A case of systemic lupus erythematosus complicated by alveolar hemorrhage and cytomegalovirus colitis.

We report a rare case of systemic lupus erythematosus (SLE) complicated by alveolar hemorrhage and cytomegalovirus (CMV) colitis. Despite the successful treatment of lupus nephritis by steroid pulse therapy, the patient developed an acute alveolar hemorrhage 2 months later. Cyclophosphamide pulse therapy ameliorated the hemorrhage. One month later, she suddenly developed melena secondary to CMV colitis. Antiviral therapy was successful. We emphasize the importance of timely and precise differential diagnosis for successful management of complicated SLE.

Low-dose epoprostenol improved pulmonary hypertension in a patient with systemic lupus erythematosus.

A 43-year-old Japanese woman was referred to our hospital in 1997 because of Raynaud's phenomenon. Systemic lupus erythematosus was diagnosed on the basis of the presence of antinuclear antibody (1:1,280), anti-DNA antibody (1:640), anti-Sm antibody, antiphospholipid antibody, lymphopenia, and proteinuria. She developed pulmonary fibrosis in 1999 and pulmonary hypertension in 2001. In October 2002, a 24-hr continuous infusion of epoprostenol was started. Dyspnea, Raynaud's phenomenon, and pulmonary hypertension improved with low-dose epoprostenol (3.0 to 4.0 ng kg(-1) min(-1)). The patient could not tolerate larger doses of epoprostenol so 4.0 ng kg(-1) min(-1) was selected as the maintenance dose. The clinical course was uneventful at this dosage. It appears that pulmonary hypertension can be controlled with low-dose epoprostenol such as 3.0 to 4.0 ng kg(-1) min(-1) in some rheumatic patients.

Wegener's granulomatosis complicated with intestinal ulceration.

Abstract We report the case of a 32-year-old man who developed Wegener's granulomatosis complicated with refractory intestinal ulceration. In August 2001, he presented with a high fever, nasal bleeding, and bilateral leg numbness. These symptoms worsened, which prompted him to consult his home doctor on February 18, 2002. In spite of treatment with antibiotics, his symptoms did not improve. Furthermore, abdominal pain and melena occurred as additional symptoms in March 2002. He was admitted to our hospital on April 5, 2002. A deformed nose condition (the so-called saddle nose) was observed at this time. Laboratory data showed a high erythrocyte sedimentation rate (103 mm/h) and a high level of serum C-reactive protein (14.98 mg/dl), and hematuria and proteinuria were also observed. The patient was positive for an antineutrophil cytoplasmic antibody specific for proteinase-3 (PR3-ANCA). A chest computed tomography (CT) scan revealed multiple pulmonary nodules in the lung field. A biopsied-specimen from the nasal mucosa showed necrotizing granulomatosis with giant cells. Together with his symptoms and the laboratory and pathological findings, the patient was diagnosed as having Wegener's granulomatosis. A colon fiberscopy showed multiple ulcerations with bleeding from the terminal ileum to the ascending colon, and nodular lesions at the terminal ileum. We started a combination therapy of prednisolone (60 mg/day) and cylophosphamide (100 mg/day) orally. The patient's gastrointestinal symptoms disappeared and abnormal serological indicators improved. Although Wegener's granulomatosis complicated with refractory intestinal ulceration is rare, this case indicates that the gastrointestinal region is also a target organ of Wegener's granulomatosis.

Identification of candidate genes for Sjögren's syndrome using MRL/lpr mouse model of Sjögren's syndrome and cDNA microarray analysis.

Sjögren's syndrome is a chronic autoimmune disease characterized by focal lymphocytic infiltration of lacrimal and salivary glands, but the precise mechanism of this syndrome is poorly understood. To clarify the mechanism of onset and progression of Sjögren's syndrome, it is necessary to identify Sjögren's syndrome-related genes. For this purpose, we used MLR/MpJ-lpr/lpr (MRL/lpr) mouse as a model of human secondary Sjögren's syndrome and analyzed specific mRNA expression pattern in MRL/lpr mouse salivary glands by in-house cDNA microarray. Among arrayed 2304 genes, 13 genes were isolated as highly expressed genes in MRL/lpr mouse salivary gland in comparison with MRL/MpJ-+/+ (MRL/+) mouse tissue. Subsequently, we performed RT-PCR analysis and confirmed the high expression level of nine genes; caspase3, Ly-6C.2, vimentin, Mel-14 antigen, cathepsin B, mpt1, Laptm5, Gnai2 and UCP2. Five of the nine genes have already been identified in patients with Sjögren's syndrome or mice models of the syndrome, but the remaining four genes; mpt1, Laptm5, Gnai2, and UCP2 have not been reported previously as Sjögren's syndrome-related genes. Although, further experiments are necessary to examine the relationship between these four genes and Sjögren's syndrome, our system of mouse model of Sjögren's syndrome combined with in-house cDNA microarray is suitable for the isolation of Sjögren's syndrome-related genes.