Background Systemic sclerosis (SSc) is one of the most complex and heterogeneous systemic disease. The diagnosis can be helped by the positivity of different characteristic auto-antibodies steered toward topoisomerase I, centromeric protein, RNA polymerase and fibrillarin. Different presentations of the disease can be associated with some specificities of auto-antibodies. Recently, some studies have reported the presence of anti-citrullinated protein antibodies (ACPA) in patients with SSc.
Objectives We have conducted a systematic literature review in order to define the prevalence of ACPA in SSc, and to observe their influence on clinical and paraclinical presentation of the disease.
Methods Literature search was performed in PubMed Medline database on 15 february 2015. Publication were identified through a search that used the terms: (“systemic sclerosis”(MeSH) and “ACPA or anti-CCP or rheumatoid factor or cohort or value diagnostic”). In a first step, we have selected all cohorts over 50 patients with SSc and ACPA identification. In a second step we have included all studies in which the clinical profile of scleroderma was detailed according to their ACPA status. Meta-analyses were performed when there were two or more studies with similar clinical description.
Results For the first question, we identified 13 observational studies merging 1231 patients with SSc. ACPAs were positive for 113 patients, giving a prevalence of ACPA in SSc of 9.2%. In a second step, we identified 9 studies reporting clinical and paraclinical aspects according to their ACPA status. Information was enough to perform a meta-analysis for arthralgia, arthritis, joint erosions, skin sclerosis, pulmonary arterial hypertension, pulmonary fibrosis and gastrooesophageal reflux. We found a significant association between ACPA positivity and the presence of arthritis (OR: 25.48 [11.82–54.47]), joint erosions on X-rays (OR: 13.06 [4.68–36.43]), pulmonary arterial hypertension (OR: 7.72 [2.38 – 25.5]), and pulmonary fibrosis (OR: 2.75 [1,21–6.24]) (Fig 1).
Conclusions ACPA prevalence in scleroderma is 9.2%. Our systematic literature review shows a significant increased risk for erosive arthritis, pulmonary hypertension and pulmonary fibrosis in ACPA positive SSc. ACPA should be systematically included in the check-up of systemic sclerosis.
Disclosure of Interest None declared