Systemic sclerosis-rheumatoid arthritis overlap syndrome: a unique combination of features suggests a distinct genetic, serological and clinical entity

Abstract

To date this is the largest SSc-RA overlap cohort. Genetics, clinical and immunolaboratory features suggest a mixed phenotype. Our data suggest that SSc-RA overlap syndrome may be a distinct genetic, immunological and clinical entity.

A case of pulmonary fibrosis associated with rheumatoid arthritis, scleroderma sine scleroderma and ANCA associated vasculitis.

“…For example, ANCA positivity is associated with increased incidence of PF in RA patients (Cambridge et al 1994). Similarly, increased incidence of PF is seen in SSc patients with ANCA positivity (Derrett-Smith et al 2013) as well as in SSc-RA overlap patients (Szücs et al 2007). As expected, the prognosis of PF in overlap syndromes is worse than individual entities (Cambridge et al 1994;Derrett-Smith et al 2013;Szücs et al 2007).…”

“…Similarly, increased incidence of PF is seen in SSc patients with ANCA positivity (Derrett-Smith et al 2013) as well as in SSc-RA overlap patients (Szücs et al 2007). As expected, the prognosis of PF in overlap syndromes is worse than individual entities (Cambridge et al 1994;Derrett-Smith et al 2013;Szücs et al 2007). Also, ANCA positivity in PF can predispose to development of ANCA associated vasculitis (AAV) (Arulkumaran et al 2011).…”

“…The prognosis in these patients depends on several factors like histology, baseline lung function, presence of autoantibodies and its titer level (Morgenthau and Padilla 2009). This is worse in overlap syndromes (Cambridge et al 1994;Derrett-Smith et al 2013;Szücs et al 2007). …”

“…This susceptibility to lung fibrosis may be related to an inflammatory process or oxidative stress by ANCA in patients primed to develop fibrosis because of their SSc (Derrett-Smith et al 2013). Similarly, study by Szucus et al in SSc-RA overlap patients showed pulmonary fibrosis in 77% of patients which is much higher than individual diseases alone (Szücs et al 2007). …”

Das Deutsche Netzwerk für Systemische Sklerodermie (DNSS)

“…Mitteilung). CCP-Antikörper zeigen -wie bei der rheumatoiden Arthritis -auch bei der SSc ein deutlich erhöhtes Risiko für eine erosive Arthritis an [19].…”