Progressive Systemic Sclerosis-Associated Interstitial Lung Disease With Clinical-Radiographic Dissociation and Delayed Transplant Access: A Case Report

TL;DR

A man with progressive lung disease worsened after COVID-19, showing a mismatch between symptoms and imaging, and faced delays in getting a lung transplant.

Contribution

Highlights clinical-radiographic dissociation in progressive ILD and systemic barriers to lung transplantation.

Findings

Patient's oxygen requirement increased significantly despite stable radiographic findings.

Escalation of immunosuppression did not halt clinical decline.

Insurance issues delayed lung transplant access, worsening prognosis.

Abstract

Systemic sclerosis-associated interstitial lung disease (ILD) is a major contributor to morbidity and mortality in patients with diffuse systemic sclerosis. We present the case of a 47-year-old man with fibrotic non-specific interstitial pneumonia who experienced worsening hypoxemia and dyspnea following COVID-19, despite ongoing treatment with mycophenolate mofetil and prednisone. His oxygen requirement increased from 6-8 L/minute to 15 L/minute at rest, even as imaging showed stable fibrosis with improvement in ground-glass opacities, highlighting a clinical-radiographic dissociation. Given concern for progressive fibrosing ILD, immunosuppression was escalated to intravenous cyclophosphamide. Referral for lung transplantation was delayed due to insurance non-acceptance at the region’s transplant center, compounding the urgency of his decline. This case underscores the importance of timely recognition of progressive ILD, the limitations of radiographic assessment alone, and the impact of systemic barriers on access to life-saving interventions.

Figures