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2014 ; 16
(3
): R111
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Scleroderma-polymyositis overlap syndrome versus idiopathic polymyositis and
systemic sclerosis: a descriptive study on clinical features and myopathology
#MMPMID24886750
Bhansing KJ
; Lammens M
; Knaapen HK
; van Riel PL
; van Engelen BG
; Vonk MC
Arthritis Res Ther
2014[May]; 16
(3
): R111
PMID24886750
show ga
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 ?(2) 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.