Sarcoidosis

Robert P. Baughman, M.D.

Daniel A. Culver, D.O.

Ulrich Costabel, M.D.

This issue of Seminars in Respiratory and Critical Care Medicine is dedicated to sarcoidosis. We have chosen to tackle the diverse aspects of sarcoidosis by asking our contributors to update us on the etiology, diagnosis, and management of the disease. Drs. Oswald-Richter and Drake have written an article summarizing the information to date regarding the possible role of infection as a cause for sarcoidosis. Although the concept that infections such as mycobacteria may cause sarcoidosis is not new, recent studies have demonstrated specific inflammatory reactions to mycobacteria in sarcoidosis patients. At the same time, our knowledge regarding genetic susceptibility to this as yet unknown antigen is also growing. Dr. Grunewald has provided a summary of work in this area. This especially includes work regarding the way a patient’s genetic profile, such as human leukocyte antigen (HLA), may influence the manifestations and outcome of sarcoidosis. Dr. Zissel and colleagues have reviewed the immunologic reaction that is sarcoidosis. This of course includes the formation of the granuloma.

The diagnosis of sarcoidosis can be at times challenging. Dr. Ulrich Costabel’s group has nicely reviewed some of the techniques that pulmonologists may use in the diagnosis of sarcoidosis. This includes bronchoalveolar lavage (BAL) and endobronchial ultrasonography. It seems that these techniques may be complementary in making a diagnosis. The total body imaging of positron emission tomography (PET) has allowed clinicians to identify potential new areas to diagnose. In addition to diagnosis, the pulmonologist is usually involved in assessing the extent of pulmonary disease and response to treatment. Drs. Keir and Wells review the role of pulmonary function tests and other modalities in this important aspect of care.

The majority of the remaining articles deal with specific aspects of sarcoidosis care. Two causes of significant morbidity in sarcoidosis are neurological and cardiac manifestations. Drs. Vargas and Stern provide their expertise in this area. Dr. Nunes and colleagues discuss their experience in dealing with cardiac sarcoidosis in their large clinic in France. Although both of these articles review the literature, the authors present their own expertise in providing diagnosis and treatment recommendations for these two manifestations.

The skin, eye, and joint manifestations may not be life threatening, but they are common, difficult problems in sarcoidosis management. Drs. Marchell and Judson have summarized the various manifestations of skin disease and provided an algorithm about treatment. Dr. Baughman and colleagues discuss the diagnosis and management of ocular sarcoidosis. Dr. Sweiss is a rheumatologist with broad experience in the management of sarcoidosis. She and her group provide a primer about the various rheumatologic manifestations of sarcoidosis. Dr. Burke and his colleagues discuss the complicated relationship between vitamin D and calcium metabolism in sarcoidosis.

The final section is devoted to therapy. Drs. De Vries, Lower, and Drent combine their experience in dealing with the effects of sarcoidosis on the overall quality of life of the patient. They focus on fatigue, an area where new studies are providing insights into etiology and providing treatment options. Sarcoidosis-associated pulmonary hypertension is an increasingly recognized complication of pulmonary disease. Drs. Palmero and Sulica review the diagnosis and management of this complication of the disease. The final article is offered by Drs. Lazar and Culver, who summarize the various drugs and provide an approach to therapy for sarcoidosis.

We hope that you find this issue as edifying as we did in preparing it.