Systemic sclerosis sine scleroderma: distinct features in a large Brazilian cohort

Marangoni, Roberta Gonçalves; Rocha, Luiza F.; Rio, Ana Paula Toledo Del; Nh, Yoshinari; Marques-Neto, João Francisco; Sampaio-Barros, Percival D.

doi:10.1093/rheumatology/ket163

Cited by 36 publications

(49 citation statements)

References 14 publications

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“…9 The large group of ssSSc patients reported by Marangoni et al confirmed that esophageal involvement was the most frequent visceral manifestation (83%) and was followed by pulmonary involvement (68%). 8 Similar findings were described in the German and Spanish groups of ssSSc patients. 8,10 Esophageal dysmotility was shown in 73% and 45% of the patients, while interstitial lung disease was found in 73% and 64%, respectively.…”

Section: Clinical Picturesupporting

confidence: 78%

“…8 Similar findings were described in the German and Spanish groups of ssSSc patients. 8,10 Esophageal dysmotility was shown in 73% and 45% of the patients, while interstitial lung disease was found in 73% and 64%, respectively. Gastrointestinal manifestations include swallowing disturbances, crampy abdominal pain, nausea or vomiting.…”

Section: Clinical Picturesupporting

confidence: 78%

“…Marangoni et al retrospectively analyzed 947 consecutive patients with systemic sclerosis treated in two academic medical centers in Brazil. 8 They were able to identify a very high rate of the ssSSc patients, i.e. 79 cases (8.3%).…”

Section: Prevalencementioning

confidence: 97%

“…1.5 and 7.5%, respectively. 8,10 Most of the literature is limited to descriptions of isolated cases, usually containing additional rare features, complications or associated conditions. According to Marangoni et al, the total number of 139 cases was reported until 2012.…”

Section: Prevalencementioning

confidence: 99%

“…According to Marangoni et al, the total number of 139 cases was reported until 2012. 8 In 2014, Diab et al analyzed data from 1,417 subjects with systemic sclerosis in the Canadian Scleroderma Research Group registry. Twenty-seven subjects (1.9%) were considered as ssSSc with a mean follow-up of 2.4 years.…”

Section: Prevalencementioning

confidence: 99%

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Systemic sclerosis sine scleroderma

Kucharz¹,

Kopeć-Mędrek²

2017

Adv Clin Exp Med

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Systemic sclerosis is a rare generalized disease with scleroderma, i.e. skin thickening as one of the most common symptoms. The disease has 2 main subsets, diffuse and limited forms. The subset known as systemic sclerosis sine scleroderma (ssSSc) is a very rare subset characterized by the total or partial absence of cutaneous manifestations of systemic sclerosis with the occurrence of internal organ involvement and serologic abnormalities. The classification of ssSSc into 3 groups was proposed. Type I (complete) is characterized by the lack of any cutaneous changes typical for the disease at least until systemic sclerosisrelated insufficiency of any internal organ occurs. Type II (incomplete) is characterized by the absence of sclerodactyly, but other cutaneous involvements (e.g. calcifications, telangiectasies, pitting scars) can be found. Type III (delayed) is characterized by clinical internal organ involvement typical for systemic sclerosis that has appeared before skin changes (complete or incomplete). In general, the demographic and clinical characteristics of the ssSSc patients suggest that they are similar to those with diffuse or limited form of the disease. Diagnosis of ssSSc still remains difficult and this disease form should be considered in all cases of unexplained fibrotic involvement of the internal organs.

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Section: Clinical Picturesupporting

confidence: 78%

Section: Clinical Picturesupporting

confidence: 78%

Section: Prevalencementioning

confidence: 97%

Section: Prevalencementioning

confidence: 99%

Section: Prevalencementioning

confidence: 99%

See 3 more Smart Citations

Systemic sclerosis sine scleroderma

Kucharz¹,

Kopeć-Mędrek²

2017

Adv Clin Exp Med

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Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma

et al. 2023

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ImportanceSystemic sclerosis (SSc) sine scleroderma (ssSSc) is a subset of SSc defined by the absence of skin fibrosis. Little is known about the natural history and skin manifestations among patients with ssSSc.ObjectiveTo characterize the clinical phenotype of patients with ssSSc compared with patients with limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) within the EUSTAR database.Design, Setting, and ParticipantsThis longitudinal observational cohort study based on the international EUSTAR database included all patients fulfilling the classification criteria for SSc assessed by the modified Rodnan Skin score (mRSS) at inclusion and with at least 1 follow-up visit; ssSSc was defined by the absence of skin fibrosis (mRSS = 0 and no sclerodactyly) at all available visits. Data extraction was performed in November 2020, and data analysis was performed from April 2021 to April 2023.Main Outcomes and MeasuresMain outcomes were survival and skin manifestations (onset of skin fibrosis, digital ulcers, telangiectasias, puffy fingers).ResultsAmong the 4263 patients fulfilling the inclusion criteria, 376 (8.8%) were classified as having ssSSc (mean [SD] age, 55.3 [13.9] years; 345 [91.8%] were female). At last available visit, in comparison with 708 patients with lcSSc and 708 patients with dcSSc with the same disease duration, patients with ssSSc had a lower prevalence of previous or current digital ulcers (28.2% vs 53.1% in lcSSc; P &lt; .001; and 68.3% in dcSSc; P &lt; .001) and puffy fingers (63.8% vs 82.4% in lcSSc; P &lt; .001; and 87.6% in dcSSc; P &lt; .001). By contrast, the prevalence of interstitial lung disease was similar in ssSSc and lcSSc (49.8% and 57.1%; P = .03) but significantly higher in dcSSc (75.0%; P &lt; .001). Skin telangiectasias were associated with diastolic dysfunction in patients with ssSSc (odds ratio, 4.778; 95% CI, 2.060-11.081; P &lt; .001). The only independent factor for the onset of skin fibrosis in ssSSc was the positivity for anti–Scl-70 antibodies (odds ratio, 3.078; 95% CI, 1.227-7.725; P = .02). Survival rate was higher in patients with ssSSc (92.4%) compared with lcSSc (69.4%; P = .06) and dcSSc (55.5%; P &lt; .001) after up to 15 years of follow-up.Conclusions and RelevanceSystemic sclerosis sine scleroderma should not be neglected considering the high prevalence of interstitial lung disease (&gt;40%) and SSc renal crisis (almost 3%). Patients with ssSSc had a higher survival than other subsets. Dermatologists should be aware that cutaneous findings in this subgroup may be associated with internal organ dysfunction. In particular, skin telangiectasias in ssSSc were associated with diastolic heart dysfunction.

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