ArticleRMD open2023
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.
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Article in RMD open, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 13 papers, 1 of them a synthesis that pooled it.
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13citing papers in PubMed, 1 pooled it
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13 citing papers in PubMed, 1 synthesis or guideline pooled it, 19 citations in OpenAlex.
- 2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis.Advances in rheumatology (London, England) · 2024Guideline
- The clinical profile of systemic sclerosis without a 'scleroderma pattern' at nailfold capillaroscopy: results from the multicenter SPRING registry of the Italian Society of Rheumatology.Journal of translational autoimmunity · 2026Article
- Mycophenolate Mofetil Treatment Reduces the Risk of Treatment Escalation Due to Vascular Complications in Limited Cutaneous Systemic Sclerosis: Emulation of a Target Trial From the Italian Rheumatology Society SPRING Registry.Arthritis care & research · 2026Article
- Article
- Gastrointestinal involvement in very early and established systemic sclerosis: insights from the SPRING-SIR national Italian registry.Rheumatology (Oxford, England) · 2026Article
- Age and onset timing of Raynaud's phenomenon and first non-Raynaud symptom as prognostic factors in systemic sclerosis: a retrospective analysis from the Italian national multicenter Systemic Sclerosis Progression INvestiGation registry of the Italian Society for Rheumatology (SPRING-SIR).Therapeutic advances in musculoskeletal disease · 2026Article
- Seronegative Rheumatoid Arthritis: A Distinct Immunopathological Entity with Erosive Potential.Medical sciences (Basel, Switzerland) · 2025Review
- Article
- Rapidly Progressing Secondary Organizing Pneumonia in a Case of Systemic Sclerosis Sine Scleroderma - A Diagnostic Urgency: A Case Report and Literature Review.Journal of community hospital internal medicine perspectives · 2025Article
- Virus-Induced MicroRNA Modulation and Systemic Sclerosis Disease.Biomedicines · 2024Review
- Practice pattern for the use of intravenous iloprost for the treatment of peripheral vasculopathy in systemic sclerosis: A case-control study from the Italian national multicenter "SPRING" (Systemic Sclerosis Progression InvestiGation) Registry.Journal of scleroderma and related disorders · 2024Article
- Systemic sclerosis sine scleroderma with atypical clinical course: a rare case report.Annals of medicine and surgery (2012) · 2023Article
- Treatable Traits in Systemic Sclerosis.Clinical reviews in allergy & immunology · 2023Review
Corrections and comments
- Erratum issued
Authors and funding
73 authors at 20 institutions in 1 country.
Rossella De AngelisRheumatology Unit - Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy r.deangelis@staff.univpm.it.ORCID 0000-0001-5169-3511
Clodoveo FerriRheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy.
Dilia GiuggioliRheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy.
Gianluigi BajocchiRheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, Reggio Emilia, Italy.
Lorenzo DagnaUnit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, Milano, Italy.
Silvia Bellando-RandoneDepartment of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy.
Giovanni ZanframundoDepartment of Rheumatology, Policlinico San Matteo, Pavia, Italy, Pavia, Italy.ORCID 0000-0001-5042-1282
Rosario FotiRheumatology Unit, A.O.U Policlinico S. Marco, Catania, Italy, Catania, Italy.
Fabio CacciapagliaRheumatology Unit, Department of Emergency and Organ Transplantations, University of Bari, Bari, Italy.ORCID 0000-0001-7479-4462
Giovanna CuomoDepartment of Precision Medicine - University of Campania "Luigi Vanvitelli", Naples, Italy, Caserta, Italy.
Alarico ArianiDepartment of Medicine, Internal Medicine and Rheumatology, Azienda Ospedaliero Universitaria di Parma, Parma, Italy, Parma, Italy.ORCID 0000-0003-1428-6102
Edoardo RosatoDepartment of Translational and Precision Medicine, Sapienza University of Rome, Italy, Roma, Italy.ORCID 0000-0002-7417-8093
Gemma LepriDepartment of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy.ORCID 0000-0003-4141-6937
Francesco GirelliDepartment of Medicine, Rheumatology Unit, Ospedale GB Morgagni - L Pierantoni, Forlì, Italy, Forlì, Italy.
Valeria RiccieriDepartment of Rheumatology, Sapienza University of Rome, Rome, Italy, Rome, Italy.
Elisabetta ZanattaDepartment of Rheumatology, University of Padua, Padua, Italy.
Silvia Laura BoselloInstitute of Rheumatology and Affine Sciences, Division of Rheumatology, Catholic University of the Sacred Heart, Rome, Italy, Rome, Italy.
Ilaria CavazzanaRheumatology and Clinical Immunology, ASST Spedali Civili of Brescia; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy, Brescia, Italy.
Francesca IngegnoliDivision of Clinical Rheumatology, ASST Pini, Dept. of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan, Italy, Milano, Italy.
Maria De SantisRheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, and Humanitas University, Pieve Emanuele, Milan, Italy, Rozzano, Italy.ORCID 0000-0002-3196-1336
Giuseppe MurdacaResearch Hospital San Martino, University of Genoa, Genoa, Italy, Genova, Italy.
Giuseppina AbignanoRheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy, Potenza, Italy.
Nicoletta RomeoAzienda Ospedaliera S Croce e Carle, Cuneo, Italy.
Alessandra Della RossaDepartment of Rheumatology, University of Pisa, Pisa, Italy, Italy.
Maurizio CaminitiDepartmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy, Italy.
Anna Maria IulianoRheumatology Unit, San Camillo - Forlanini Hospital, Rome, Italy, Italy.
