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Semin Respir Crit Care Med 2025; 46(06): 564-575
DOI: 10.1055/a-2768-2520
Review Article

Advanced Pulmonary Sarcoidosis

Authors

  • Paolo Spagnolo

    1   Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Respiratory Disease Unit, University of Padova, Padova, Italy

  • Zehra Dhanani

    2   Department of Thoracic Medicine and Surgery, Temple University Hospital, Philadelphia, Pennsylvania, United States

  • Paolo Cameli

    3   Respiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy

  • Giordano Fiorentù

    1   Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Respiratory Disease Unit, University of Padova, Padova, Italy

  • Rohit Gupta

    4   Deaprtment of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
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Abstract

In sarcoidosis, pulmonary manifestations are almost universal; however, their severity ranges from asymptomatic to respiratory failure and death. Approximately 20% of patients progress to advanced pulmonary sarcoidosis (APS), a disease phenotype that is driven mostly by pulmonary fibrosis and associated complications, including bronchiectasis, chronic pulmonary aspergillosis, and pulmonary hypertension, which may result from multiple mechanisms. APS may be burnt out but is often active as confirmed by 18F-fluorodeoxyglucose-PET. APS is a major cause of morbidity and mortality in patients with sarcoidosis, but its natural history is largely unknown and evidence-based treatment guidelines are lacking. Here, we review the major patterns of APS with emphasis on clinical manifestations, pathophysiology, and management, although this is based on expert opinion and may include, based on the predominant disease phenotype, anti-inflammatory/immunosuppressive treatment, antifibrotics, pulmonary rehabilitation, antibiotics, vasodilators, and, in highly selected patients, lung transplantation.

Keywords

sarcoidosis - pulmonary fibrosis - pulmonary hypertension - advanced disease - bronchiectasis - pulmonary aspergilloma

Contributors's Statement

P.S.: conceptualization, methodology, resources, supervision, validation, writing - original draft, writing - review & editing. Z.D.: methodology, writing - original draft, writing - review & editing. P.C.: conceptualization, methodology, writing - original draft, writing - review & editing. G.F.: conceptualization, methodology, writing - original draft, writing - review & editing. R.G.: conceptualization, methodology, validation, writing - original draft, writing - review & editing.




Publication History

Received: 05 July 2025

Accepted: 04 December 2025

Accepted Manuscript online:
11 December 2025

Article published online:
05 January 2026

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