This case report describes a 65-year-old male with established systemic sclerosis (SSc) presenting with primary cardiac involvement (SSc-pHI). Multimodal assessment revealed elevated cardiac biomarkers, frequent arrhythmias, diastolic dysfunction, and myocardial fibrosis on cardiac magnetic resonance (CMR), despite asymptomatic status. This underscores the critical role of proactive screening in high-risk SSc patients[1-3].

Introduction

Systemic sclerosis is characterized by vasculopathy, fibrosis, and autoimmunity. Cardiac involvement (SSc-pHI) affects 15–35% of patients and accounts for 30% of SSc-related deaths[4-5]. Pathogenesis involves microvascular dysfunction ("cardiac Raynaud's"), inflammation, and fibrosis, often presenting subclinically[6]. We present a case highlighting diagnostic strategies for SSc-pHI.

Case Presentation

A 65-year-old male with >10-year diffuse cutaneous SSc (dcSSc)[7] presented with worsening skin sclerosis. Cardiac workup revealed:

Biomarkers:      Elevated CK (1134.6 IU/L), CK-MB (76.4 IU/L), BNP (194 pg/ml); normal      Troponin-I (0.64 ng/ml)[8]

ECG/Holter: Sinus      rhythm with left ventricular high voltage; frequent atrial premature      complexes (1,776/24h)[9]

Echocardiography:      Diastolic dysfunction, mild pulmonary hypertension (PASP 43 mmHg)[10]

CMR: Focal fibrosis      in mid-inferoseptal/anterior-septal LV segments (LGE-positive)[11]

Assessment and Diagnosis

SSc-pHI diagnosis followed structured risk stratification (UKSSG criteria: male, >65 years, dcSSc, ILD)[12] and multimodal assessment:

Biomarkers:      Elevated BNP and CK-MB suggested myocardial stress[8]

Echocardiography:      Diastolic dysfunction—a stronger mortality predictor than PH in SSc[10]

Arrhythmias: High      atrial ectopy burden linked to myocardial fibrosis[9]

CMR: Gold standard      for detecting replacement fibrosis (LGE)[11,13]

Exclusion of      secondary causes: Non-coronary fibrosis pattern ruled out ischemic      cardiomyopathy[14]

Diagnosis: SSc-pHI with myocardial fibrosis, diastolic dysfunction, and arrhythmias.

Management

Combination therapy addressed SSc and cardiac complications:

Immunosuppression:      Prednisone 30 mg/day, cyclophosphamide, methotrexate (targeting myocardial      inflammation)[15]

Cardioprotection:      Benazepril (ACE inhibitor) for diastolic dysfunction[16]

PH/ILD: Ambrisentan      (discontinued after improvement), pirfenidone[17]

Discussion

This case illustrates key principles:

Subclinical      presentation: Significant cardiac pathology may exist without symptoms,      necessitating screening in high-risk patients[18]

CMR superiority:      Detects early fibrosis (LGE/T1 mapping) undetectable by      echocardiography[11,13]

Arrhythmia      significance: Atrial ectopy >500/24h correlates with fibrosis and      mortality[9]

Therapeutic      balance: Immunosuppression (e.g., cyclophosphamide[15]) and      cardioprotective agents (ACE inhibitors[16]) are complementary.

Conclusion

Proactive cardiac assessment using biomarkers, echocardiography, Holter, and CMR is essential for early SSc-pHI detection[10,13,19]. Multimodal strategies enable timely intervention to improve outcomes in this high-mortality complication[20].