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131.
Kavish J Bhansing Martin Lammens Hanneke KA Knaapen Piet LCM van Riel Baziel GM van Engelen Madelon C Vonk 《Arthritis research & therapy》2014,16(3):R111
Introduction
The objective was to characterize the clinical and myopathologic features of patients with scleroderma-polymyositis (SSc-PM) overlap compared with a population of patients with systemic sclerosis (SSc) and polymyositis (PM).Methods
A three-way comparison of patients with SSc-PM overlap (n = 25) with patients with SSc (n = 397) and PM (n = 40) on clinical and myopathologic features and causes of death. One neuropathologist blinded for the diagnosis evaluated all recent available muscle biopsies. Biopsies were scored for presence of inflammation, necrotic muscle fibers, rimmed vacuoles, fibrosis, and immunohistochemical staining. Clinical or myopathologic characteristics were compared by using the χ2 test or one-way analysis of variance (ANOVA).Results
The prevalence of SSc-PM overlap in the Nijmegen Systemic Sclerosis cohort was 5.9%. The mortality was 32% (eight of 25) in SSc-PM, of which half was related to cardiac diseases. The prevalence of pulmonary fibrosis was significantly increased in SSc-PM (83%) (P = 0.04) compared with SSc (49%) and PM (53%). SSc or myositis-specific antibodies were nearly absent in the SSc-PM group. In almost all biopsies (96%) of SSc-PM patients, necrotic muscle fibers were present, which was significantly increased compared with PM patients (P = 0.02).Conclusions
Patients with SSc-PM have increased prevalence of pulmonary fibrosis and cardiac disease as the cause of death compared with patients with SSc and PM . In addition, we found that necrotizing muscle fibers with inflammation characterize SSc-PM overlap in muscle biopsies. Further research should focus on underlying mechanisms causing necrosis, inflammation, and fibrosis and their relation to pulmonary involvement and mortality in patients with SSc-PM overlap. 相似文献132.
Esther Reefman Marcelus CJM de Jong Hilde Kuiper Marcel F Jonkman Pieter C Limburg Cees GM Kallenberg Marc Bijl 《Arthritis research & therapy》2007,8(6):R156
Apoptotic cells are thought to play an essential role in the pathogenesis of systemic lupus erythematosus (SLE). We hypothesise
that delayed or altered clearance of apoptotic cells after UV irradiation will lead to inflammation in the skin of SLE patients.
Fifteen SLE patients and 13 controls were irradiated with two minimal erythemal doses (MEDs) of ultraviolet B light (UVB).
Subsequently, skin biopsies were analysed (immuno)histologically, over 10 days, for numbers of apoptotic cells, T cells, macrophages,
and deposition of immunoglobulin and complement. Additionally, to compare results with cutaneous lesions of SLE patients,
20 biopsies of lupus erythematosus (LE) skin lesions were analysed morphologically for apoptotic cells and infiltrate. Clearance
rate of apoptotic cells after irradiation did not differ between patients and controls. Influx of macrophages in dermal and
epidermal layers was significantly increased in patients compared with controls. Five out of 15 patients developed a dermal
infiltrate that was associated with increased epidermal influx of T cells and macrophages but not with numbers of apoptotic
cells or epidermal deposition of immunoglobulins. Macrophages were ingesting multiple apoptotic bodies. Inflammatory lesions
in these patients were localised near accumulations of apoptotic keratinocytes similar as was seen in the majority of LE skin
lesions. In vivo clearance rate of apoptotic cells is comparable between SLE patients and controls. However, the presence of inflammatory
lesions in the vicinity of apoptotic cells, as observed both in UVB-induced and in LE skin lesions in SLE patients, suggests
that these lesions result from an inflammatory clearance of apoptotic cells. 相似文献
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