Sarcoidosis: Advances in Therapy

Robert P. Baughman, MD

Marc Judson, MD

This issue of Seminars of Respiratory and Critical Care Medicine focuses on advances in the treatment of sarcoidosis. As sarcoidosis is a multisystem disease, the issue provides an update on the clinical approach to both pulmonary and extrapulmonary manifestations.

The clinical outcome of sarcoidosis is highly variable. Many patients have an asymptomatic or mild clinical course. Drs Dornfield Castro and Pereira provide a review of what features of sarcoidosis are associated with nonlife-threatening disease.

A relatively smaller number of sarcoidosis patients develop severe disease that may have significant morbidity and some mortality. These forms of the disease have been described as “advanced sarcoidosis.” Drs Gupta and Baughman provide a summary of advanced disease, especially pulmonary fibrosis. Several other manifestations of sarcoidosis can lead to advanced disease. Sarcoidosis-associated pulmonary hypertension, reviewed by Dr. Huitema, is associated with increased mortality. Increasing recognition of this condition has led to clinical treatment trials specifically for this condition. Cardiac sarcoidosis has long been a concern due to the risk of sudden death. Historically considered a rare condition except in Japan, advanced imaging and other diagnostic techniques have improved the diagnostic accuracy and prognostic assessment of cardiac sarcoidosis. Furthermore, imaging studies have improved the assessment of the disease activity of cardiac sarcoidosis that has led to improved treatment for this condition. Dr. Birnie provides a balanced review of this complex subject. Neurosarcoidosis is another form of sarcoidosis, which can have devastating consequences. Dr Voortman discusses early recognition and treatment of this entity. Her discussion includes sarcoidosis-associated small fiber neuropathy, a clinically important form of neurosarcoidosis that can greatly impact quality of life. Dr. Seve's group discusses the diagnosis and management of ocular sarcoidosis that can lead to blindness if left untreated.

The skin is one of the most common tissues involved with sarcoidosis, and recognition of this form of the disease can help secure the diagnosis. Dr. Rosenbach's group provides an atlas of the various cutaneous manifestations of the disease. The liver is also commonly involved with sarcoidosis and may require systemic therapy. Dr. Mahevas leads a scholarly review of this topic.

Given that sarcoidosis may involve numerous organs, the health care provider needs to determine which organs in a sarcoidosis patient require screening for disease. Dr Judson provides an overview of what testing should be performed in all patients and when should one perform more detailed testing. Dr James tackles the problematic issue of sarcoidosis overlap syndromes, where patients present with features of sarcoidosis as well as additional inflammatory diseases.

Bone health is a major problem in the aging population. Corticosteroids accelerate bone loss, and vitamin D metabolism can be quite abnormal in sarcoidosis. Drs Zhou and Lower provide a review of monitoring calcium and bone health in sarcoidosis, with guidance on treatment.

Sarcoidosis can have a major impact on the quality of life. Dr Obi reviews the most recent information regarding how to monitor quality of life in sarcoidosis patients. While these instruments are mostly used to therapeutic interventions in sarcoidosis cohorts, they may soon be applied to daily practice in individual patients.

As a bonus treat that we suspect will be useful to clinicians, this issue contains an atlas of radiological and histological features of sarcoidosis. These examples, put together by Dr. Shaikh and colleagues, provide a sampling of the many faces of sarcoidosis.

We believe that the issue will provide a useful clinical resource of this enigmatic disease for the next several years. We thank all the authors for their scholarly work and hope that this issue will assist you in your understanding of sarcoidosis and its management.

Publication History

Article published online:
10 August 2020