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A case report of clubbing as the initial sign of systemic sclerosis sine scleroderma: A rare possible presentation.

ABSTRACT:

Key clinical message

Even in the absence of characteristic cutaneous symptoms of scleroderma, systemic sclerosis should be considered in the differential diagnosis of patients initially diagnosed with idiopathic interstitial lung disease.

Abstract

Systemic sclerosis (SSc) is an idiopathic connective tissue disorder characterized by multisystem involvement. Although skin thickening is a hallmark manifestation of SSc, a subset known as systemic sclerosis sine scleroderma (ssSSc) presents with internal organ involvement and positive serologic markers in the absence of significant cutaneous manifestations. We report the case of a 36-year-old Iranian woman who presented with clubbing as an initial symptom of ssSSc. Notably, clubbing as the sole initial sign of the disease has not been previously reported. Timely diagnosis of ssSSc is crucial to facilitate appropriate treatment and prevent disease progression. Physicians should adopt a comprehensive approach when evaluating patients presenting with limited clinical features, as they might be indicative of underlying ssSSc.

SUBMITTER: Amirpour A

PROVIDER: S-EPMC10587653 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature

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A case report of clubbing as the initial sign of systemic sclerosis sine scleroderma: A rare possible presentation.

Amirpour Anahita A Ghorbani Parastoo P Ghadirzadeh Erfan E Charkazi Elham E Paraandavaji Elham E

Clinical case reports 20231019 10

<h4>Key clinical message</h4>Even in the absence of characteristic cutaneous symptoms of scleroderma, systemic sclerosis should be considered in the differential diagnosis of patients initially diagnosed with idiopathic interstitial lung disease.<h4>Abstract</h4>Systemic sclerosis (SSc) is an idiopathic connective tissue disorder characterized by multisystem involvement. Although skin thickening is a hallmark manifestation of SSc, a subset known as systemic sclerosis sine scleroderma (ssSSc) pre ...[more]

PMID: 37867549

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Project description: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 < .001; and 68.3% in dcSSc; P < .001) and puffy fingers (63.8% vs 82.4% in lcSSc; P < .001; and 87.6% in dcSSc; P < .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 < .001). Skin telangiectasias were associated with diastolic dysfunction in patients with ssSSc (odds ratio, 4.778; 95% CI, 2.060-11.081; P < .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 < .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 (>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.

Dataset Information

A case report of clubbing as the initial sign of systemic sclerosis sine scleroderma: A rare possible presentation.

Key clinical message

Abstract

Publications

A case report of clubbing as the initial sign of systemic sclerosis sine scleroderma: A rare possible presentation.

Similar Datasets

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