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Systemic Sclerosis Sine Scleroderma.

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    Systemic sclerosis sine scleroderma, a rare variant of limited systemic sclerosis without skin symptoms, presents diagnostic challenges. Early identification is crucial for managing associated pulmonary artery hypertension and improving patient outcomes.

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    Area of Science:

    • Rheumatology
    • Internal Medicine
    • Cardiology

    Background:

    • Systemic sclerosis sine scleroderma (SScS) is a rare variant of limited systemic sclerosis characterized by the absence of skin involvement.
    • SScS shares clinical, laboratory, and prognostic features with classical systemic sclerosis, making diagnosis difficult without cutaneous manifestations.

    Observation:

    • A 60-year-old female presented with dyspnea on exertion and Raynaud's phenomenon.
    • Diagnostic investigations revealed SScS with coexisting pulmonary artery hypertension.

    Findings:

    • The case highlights the diagnostic delay in SScS due to the lack of skin symptoms.
    • Pulmonary artery hypertension was identified as a significant comorbidity in this patient with SScS.

    Implications:

    • Delayed diagnosis of SScS can lead to increased morbidity and mortality.
    • This case underscores the importance of considering SScS in patients with Raynaud's phenomenon and cardiopulmonary symptoms, even without skin findings.
    • Prompt diagnosis and management are essential for patients with SScS and pulmonary artery hypertension.