‘You’d think that if we’re comfortable with [managing] lupus, we’d also be comfortable with sarcoidosis. We should be taking a stronger role [with this condition].’ —Dr. Yee
Treatment
Corticosteroids and repository adrenocorticotropic hormone (ACTH) are the only medications approved by the U.S. Food & Drug Administration for the treatment of sarcoidosis. “However, [corticosteroids and ACTH] are my first line of defense and my last resort at the same time,” Dr. Yee said.
Conventional anti-metabolites are frequently used off label with methotrexate, azathioprine, mycophenolate mofetil and leflunomide.4 Hydroxychloroquine, thalidomide, cyclosporine and cyclophosphamide are used less commonly.
Tumor necrosis factor (TNF) inhibitors have proven effective. Infliximab has been shown to improve both pulmonary and extra-pulmonary disease manifestations.5,6 “The bottom line is that infliximab and adalimumab work [for sarcoidosis], but etanercept doesn’t; this is probably[for] the same reason it doesn’t work in Crohn’s disease. As a soluble receptor, [etanercept] cannot get to the granuloma,” Dr. Yee said.
The heart: What about patients with cardiac sarcoidosis? In 2003, the ATTACH trial studied the use of infliximab for the treatment of patients with non-sarcoidosis heart failure with negative and detrimental results.7 In patients receiving high-dose infliximab (i.e., 10 mg/kg), the combined risk of death from any cause or hospitalization for heart failure was increased through 28 weeks compared with patients not on infliximab or those on low-dose infliximab (5 mg/kg). This finding led to the contraindication for TNF inhibitors in patients with reduced ejection fraction, of which we are all aware.
“For many years, the cardiologists at my institution fought back at me when we had a [patient with] cardiac sarcoidosis, and I wanted to give infliximab. Fortunately, this issue has now been settled. In 2020, Baker et al. showed absolutely no deterioration of cardiac function in patients given infliximab for cardiac sarcoidosis and a reduction in steroid dependence,” Dr. Yee said.8
Of note, recent studies also show promise for tofacitinib for the treatment of sarcoidosis.9
Stopping Medications
When it comes to stopping medications in patients with sarcoidosis, data come from retrospective studies.
Dr. Yee identified eight patients in his clinic who could come off TNF inhibitor treatment after establishing complete remission.10 Before TNF inhibitors were stopped, all eight patients remained stable off systemic corticosteroid therapy for at least a year. So, he said, “the sustained capacity to remain off corticosteroids during TNF inhibitor therapy may be a favorable prognostic indicator for maintained disease remission.” 10
