PO51 - Fibrotic pulmonary sarcoidosis with persistent inflammatory activity: corticosteroid toxicity and barriers to treatment escalation
Pedro Marinho Manso (Portugal)1; Ana Castelo Grande (Portugal)1; Nuno de Barros Ferreira (Portugal)1; Ivone Gonçalves (Portugal)1;
1 - ULS do Tâmega e Sousa;
Keywords: Pulmonary sarcoidosis; Pulmonary fibrosis; Corticosteroid toxicity;
Select the theme: Controversies in Sarcoidosis Treatment
Type: Clinical Cases
Presentation: Poster Presentation

Introduction: Fibrotic pulmonary sarcoidosis is difficult to manage when irreversible damage coexists with reversible inflammatory activity and treatment escalation is constrained by toxicity and infectious risk. 

Clinical Case Description: A 39-year-old former smoker presented in 2018 with 6 months of progressive exertional dyspnea. Pulmonary function tests showed restriction (FVC 47%, TLC 56%) and severe diffusion impairment (DLCO 36%). High-resolution chest CT revealed upper-lobe-predominant fibrotic distortion with traction bronchiectasis, bilateral ground-glass opacities, and bulky calcified mediastinal/hilar adenopathy. Serum ACE was 298 U/L, and bronchoalveolar lavage showed lymphocytosis with an increased CD4/CD8 ratio (3,98), supporting stage IV pulmonary sarcoidosis. Prednisone improved symptoms and reduced inflammatory parenchymal abnormalities and adenopathy, but fibrosis persisted. After loss to follow-up, he remained on prolonged corticosteroids and developed toxicity, including cushingoid habitus, weight gain, and obstructive sleep apnea. Methotrexate was introduced in January 2023 as a steroid-sparing agent but stopped after hepatotoxicity in the setting of hepatic steatosis and alcohol-related cholestatic liver dysfunction. In April 2023, he was hospitalized with COVID-19 pneumonia and hypoxemic respiratory failure; because radiologic and functional worsening persisted after infection, corticosteroids were reintroduced with improvement, suggesting ongoing sarcoid activity. Despite azathioprine escalation, lung function remained impaired (FVC 47–50%, DLCO 44–47%), with severe exertional desaturation on 6-minute walk testing (91% to 79%). Follow-up CT showed increasing ground-glass abnormalities superimposed on established fibrosis, PET-CT demonstrated hypermetabolic granulomatous disease involving nodal, pulmonary, and subcutaneous sites, and echocardiography suggested pulmonary hypertension. Infliximab was planned, but latent tuberculosis screening was positive, prompting isoniazid before biologic therapy; he was also referred for lung transplantation assessment. 

Conclusions: This case highlights the therapeutic dilemma of advanced pulmonary sarcoidosis: distinguishing fixed fibrosis from treatable inflammation while minimizing cumulative corticosteroid harm. PET-guided reassessment, steroid-sparing strategies, management of infectious risk, and timely referral for biologic therapy and lung transplantation are crucial in refractory disease.