Registry of the Spanish Network for Systemic Sclerosis: Clinical Pattern According to Cutaneous Subsets and Immunological Status

Simeón-Aznar, Carmen Pilar; Fonollosa-Plà, Vicent; Tolosa-Vilella, Carles; Espinosa, Gerard; Ramos-Casals, Manuel; Campillo, Mercedes; García–Hernández, Francisco J.; Castillo-Palma, María Jesús; Sánchez-Román, Julio; Callejas‐Rubio, José Luis; Ortego-Centeno, Norbérto; Egurbide-Arberas, María Victoria; Trapiellla-Martínez, Luis; Gallego‐Villalobos, María; Sáez‐Comet, Luis; Velilla-Marco, José; Camps-García, M.T.; Ramón-Garrido, E. de; Marcos, Eva María Esteban; Pallarés-Ferreres, L.; Hidalgo-Tenorio, Carmen; Sabio-Sánchez, J M; Torre, Ricardo Gómez-de la; Salvador-Cervelló, Gonzalo; Ríos-Blanco, Juan José; Gil‐Aguado, Antonio; Vilardell‐Tarrés, Miquel

doi:10.1016/j.semarthrit.2011.10.004

Cited by 95 publications

(102 citation statements)

References 36 publications

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“…Patients with dcSSc typically develop the ILD early in the course of their disease. In the Registry of the Spanish Network for Systemic Sclerosis which included 916 patients with SSc, ILD was found in 70% of patients with dcSSc compared to patients with lcSSc or ssSSc in whom ILD was present approximately 39% of the time [17]. In this study, lung function was further characterized by SSc subgroup.…”

Section: Subsets Of Scleroderma Associated With Ildmentioning

confidence: 62%

“…The association between SSC and ILD is strongest in patients who suffer from dcSSc although there is a well described association with lcSSC and ssSSc [17,18]. Patients with dcSSc typically develop the ILD early in the course of their disease.…”

Section: Subsets Of Scleroderma Associated With Ildmentioning

confidence: 99%

“…Specific autoantibodies such as the anti-SCL-70, RNP, anti U11/ U12 RNP, anti Th/To and antihistone antibodies have been reported to be associated with an increased risk of ILD in SSc [19] and others such as anticentromere antibodies are protective [17,20]. However, because the antibody associations are neither specific nor sensitive [18], the efficacy of serologies as a clinical predictor of ILD is limited.…”

Section: Subsets Of Scleroderma Associated With Ildmentioning

confidence: 99%

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Interstitial Lung Disease in Systemic Sclerosis: Diagnosis and Management

Schol

Carr²,

Frech³

et al. 2012

Rheumatology

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Section: Subsets Of Scleroderma Associated With Ildmentioning

confidence: 62%

Section: Subsets Of Scleroderma Associated With Ildmentioning

confidence: 99%

Section: Subsets Of Scleroderma Associated With Ildmentioning

confidence: 99%

See 1 more Smart Citation

Interstitial Lung Disease in Systemic Sclerosis: Diagnosis and Management

Schol

Carr²,

Frech³

et al. 2012

Rheumatology

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“…Currently accepted disease subtypes include scleroderma with morphea (localized scleroderma), limited cutaneous systemic sclerosis (lSSc), diffuse cutaneous systemic sclerosis (dcSSc), and systemic sclerosis sine scleroderma (ssSSc). The classification of SSc into ISSc, dcSSc, and ssSSc subsets is the one that most closely reflects the natural history of the disease, as each of these subtypes reflect clear clinical differences [1].…”

Section: Introductionmentioning

confidence: 99%

Incidence and prevalence of Systemic Sclerosis (SSc) in Poland – differences between rural and urban regions

Kanecki

Goryński

Tarka

et al. 2017

Ann Agric Environ Med.