Giovanni CianoHospital of Ariano Irpino, Local Health Department, Ariano Irpino, Avellino, Italy.
Lorenzo BerettaReferral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy.ORCID 0000-0002-6529-6258
Gianluca BagnatoDepartment of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
Ennio LubranoDepartment of Rheumatology, University of Molise, Campobasso, Italy.ORCID 0000-0003-1471-6467
Ilenia De AndresRheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione "Garibaldi", Catania, Italy.
Alessandro GiolloRheumatology Section, Department of Medicine, University of Verona, Verona, Italy.
Marta SaraccoRheumatology Unit, Mauriziano-Umberto I Hospital, Torino, Italy.
Cecilia AgnesSan Lorenzo Hospital, Carmagnola, Turin, Italy.
Edoardo CipollettaRheumatology Unit - Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy.
Federica LumettiRheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy.
Amelia SpinellaRheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy.
Luca MagnaniRheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, Reggio Emilia, Italy.
Corrado CampochiaroUnit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, Milano, Italy.
Giacomo De LucaUnit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, Milano, Italy.ORCID 0000-0002-5306-7714
Veronica CodulloDepartment of Rheumatology, Policlinico San Matteo, Pavia, Italy, Pavia, Italy.ORCID 0000-0003-2557-8514
Elisa VisalliRheumatology Unit, A.O.U Policlinico S. Marco, Catania, Italy, Catania, Italy.
Claudio Di VicoDepartment of Precision Medicine - University of Campania "Luigi Vanvitelli", Naples, Italy, Caserta, Italy.
Antonietta GiganteDepartment of Translational and Precision Medicine, Sapienza University of Rome, Italy, Roma, Italy.
Greta PellagrinoDepartment of Rheumatology, Sapienza University of Rome, Rome, Italy, Rome, Italy.
Erika PigattoDepartment of Medicine, Villa Salus Hospital, Venice, Italy.
Maria-Grazia LazzaroniRheumatology and Clinical Immunology, ASST Spedali Civili of Brescia; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy, Brescia, Italy.
Franco FranceschiniRheumatology and Clinical Immunology, ASST Spedali Civili of Brescia; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy, Brescia, Italy.
Elena GeneraliRheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, and Humanitas University, Pieve Emanuele, Milan, Italy, Rozzano, Italy.
Gianna MennilloRheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy, Potenza, Italy.
Simone BarsottiDepartment of Rheumatology, University of Pisa, Pisa, Italy, Italy.
Giuseppa Pagano MarianoDepartmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy, Italy.
Federica FuriniRheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy.
Licia VultaggioRheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy.
Simone ParisiRheumatology Unit, Città della Salute e della Scienza, Turin, Italy.ORCID 0000-0003-4496-8315
Clara Lisa PeroniRheumatology Unit, Città della Salute e della Scienza, Turin, Italy.
Davide RozzaEpidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
Anna ZanettiEpidemiology Unit, Italian Society for Rheumatology, Milan, Italy.ORCID 0000-0001-8408-451X
Greta CarraraEpidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
Gianpiero LandolfiEpidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
Carlo Alberto ScirèRheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy.ORCID 0000-0001-7451-0271
Gerolamo BianchiRheumatology Unit, Department of Musculoskeletal Sciences, Local Health Trust 3, La Colletta Hospital, Genoa, Italy.
Enrico FusaroRheumatology Unit, Città della Salute e della Scienza, Turin, Italy.
Gian Domenico SebastianiRheumatology Unit, San Camillo - Forlanini Hospital, Rome, Italy, Italy.
Marcello GovoniRheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy.
Salvatore D'AngeloRheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy, Potenza, Italy.ORCID 0000-0002-7442-1110
Franco CozziDepartment of Medicine, Villa Salus Hospital, Venice, Italy.ORCID 0000-0003-3627-3927
Serena GuiducciDepartment of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy.
Andrea DoriaDepartment of Rheumatology, University of Padua, Padua, Italy.ORCID 0000-0003-0548-4983
Carlo SalvaraniRheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy.
Florenzo IannoneRheumatology Unit, Department of Emergency and Organ Transplantations, University of Bari, Bari, Italy.
Marco Matucci-CerinicUnit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, Milano, Italy.ORCID 0000-0002-9324-3161
SPRING-SIR (Systemic Sclerosis PRogression INvestiGation group of the Italian Society of Rheumatology)
University of Modena and Reggio Emilia · ITSapienza University of Rome · ITSocietà Italiana di Reumatologia · ITUniversity of Ferrara · ITVita-Salute San Raffaele University · ITAzienda Ospedaliera Citta' della Salute e della Scienza di Torino · ITAzienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia · ITOspedale San Carlo · ITUniversity of Florence · ITAzienda Sanitaria Unità Locale di Reggio Emilia · ITCarlo Forlanini Hospital · ITHumanitas University · ITMarche Polytechnic University · ITPoliclinico San Matteo Fondazione · ITPoliclinico Universitario di Catania · ITUniversity of Bari Aldo Moro · ITUniversity of Campania "Luigi Vanvitelli" · ITUniversity of Padua · ITUniversity of Pisa · ITAzienda di Rilievo Nazionale ed Alta Specializzazione · IT
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.
methodsData involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.
resultsAmong patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001).
conclusionThe 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.
Indexed as
Autoimmune DiseasesRheumatologyScleroderma, SystemicCarbon RadioisotopesHumansSeasonsSkin UlcerCarbon-19Carbon RadioisotopesAutoimmune DiseasesAutoimmunityEpidemiologyScleroderma, Systemic
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