Ryumachi. [Rheumatism]最新文献

Objectives: To develop disease burden score concerning bone and joint diseases by evaluating the burdens of rheumatoid arthritis (RA) and osteoarthritis (OA) based on two quantitative measures of quality of life (QOL).

Methods: In a questionnaire, the qualified doctors of Japan Rheumatism Foundation were asked to evaluate patients' QOL, including 6 items of physical functions, 7 items of daily living activities and 3 items of social activities, for three severity levels defined by treatment status. The burdens of RA and OA were determined based on two quantitative measures of QOL, that is, 'principal component score' and '0-0.5-1 score'.

Results: In the two-way ANOVA of severity level and doctor's specialism, two quantitative measures of QOL showed significant differences by severity level, having approximately the same F-statistics each other. The burden of RA was 1.4-times (for 'principal component score') and 1.6-times (for '0-0.5-1 score') as great as that of OA. The differences in burden between RA and OA were observed especially in daily living activities and social activities.

Conclusions: Our methodology may be applicable to examining differences in burden between bone and joint diseases.

A 35-year-old male was admitted to our hospital because of a persistent nasal obstruction and headache. In the laboratory findings, inflammatory reactions were seen, and anti-neutrophil cytoplasmic antibody (PR 3-ANCA) was positive. He was diagnosed with Wegener's granulomatosis (WG) based on the above symptoms, PR 3-ANCA positivity and pathology of nasal mucosa. Chest radiogram showed a solitary lung lesion in the apex of the left lung. The patient was treated with steroid and cyclophosphamide. Symptoms and inflammatory reactions were improved dramatically, however, the size of the solitary lung lesion did not change. Video-assisted thoracic surgery (VATS) was performed to differentiate the lesion from neoplasm. It showed features consistent with WG pathologically. The solitary lung lesion in WG is sometimes difficult to differentiate from lung neoplasm in clinical course, if the lesion does not improve by the standard therapy for WG. So in these cases, VATS is needed to confirm these lesions pathologically.

We investigated the features and the usefulness of gallium scintigraphy in the diagnosis and the assessment of Adult Still's disease (ASD) by retrospective case review. Gallium scintigraphy have been done for 11 cases of ASD (3 males and 8 females) and 4 females were positive. Among these, 67 Ga-citrate was accumulated to the bone marrow in all 4 cases and to the major joints in 2 cases. Positive cases were rather serious and administered more immunosuppressants than negative cases. In order to characterize gallium scintigraphy findings of ASD, i.e. bone marrow accumulation, we analyzed 130 cases of collagen vascular diseases. Although 101 cases (77.7%) were positive, only 7 cases (5.4%) showed the accumulation of 67Ga-citrate to the bone marrow. These include 3 cases with ASD, and 1 case with systemic lupus erythematosus, polyarteritis nodosa, Wegener's granulomatosis and Sjögren's syndrome. We also accumulated 18 patients who exhibited bone marrow accumulation of 67Ga-citrate, and found that 7 patients had collagen vascular and their related diseases. In conclusion, bone marrow accumulation in gallium scintigraphy is a specific feature of collagen vascular diseases, especially ASD, and it is suggested that cases with positive gallium scintigraphy in ASD can be serious and resistant to treatment.

We described three children with juvenile dermatomyositis (JDM) refractory to the conventional therapy. They were successfully treated with intravenous cyclophosphamide (IVCY) pulses, and two of them were administered plasma exchange (PE) before IVCY. Case 1. A 17-year-old girl with JDM was previously treated for 2 years with the combination of prednisolone, intravenous gamma-globulin, methotrexate, and azathioprine. However, muscle weakness gradually progressed. She failed to hold her sitting position and to rise her arms, but both serum CK and aldolase were stable. After the episode of aspiration pneumonia the follow-up muscle biopsy was performed, which revealed muscle degeneration and massive mononuclear cell infiltration in perivascular area. The erythrocyte sedimentation rate (ESR) and fibrin degradation product E (FDP-E) levels were gradually increased. Because the active inflammation of muscle and muscle vasculature was suspected, the PE and IVCY combination therapy was administered. During the 6 courses of the therapy, muscle weakness was markedly improved so that she could hold herself at the sitting position and could have meals by herself. Case 2. A 5-year-old boy with JDM was treated for 8 months with prednisolone p.o., but his muscle strength became worse. The muscle enzyme levels, such as serum CK and aldolase, were not reflecting his status of the disease, but FDP-E levels were increased. Muscle MRI and biopsy revealed the inflammatory changes of perivascular area of muscle. The PE and IVCY combination therapy was effective, and he became able to walk and run by himself. Case 3. A 14-year-old boy was diagnosed as having JDM when he was 10 years of age, and treated with prednisolone p.o., and subsequently with intravenous methylprednisolone pulses and azathioprine. Three years later the flares were observed accompanied with the elevations of serum CK and FDP-E. The administration of IVCY improved muscle strength as well as serum muscle enzyme and FDP-E levels. These findings indicated that the clinical manifestations of JDM should be closely monitored, that the serum levels of muscle enzymes including CK and aldolase were sometimes not indicative for the flares of JDM, and that muscle MRI and re-biopsy examination were needed for the children with progressive muscle weakness. In addition, determination of ESR and FDP-E was not specific but helpful to detect flares of the disease in some cases.

