Ryumachi. [Rheumatism]最新文献

We report a case with microscopic polyangiitis (MPA) complicated by varicella zoster encephalitis. A 60-year-old woman caught a common cold and had acute otitis media in April 1998. Proteinuria and hematuria with hyaline cast were noted at the routine medical check in May, and she was referred to our hospital because of high fever and chest pain. MPA was diagnosed with acute progressive renal failure, pleuritis and elevated anti-neutrophil cytoplasmic myeloperoxidase antibody (MPO-ANCA). Corticosteroid therapy was administered under hemodialysis but MPA was flared several times with various symptoms including interstitial pneumonitis, alveolar hemorrhage and erythema multiforme exudativum. During the course of the disease she developed disseminated varicella zoster and encephalitis. Positive polymerase chain reaction to varicella zoster in cerebrospinal fluid helped to differentiate her encephalitis from central nervous system symptoms due to microscopic angiitis and herpes simplex encephalitis. Combination of corticosteroid and acyclovir therapies for MPA and varicella zoster encephalitis under hemodialysis were successful. The diagnostic process and therapies to these complicated contexts were thought to be very important.

We encountered three patients with pediatric systemic sclerosis. Patient 1 had systemic scleroderma, pigmentation and interstitial pneumonia at the age of 10 years. Nine months after disease onset, she was treated with intravenous cyclophosphamide pulse therapy as induction therapy. After the initial treatment, the following clinical manifestations were dramatically improved: interstitial pneumonia, scleroderma and total skin score. Patient 2 was a 7-year-old girl, who complained of systemic scleroderma and pigmentation, and was found to have pulmonary hypertension. Six months after disease onset, she was administrated intravenous cyclophosphamide pulse therapy. Her scleroderma and total skin score were improved and the pulmonary hypertension did not deteriorate. Patient 3 was a 15-year-old girl. Her initial treatment was vitamin E alone. She was admitted to our hospital two and half years after disease onset. Although she was given immunosuppressive therapy including cyclophosphamide, the severe condition of persisted, and she died after five months. It became possible for patient 1 and 2 to achieve and maintain a marked improvement of the clinical manifestations as a result of cyclophosphamide pulse therapy early in the course of the disease. We further observed that their total skin score was decreasing while the clinical manifestations improved.

We report a case of psoriatic arthropathy complicated with HTLV-I carrier and secondary amyloidosis. She was a 45-year-old woman and was diagnosed as psoriatic arthropathy and HTLV-I carrier in December 1999. She was treated with combination therapy corticosteroid, salazosulfapyridine, methotrexate, cyclophosphamide, and gold sodium. However, her arthralgia and fever was not completely improved. In April in 2001, she was admitted to our hospital because of nausea, vomiting, and diarrhea. Her colon fiberscopic examination showed rubber, erosion, and multiple ulcers of the rectum through descending colon mucosa. Her biopsied specimens of the colon mucosa showed Congo Red dye stained amyloid deposits. Because the loss of stain for Congo Red dye after exposure to potassium permanganate, the deposits were amyloid A protein. This case is considered as a rare case of psoriatic arthropathy and HTLV-I carrier complicated with secondary amyloidosis in a relatively short period (approximately two years) after onset of psoriatic arthropathy.

The patient was a 24-year-old man. Bürger's disease was diagnosed initially based on the finding of bilateral radial artery obstruction, and the patient underwent vascular reconstructive surgery. After the operation, however, formation of false aneurysms was observed frequently in the left brachial artery at the elbow, which necessitated performance of vascular reconstructive surgery up to five times in total, including of the right common femoral artery, which had been punctured several times for investigational procedures. During the clinical course, fever, oral aphthoid ulcers, genital ulcers, and nodular erythema appeared, and incomplete Behçet's disease was diagnosed. Histopathological examination suggested that the vascular lesion was consistent with necrotizing angiitis. The systemic inflammatory findings and angiitis improved following steroid administration. Vascular Behçet's disease is known as a special type of Behçet's disease, but usually develops in the patients with a long history of the disease. Our patient was believed to be very instructive because, when Behçet's disease occurs with angiitis and the epidemiological characteristics agree with those of Bürger's disease like in our patient, differentiation may be difficult.

A 65-year old female, who had been suffered from rheumatoid arthritis, was admitted to our hospital because of fever, oral ulcers, perianal skin ulcers, petechiae in the both legs, hepatosplenomegaly and cervical lymphadenopathy. Her laboratory data showed severe anemia, leukocytopenia, and thrombocytopenia as well as low PT activity, prolonged APTT, decreased fibrinegen and elevated FDP. In addition to raised values of liver enzymes and triglyceride, marked elevation of several cytokines were found. IgM and IgG class antibodies to cytomegalovirus were demonstrated positive and their titers were 2.60 and 938.0, respectively. The study for the aspiration of bone marrow revealed hemophagocytosis of erythrocytes, leukocytes and thrombocytes. Based upon these findings, she was diagnosed as having hemophagocytic syndrome associated with cytomegalovirus infection. Steroid treatment inducing mini-pulse therapy was introduced to her and bought full recovery from the illness. The association of hemophagocytic syndrome to rheumatoid arthritis was reviewed in the literature and five cases were documented to have good prognosis with steroid treatment.

We analyzed the effects of three therapies on 30 patients with childhood systemic lupus erythematosus, and classified the patients into three groups. The therapies were as follows; Group A (8 cases), methylpredni-solone (mPSL) pulses plus oral prednisolone (PSL) alone, Group B (10 cases), mPSL pulses plus oral PSL and mizoribine (MZB) or azathioprine (AZP), Group C (12 cases), mPSL pulses and intravenous cyclophosphamide (IVCY) pulse therapy plus oral PSL and MZB or AZP. Three years after treatment, we compared the laboratory data (C3, C4, CH50 and anti-DNA antibody), the SLEDAI scores and numbers of relapses in these three groups. We demonstrated that group C had the best data, and this data indicated that the median C3, C4 and CH50 increased and that the median anti-DNA antibody and the mean of the numbers of relapses decreased. In conclusion, the combination of immunosuppressants and IVCY appeared to offer great benefits in childhood systemic lupus erythematosus.

We report a case of adult onset Still's disease in a 77 year old man, who was diagnosed as a esophageal cancer 9 months after the onset of this disease. At first malignant lesions in any organs were not found and steroid (prednisolone) therapy was begun and the patient was recovered from manifestations. But while tapering prednisolone to 15 mg, fever, arthritis and rash were observed again. Repeated examination revealed that he had suffered from esophageal cancer. This case is considered as a paraneoplastic syndrome of the esophageal cancer. Patients with adult onset Still's disease should be followed as a paraneoplastic syndrome.

We report a case of 58 years old female with primary Sjögren's syndrome who accompanied pulmonary hypertension and glomerular damage. Renal biopsy revealed interstitial nephritis and glomerular damage. Pulmonary perfusion scintigram revealed diffusely decreased pulmonary perfusion, but the defect was not observed. Immunocomplex positive indicated that immune disorder would damage her lung and kidney. Proteinuria and pulmonary hypertension were improved by high dose of prednisolone and low dose of oral cyclophosphamide treatment. No previous reports had shown pulmonary hypertension and glomerular damage complicated with primary Sjögren's syndrome in same patients at same time. But some reports had suggested immune disorder had caused pulmonary hypertension or glomerulonephritis in patients of primary Sjögren's syndrome. Our patient showed immune disorder, and it might cause pulmonary hypertension and glomerular damage.