Ryumachi. [Rheumatism]最新文献

Purpose: To compare the bone-mass effects of intermittent cyclic etidronate administration in patients of various rheumatic disease patients with corticosteroid-induced osteoporosis.

Patients and methods: We evaluated bone mineral density (BMD) of lumbar spine in 34 female patients (mean age: 46.4 +/- 13.7 y. o. 17-71) treated with long term corticosteroid (> 6 months). Eighteen patients cyclically received etidronate orally (400 mg or 200 mg etidronate daily for 2 weeks, followed by 10-12 weeks drug-free periods). Twelve in these 18 patients received 400 mg (group A) and another 6 patients were treated with 200 mg/day (group B). Sixteen patients free from etidronate administrations were analysed as a control group.

Results: Cyclical etidronate therapy showed significant increase in BMD. The BMD of lumbar spine increased from 0.760 +/- 0.10 g/cm 2 to 0.783 +/- 0.11 g/cm 2 (%change from baseline 2.91 +/- 2.56%/year) in group A treated patients after 12 months. Reduced BMD (%change from baseline 1.55 +/- 2.48%) was observed in 16 control group patients (P < 0.0012). The BMD in group A was significantly high compared to group B or control after the etidronate treatment. In 7 of group A, BMD increased significantly on 6 months but no more significant increase was shown on 12 months compared to the value on 6 months. On the other hand the BMD tend to increased for after 2 years in intermittent cyclic etidronate treatment in 8 cases of group A. There were no adverse effects and abnormal laboratory data related to the administration of etidronate. Although only 2 cases of group A showed the findings of compression fracture before the study, but no new compression fracture appeared in any group during this study.

Conclusion: It was shown that cyclical etidronate therapy is effective for steroid induced osteoporosis.

The patient was a 58 year-old woman. Starting about seven months before the initial examination, the patient began to experience a general malaise, coupled with reddish purple scaling eruption on the MP joints, knuckles, elbows, and knees, and dark purplish erythema on both upper eyelids. About two months before the initial examination, the patient visited a local medical doctor because she began to experience arthralgia and muscular weakness. Based on the condition of her weakening proximal muscles and increased levels of myogenic enzymes, the patient was diagnosed as having Dermatomyositis. Oral administration of PSL (25 mg a day) was initiated, and the patient was referred to our department to undergo thorough examination. Although deterioration in muscular strength and elevation of myogenic enzyme levels were mild during the initial examination, the level of KL-6 was significantly elevated to 2600 U/ml. Numerous blood gas analyses and chest CT did not reveal any exacerbation of interstitial pneumonia. Various tests were performed to determine whether or not there was any malignancy, and although a small amount of ascites fluid was detected, diagnostic imaging revealed no clear abnormalities. Since cytological diagnosis of the ascites fluid was class V, an exploratory laparotomy was performed, and ovarian cancer complicated by peritoneal metastasis was discovered. As a result, the patient was diagnosed as having dermatomyositis accompanied by ovarian cancer. The level of KL-6 in the ascites fluid was significantly high at 10,900 U/ml, and immunohistological staining using anti-KL-6 antibodies confirmed the presence of KL-6 in the ovarian tumor, thus suggesting that KL-6 was produced by the ovarian cancer.

A 24-year-old Japanese woman was admitted to our hospital in January 2000 with daily diarrhea, abdominal distention, and abdominal pain. Raynaud's phenomenon with erythroderma desquamativum eruptions had been found in 1992. In 1999, small intestinal transillumination showed dilation of the second and third portion of the duodenum. She was diagnosed as amyopathic dermatomyositis because of Gottron's sign despite not having symptoms of myositis, normal serum CPK levels and no histological abnormality by muscle biopsy. In addition, the patient was diagnosed as systemic sclerosis sine scleroderma because Raynaud's phenomenon, leukoma and gastrointestinal dilation were present. Also, antinuclear antibody and anti centromere antibody were positive and anti Scl-70 antibody was dull-positive, despite the absence of scleroderma, extreme edema, and bone resorption. Careful, long term observation will be required because of the splanchnopathy in youth and the complications of systemic sclerosis.

