INTRODUCTION
Dermatomyositis (DM) is an autoimmune myopathy with characteristic dermatologic features.1 Tofacitinib is an immunomodulator with proven efficacy against numerous immune-mediated disorders, including rheumatoid arthritis (RA) and psoriasis.2 Several reports have demonstrated oral tofacitinib's ability to treat the cutaneous and extracutaneous manifestations of refractory dermatomyositis (DM).1,4,5 However, evidence for sustained improvement remains limited.2,3 The goal of this study is to investigate the long-term response of recalcitrant DM to oral and topical tofacitinib at varied dosing regimens.
A retrospective review was conducted of all patients from May 2012 to March 2021 who were treated for DM with tofacitinib. This study was approved by the Institutional Review Board at the Medical University of South Carolina. Four patients were identified (Table 1). All were white and refractory to numerous therapies. The median duration of treatment prior to initiating tofacitinib was 7.5 years (range 1-22).
Tofacitinib was used adjunctively in all patients (Table 2). The median duration of tofacitinib therapy was 23 months (range 11-42). Topical therapy was used in one patient. All patients exhibited favorable initial and sustained clinical responses. The cutaneous response to therapy was scored as mild (2), moderate (1) and significant (1) relative to pre-treatment baseline using patientreported factors (pruritus and pain) and physical examination findings (erythema, tenderness, and affected area).
In patient 1, topical therapy was only applied to the eyebrows, and this led to hair regrowth and reduced inflammation. Remarkable improvement was observed in patients 2 and 3 (Figure 1), and these effects were enhanced when dosing was increased to three times daily after 3 and 4 months, respectively. Concurrent therapies were tapered to 5 mg of daily prednisone (patient 2) and a reduced dose of intravenous immunoglobulin (IVIG) with topical steroids (patient 3). Patient 4 initially presented with anti-synthetase syndrome and was eventually diagnosed with RA. Although his cutaneous disease and RA
A retrospective review was conducted of all patients from May 2012 to March 2021 who were treated for DM with tofacitinib. This study was approved by the Institutional Review Board at the Medical University of South Carolina. Four patients were identified (Table 1). All were white and refractory to numerous therapies. The median duration of treatment prior to initiating tofacitinib was 7.5 years (range 1-22).
Tofacitinib was used adjunctively in all patients (Table 2). The median duration of tofacitinib therapy was 23 months (range 11-42). Topical therapy was used in one patient. All patients exhibited favorable initial and sustained clinical responses. The cutaneous response to therapy was scored as mild (2), moderate (1) and significant (1) relative to pre-treatment baseline using patientreported factors (pruritus and pain) and physical examination findings (erythema, tenderness, and affected area).
In patient 1, topical therapy was only applied to the eyebrows, and this led to hair regrowth and reduced inflammation. Remarkable improvement was observed in patients 2 and 3 (Figure 1), and these effects were enhanced when dosing was increased to three times daily after 3 and 4 months, respectively. Concurrent therapies were tapered to 5 mg of daily prednisone (patient 2) and a reduced dose of intravenous immunoglobulin (IVIG) with topical steroids (patient 3). Patient 4 initially presented with anti-synthetase syndrome and was eventually diagnosed with RA. Although his cutaneous disease and RA
