have been previously infected with Mycobacterium tuberculosis.

Objective To establish proper management of Pneumocystis jiroveci pneumonia (PCP) in rheumatoid arthritis (RA) patients treated with infliximab. PCP has been observed in 0.4% of patients with RA treated with infliximab in Japan. Methods Data from patients with RA (n = 21) who were diagnosed with PCP during infliximab treatment and from 102 patients with RA who did not develop PCP during infliximab therapy were collected from 14 rheumatology referral centers in Japan. A retrospective review of these patients and a case–control study to compare patients with and without PCP were performed. Results The median length of time from the first infliximab infusion to the development of PCP was 8.5 weeks. At the onset of PCP, the median dosages of prednisolone and methotrexate were 7.5 mg/day and 8 mg/week, respectively. Pneumocystis jiroveci was microscopically identified in only 2 patients, although the polymerase chain reaction test for the organism was positive in 20 patients. The patients with PCP had significantly lower serum albumin levels (P < 0.001) and lower serum IgG levels (P < 0.001) than the patients without PCP. Computed tomography of the chest in all patients with PCP revealed ground-glass opacity either with sharp demarcation by interlobular septa or without interlobular septal boundaries. Sixteen of the 21 patients with PCP developed acute respiratory failure, but all survived. Conclusion PCP is a serious complication that may occur early in the course of infliximab therapy in patients with RA. For the proper clinical management of this infectious disease, physicians need to be aware of the possibility of PCP developing during infliximab therapy.