Pulmonary sarcoidosis: diagnostic and treatment update

Abstract

Sarcoidosis is a multisystem disorder of uncertain etiology, characterized by non-caseating granuloma formation in the affected organs. Significant progress has been made in the understanding of the immunopathological aspects of this disease over recent years. However, the causative agent(s) remain unknown. There remains controversy around the best treatment regimens and duration of immunosuppressive therapy. This is largely related to the heterogeneity of disease, a paucity of large randomized controlled trials and the fact that a significant proportion of patients do not require treatment. This review aims to summarize the evidence for the management of pulmonary sarcoidosis and to provide an update on recent developments in diagnosis and treatment.

Keywords::

• corticosteroids
• granulomatous inflammation
• immunosuppression
• sarcoidosis

Acknowledgements

The authors would like to thank D Rowlands for providing the histopathology image (Figure 1).

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Sarcoidosis is a multisystem granulomatous disease of uncertain etiology with a propensity to involve pulmonary parenchyma in a majority of cases.

• Endobronchial ultrasound-guided transbronchial needle aspiration is an increasingly utilized tool for the diagnosis of sarcoidosis and as experience with this technique expands in coming years it may supersede transbronchial biopsy as the primary histological diagnostic modality.

• Sarcoidosis provides a considerable challenge in terms of treatment options due to a paucity of high-quality randomized clinical trials.

• The management of pulmonary sarcoidosis can be dichotomized into corticosteroid responsive and corticosteroid non-responsive groups and a pragmatic approach to treatment is recommended in view of a limited evidence base for effective therapies.

• A novel treatment option in pulmonary (and extrapulmonary) sarcoidosis is the use of monoclonal antibodies against TNF-α. It seems likely that we will experience an expansion in the use of this pharmacological therapy in sarcoidosis, particularly in patients with multisystem involvement.

• The pathophysiology of pulmonary hypertension in sarcoidosis is uncertain but multifaceted and poses a diagnostic and treatment challenge for pulmonologists.

Notes