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Kanecki K, Goryński P,Tarka P, WierzbaW, Tyszko P. Incidence and prevalence of Systemic Sclerosis (SSc) in Poland -differences between rural and urban regions. Ann Agric Environ Med. 2017; 24(2): 240-244. doi: 10.5604/12321966.1233570 Abstract Introduction. Systemic sclerosis (SSc) is a rare and potentially severe connective tissue disease, characterized by skin fibrosis and involvement of internal organs. Because of its rarity and heterogeneous clinical presentation, reliable epidemiological studies on SSc have been particularly difficult to carry out. Objectives. The purpose of this study was to present SSc epidemiology among hospitalized patients in Poland. Materials and method. The analysis was based on population-based administrative data, taken from a Polish hospital morbidity study carried out by the National Institute of Public Health between January 2008 -December 2012. Results. Analyzed data covered 9,049 hospitalization records. The final sample comprised 3,653 patients with first-time hospitalizations for SSc. The average age of the sample was 53 years (SD 16.2; range 0-91 years); 84% of patients were female and 16% male. Based on hospitalization registers, the average SSc incidence was estimated to be 1.9/100,000 per year and peak age of incidence was 55 years. The point prevalence was estimated to be 9.4/100,000 at the end of 2012. SSc was more common in females, with F:M ratio ranging from 6.2:1-4.6:1 depending on the year. Analysis of hospitalization trends revealed that overall numbers of SSc hospitalizations increased, while first-time hospitalizations decreased between 2008-2012. Clusters of higher incidence were observed in more rural regions vs. urban regions. Conclusion. Estimated incidence of SSc in Poland was comparable to reported incidence in other European countries. Analysis of demographic factors and reports of clusters of higher incidence may suggest the existence of environmental risk factors for the development of SSc. These observations may require further research.

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“…Besides skin thickening, there were no significant differences in terms of internal organ involvement, laboratory values, serum autoantibodies or survival rate compared to lcSSc (5). Meanwhile, a Brazilian cohort of 79 ssSSc patients and the Spanish registry with 69 ssSSc patients suggested that more cardiac involvement and less telangiectasia would differentiate it from lcSSc, but a recent multicenter study of 27 ssSSc patients suggested that it is probably a forme fruste of lcSSc, meaning that the absence of skin involvement may be ascribed to a misclassification due to early or subtle involvement (3,6,7). It has been proposed that the term scleroderma be abandoned on the grounds that it is incorrectly related to the diagnosis of SSc, and be potentially replaced by designations such as morphea (localized scleroderma), lcSSc (previously reported as CREST syndrome), dcSSc and ssSSc (1).…”

mentioning

confidence: 99%

Systemic sclerosis sine scleroderma: a case report of anterior uveitis

Borges¹,

Vilaça²,

Ferreira³

et al. 2015

Reumatismo

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Systemic sclerosis (SSc) sine scleroderma (ssSSc) is characterized by the absence of skin involvement, despite other manifestations of systemic sclerosis are present. It is not known whether sSSc represents a <em>forme</em> <em>fruste</em> of limited cutaneous SSc or a distinct entity, but the 2013 American College of Rheumatology/European League Against Rheumatism criteria for the classification of SSc have considered SSc without skin involvement to be a distinct subset. The authors present the case of a 70-year old female that was referred for a consultation for Raynaud’s phenomenon and a chronic anterior uveitis (CAU). She had a history of dysphagia, diffuse pulmonary emphysema and a biopsy-documented fibrosis of the upper lobes, and an idiopathic non-ischemic dilated cardiomyopathy with severe left ventricle systolic dysfunction and left bundle branch block. Anti-nuclear and anti-centromere antibodies were positive, while manometry revealed distal esophageal hypomotility. After establishing the diagnosis of ssSSc and starting immunosuppression, the ocular disease improved, while the lung and heart diseases remained stable. This case underlines that it is very important to suspect SSc when CAU is present and/or skin thickening is absent. To our knowledge, this is the first report of CAU in a patient with ssSSc.

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Registry of the Spanish Network for Systemic Sclerosis: Clinical Pattern According to Cutaneous Subsets and Immunological Status

Cited by 95 publications

References 36 publications

Interstitial Lung Disease in Systemic Sclerosis: Diagnosis and Management

Interstitial Lung Disease in Systemic Sclerosis: Diagnosis and Management

Incidence and prevalence of Systemic Sclerosis (SSc) in Poland – differences between rural and urban regions

Systemic sclerosis sine scleroderma: a case report of anterior uveitis

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