Hospital-based first diagnoses of systemic sclerosis: Defining initial clinical presentations and diagnostic pathways.

BACKGROUND AND

OBJECTIVE

Early diagnosis of systemic sclerosis (SSc) is challenging due to heterogeneous presentation and lack of reliable biomarkers, resulting in many patients being diagnosed during hospitalization with advanced disease and severe complications. We aimed to characterize the clinical presentation, diagnostic pathway, and outcomes of patients first diagnosed with SSc during hospitalization.

METHODS

We retrospectively reviewed adult patients newly diagnosed with SSc during hospitalization (January 2016-July 2023), including those referred for SSc evaluation or with SSc features identified during admission for other diagnoses. Patients previously diagnosed as outpatients were excluded. Clinical presentations were compared between patients admitted for SSc-related reasons vs. those admitted for other reasons subsequently diagnosed with SSc.

RESULTS

Thirty-five patients were included (mean age 60.4 ± 12.8 years; 60% women). Most (68.6%) were incidentally diagnosed with SSc during hospitalization for unrelated conditions. Common indications for hospitalization included pericardial effusion (22.9%), progressive pulmonary hypertension (17.1%), and progressive interstitial lung disease (14.3%). Approximately 60% of both hospitalized patients in both groups initially saw general practitioners before were rheumatological referral. Age, sex, symptom duration, most clinical features, length of stay, hospital costs, and outcomes were similar between patients admitted for SSC-related vs. non-SSc-related reasons, except for greater gastrointestinal involvement in the latter group (P = 0.02).

CONCLUSION

Hospitalized patients first diagnosed with SSc predominantly present with diffuse cutaneous involvement and advanced cardiopulmonary complications. Most cases are identified during hospitalization for unrelated conditions, highlighting the need for increased clinical vigilance and systematic SSc evaluation in at-risk hospitalized patients to enable early detection and improve outcomes.