A 66-year-old woman with systemic scleroderma developed pancytopenia (white blood cell 750/microliter, neutrophilic cell 201/microliter, red blood cell 166 x 10(4)/microliter, hemoglobin 5.3 g/dl, hematocrit 18.1%, platelet cell 8.2 x 10(4)/microliter) 7 months after the initiation of intravenous hyper-alimentation for chronic ileus. Serum copper and zinc levels were 3 and 46 micrograms/dl, respectively. Provision of trace elements led to increase blood cell counts as well as serum copper and zinc levels. She also developed watery diarrhea frequent times a day and hypoproteinemia during the lack of trace elements. Evidence of protein-losing gastroenteropathy was shown by gastrointestinal scintigraphy using 99mTc-human serum albumin half a year after provision of trace elements and it was not shown one and a half years after continuous provision of trace elements. As patients with scleroderma sometimes develop gastrointestinal problems and are needed intravenous nutrition of long duration, they should be paid attention to lack of trace elements that can be a cause of hematologic complications.

We herein report a case of systemic sclerosis associated with microscopic polyangitis. The patient was a 54-year-old woman, who was diagnosed to have systemic sclerosis at a hospital in 1992, but she did not receive any medical treatment. She had been suffering from pyrexia, paresthesia and muscle weakness of both lower limbs since the beginning of 2001, and was introduced to our hospital. She showed hardened skin extending from her fingers to upper arms, weakness in both lower limbs and livedo reticularis. Her laboratory test showed WBC 11, 600/microliter, CRP 6.63 mg/dl, CH 50 24 U/ml, anti Scl-70 antibody 90.1 index, and MPO-ANCA 281 EU, but no impaired renal function was recognized. Chest computed tomography showed interstitial pneumonia while necrotising vasculitis of the right sural nerve was found in a biopsy specimen. Based on these findings, we diagnosed her to have systemic sclerosis accompanied with microscopic polyangitis (MPA). She received steroid treatment after the diagnosis was made, and her symptoms and the laboratory findings thereafter immediately improved. Many cases have been reported to have ANCA positive systemic sclerosis among patients with systemic sclerosis that are complicated MPO-ANCA-related vasculitis. However, since our patient demonstrated necrotising vasculitis in a sural nerve biopsy and no evidence of an impaired renal function, we diagnosed her to have systemic sclerosis complicated with MPA instead of ANCA positive systemic sclerosis. The pathological state of this patient thus seemed to be different from that of ANCA-positive systemic sclerosis. We concluded that this patient had both systemic sclerosis and MPA. It is therefore important to note that some patients who have been reported to have ANCA-positive systemic sclerosis may also have systemic sclerosis complicated with MPA.

Objectives: Pulmonary hypertension (PH) is one of the major fatal causes in patients with mixed connective tissue disease(MCTD), which showed remarkable angiopathy from large to small vessels in the lungs. However, the etiology of PH in MCTD is still unknown. Even the lung tissues of MCTD patients without overt clinical PH represent minor vascular damages such as microthrombus or slight intimal thickening. These findings suggest some serum factors cause endothelial cell damage especially to pulmonary micro vessels, which leads to PH in MCTD. To elucidate the mechanisms of PH in MCTD we studied the anti-endothelial cell antibodies (AECA) in the sera of MCTD patients, which are considered to correlate with activity in some collagen diseases, and compared the three kinds of endothelial cells, especially the effects on pulmonary endothelial cells.

Materials and methods: Sera from 14 MCTD patients who satisfied the Kasukawa's criteria in Japan including 4 cases of PH, 8 cases of non-PH and 3 untreated cases, and 5 healthy controls were analyzed as follows: (1) AECA to human pulmonary arterial endothelial cells (HPAEC), pulmonary microvascular endothelial cells (HMVE-L) and human aortic endothelial cells(HAEC) were analyzed by an indirect immunofluorescence method using flow cytometry. (2) Effects of MCTD patients' and healthy controls' sera on cell proliferation and induction of apoptosis on cultured HPAEC were investigated by methods of MTS and TUNEL. (3) A cytotoxic effect of patients' sera in combination with activated NK cells on HPAEC were studied by a method of LDH concentration.

Results: (1) Patients' sera from MCTD have IgG type AECA, and sera from MCTD patients with PH showed a higher intensity of AECA compared with non-PH and control cases (P < 0.01). (2) Only patient's sera revealed no potency of cell proliferation and induction of apoptosis in every kinds of endothelial cells compared with controls. (3) Sera from MCTD patients with PH, and from untreatment patients were high intensity of AECA, which shows cytotoxicity by addition of activated NK cells.

Conclusions: Apoptosis of pulmonary arterial endothelial cells induced by AECA in combination with activated NK cells may be the fist step of vascular damage associated with pulmonary hypertension in patients with MCTD.

Objectives: We reviewed the results of arthroplasty of the great toe with hallux valgus in rheumatoid patients, using a Swanson flexible hinge toe implant protected by grommet.

Materials and methods: Sixteen patients (26 feet) with rheumatoid arthritis were operated on from 1996 to 1999. (fifteen women, one man). The average age was 59.8 years (range, 48-73 years). The average follow-up period was 3.0 years (range, 1.0-4.5 years). In each patient, the hallux valgus angle (HVA) was measured before and after surgery, and implant breakage and radiolucency around the implant were evaluated on radiographs.

Results: Average HVA was 47.7 degrees preoperatively and 19.3 degrees postoperatively. No implant breakage was observed in 76.9% of the feet. Radiolucency of more than 2 mm was observed in only 3.8% of the feet.

Conclusions: These findings suggest that arthroplasty using a Swanson flexible hinge toe implant with a grommet is useful for treatment of hallux valgus in rheumatoid patients.