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Refractory Pulmonary Sarcoidosis: Proposal of a Definition and Recommendations for the Diagnostic and Therapeutic Approach

Korsten, Peter MD; Strohmayer, Katharina MD; Baughman, Robert P. MD; Sweiss, Nadera J. MD

doi: 10.1097/CPM.0000000000000136
Interstitial, Inflammatory, & Occupational Lung Disease
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Patients with sarcoidosis undergo spontaneous remission or may be effectively controlled with glucocorticoids alone in many cases. Progressive and refractory pulmonary sarcoidoisis constitute >10% of the patients seen at specialized centers. Pulmonary fibrosis and associated complications, such as infections and pulmonary hypertension, are leading causes of mortality. No universal definition of refractoriness exists; we therefore propose classifying patients as having refractory disease when the following criteria are fulfilled: (1) progressive disease despite at least 10 mg of prednisolone or equivalent for at least 3 months and the need for additional disease-modifying antisarcoid drugs due to a lack of efficacy, drug toxicity, or intolerability; and (2) treatment started for significant impairment of life due to progressive pulmonary symptoms. Both criteria should be fulfilled. Treatment options in addition to or instead of glucocorticoids for these patients include second-line (methotrexate, azathioprine, leflunomide) and third-line agents (infliximab, adalimumab). Other immunmodulating agents can be used, but the evidence is very limited. Newer agents with antifibrotic properties, such as pirfenidone or nintedanib, might also hold promise for the pulmonary fibrosis seen in sarcoidosis. Treating physicians have to actively look for potentially treatable complications, such as pulmonary hypertension, cardiac disease, or infections before patients are classified as treatment-refractory. Ultimately, lung transplantation has to be considered as a treatment option for patients not responding to medical therapy. In this review, we aim to propose a new definition of refractoriness, describe the associated clinical features, and suggest the therapeutic approach.

*Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany

Divisions of Pulmonary Medicine

§Rheumatology, University of Illinois Hospital and Health Science System, Chicago, IL

Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH

Disclosure: R.P.B. has research grants for sarcoidosis from Celgene, Pfizer, Forest, Gilead, Bayer, Mallinckrodt, and Novartis. He has been a consultant for Celgene, Novartis, and Mallinckrodt. The remaining authors declare that they have no conflicts of interest.

Address correspondence to: Nadera J. Sweiss, MD, Department of Rheumatology, University of Illinois Hospital and Health Science System, 840 S Wood Street, MC 733 Chicago, IL 60612. E-mail: nsweiss@uic.edu.

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