We report a case of adult polyarteritis nodosa (PN) associated with group A streptococcal infection. A 37-year-old male had suffered from high fever, polyarthralgia, myalgia, and exanthema following pharyngalgia. He was admitted to the hospital because of cutaneous ulcers and necrosis making the general condition rapidly poor. A serological streptococcal test showed a marked increase in antistreptlysin-O (ASO) and a positive reaction to C polysaccharide, suggesting fulminant streptococcal infection. Various antibiotics including penicillin agents were administered. However improvement and exacerbation were repeatedly noted. In the hospital course peripheral neuritis and subcutaneous nodes in upper extremities developed. Biopsy specimen of subcutaneous nodes revealed necrotizing angiitis. Administration of a steroid achieved complete response, and the symptoms, inflammatory reaction, and ASO level improved. While the dose of the steroid was tapered gradually, recurrence was noted. However, increasing the dose finally resulted in relief. During the 2-year follow-up, there was a correlation between the ASO level and inflammatory reaction.

A 63-year-old man visited our hospital in January 1993 because of back pain, which had been present for a year and persisted. The patient was diagnosed compression fracture of thoracic spine by another hospital. Thoracic plain radiographs revealed destructive and sclerotic changes with reduction of height of T 8, T 9 vertebral body. He had kyphosis on this level. Radiographs of the chest revealed hyperostosis of bilateral proximal clavicle. We diagnosed SAPHO syndrome (synovitis, acne, pustlosis, hyperostosis, and osteomyelitis: SAPHO) with T 8, T 9 spondylodiscitis, however without any skin manifestations. Oral indomethacin was effective, however thoracic kyphosis progressed gradually. Spastic gait and paraplegia appeared from February 1998, at last on July he was unable to walk independently. MRI showed the compression of spinal cord on T 8, T 9 level. We performed circumferential decompression and fusion with instrumentation. His paraplegia improved after surgery. We describe a rare case of SAPHO syndrome with paraplegia due to a thoracic kyphosis.

A 57-year-old woman was found to have rheumatoid arthritis (RA) in 1996. Treatment with different immunoregulatory agents, including actarit and bucillamine, produced no improvement. Therefore, combined therapy with methotrexate (MTX) (5 mg/week oral) and low-dose prednisolone (PSL) (5 mg/day) was started in April 1997. Sulindac and famotidine were also administered. In August 1997, she was admitted to our hospital because of palpitations and shortness of breath due to severe anemia. Results of laboratory studies were hemoglobin, 2.9 g/dl; reticulocyte count, 225/1000; and haptoglobin, less than 10 mg/dl. The direct and indirect Coombs'tests were positive. A diagnosis of autoimmune hemolytic anemia (AIHA) was made on the basis of the laboratory findings. Treatment with high-dose PSL (50 mg/day) was started, and the anemia improved. The hemoglobin level increased to 6.0 g/dl within the 1st week and to 12.6 g/dl 6 weeks later. We believe that the most likely explanation for this anemia was the low-dose MTX because the anemia appeared soon after treatment was started.

We report here a case of interstitional pneumonia (IP) associated with amyopathic dermatomyositis (DM). In August, 1998, a 53-year-old Japanese man was admitted to our hospital because of fever, polyarthritis and erythematous heliotrope eruption and Gottron's sign without any symptom of myositis. Serum CK level and EMG were normal. Jo-1 antibody was negative. Chest computed tomography (CT) revealed IP at both lung base areas. The patient was diagnosed as amyopathic DM with IP. When IP rapidly progressed, methylprednisolone pulse therapy and oral high dose prednisolone were not effective. High-dose intravenous cyclophosphamide seemed to be transiently effective, but oral cyclosporine A was not effective. The patient was died of respiratory failure in October 1998. During the course, we measured serum levels of LDH, sIL-2 R, s-ICAM-1 and KL-6, KL-6 could be a sensitive parameter of IP activity.