4.5 Article

Tofacitinib as a Steroid-Sparing Therapy in Pulmonary Sarcoidosis, an Open-Label Prospective Proof-of-Concept Study

期刊

LUNG
卷 199, 期 2, 页码 147-153

出版社

SPRINGER
DOI: 10.1007/s00408-021-00436-8

关键词

Sarcoidosis; Janus kinase inhibitors; Pulmonary sarcoidosis

资金

  1. NIH [RO1 EY020249, RO1 EY026572, P30 EY010572, KL2TR002370, 3T32HL094294-08S1]
  2. Research to Prevent Blindness
  3. Grandmaison Fund for Autoimmunity Research
  4. William and Mary Bauman Foundation
  5. Stan and Madelle Rosenfeld Family Trust
  6. OHSU Wheels Up Program

向作者/读者索取更多资源

In this proof-of-concept study, adding a JAK-inhibitor allowed 60% of patients with pulmonary sarcoidosis to successfully taper corticosteroids, showing potential as a promising therapy for this condition. Further investigation in randomized trials is warranted for JAK-inhibitors in the treatment of pulmonary sarcoidosis.
This is a prospective, open-label, proof-of-concept study of tofacitinib, a Janus kinase inhibitor, as a steroid-sparing therapy in corticosteroid-dependent pulmonary sarcoidosis. Five patients with corticosteroid-dependent pulmonary sarcoidosis were treated with tofacitinib 5 mg twice daily. The primary endpoint was a >= 50% reduction in corticosteroids at week 16 with no worsening in pulmonary function or respiratory symptoms. 60% of patients (3/5) met the primary endpoint. One patient was lost to follow up prior to steroid taper, and another was withdrawn due to worsening of known neurosarcoidosis. The three patients who met the primary endpoint each tapered to <= 5 mg/day prednisone, respiratory symptoms improved, and spirometry remained stable. In this proof-of-concept study, the addition of a JAK-inhibitor allowed 60% of patients with pulmonary sarcoidosis to successfully taper corticosteroids. JAK-inhibitors are a promising therapy for pulmonary sarcoidosis, which require further investigation in randomized trials.

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