Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology.
Rossella De AngelisClodoveo FerriDilia GiuggioliGianluigi BajocchiLorenzo DagnaSilvia Bellando-RandoneGiovanni ZanframundoRosario FotiFabio CacciapagliaGiovanna CuomoAlarico ArianiEdoardo RosatoGemma LepriFrancesco GirelliValeria RiccieriElisabetta ZanattaSilvia Laura BoselloIlaria CavazzanaFrancesca IngegnoliMaria De SantisGiuseppe MurdacaGiuseppina AbignanoNicoletta RomeoAlessandra Della RossaMaurizio CaminitiAnna Maria IulianoGiovanni CianoLorenzo BerettaGianluca BagnatoEnnio Giulio FavalliIlenia De AndresAlessandro GiolloMarta SaraccoCecilia AgnesEdoardo CipollettaFederica LumettiAmelia SpinellaLuca MagnaniCorrado CampochiaroGiacomo DE LucaVeronica CodulloElisa VisalliClaudio Di VicoAntonietta GiganteGreta PellagrinoErika PigattoMaria-Grazia LazzaroniFranco FranceschiniElena GeneraliGianna MennilloSimone BarsottiGiuseppa Pagano MarianoFederica FuriniLicia VultaggioSimone ParisiClara Lisa PeroniDavide RozzaAnna ZanettiGreta CarraraGianpiero LandolfiAnd Carlo Alberto ScirèGerolamo BianchiEnrico FusaroGian Domenico SebastianiMarcello GovoniSalvatore D'AngeloFranco CozziSerena GuiducciAndrea DoriaCarlo SalvaraniFlorenzo IannoneMarco Matucci Cerinicnull nullPublished in: RMD open (2023)
The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.