Abatacept (Orencia), a Promising Treatment for Early-and Late-stage Morphea Subtypes: A Follow-up Study from the Midwest of Ireland
Maria Usman Khan1,2, Fahd Adeeb1,2, Mary brady1, Siobhan Morrissey1, Joseph Devlin1, Alexander Fraser1,2
1. Department of Rheumatology, University Hospital Limerick, Limerick, Ireland 2. Graduate Entry Medical School, University of Limerick, Limerick, Ireland
Morphea is a rare autoimmune inflammatory fibrosing skin disease of unknown aetiology characterized by excessive collagen deposition resulting in significant cosmetic, functional, and psychological sequelae. The treatment is individualized despite various suggested treatment algorithms for morphea subtypes and limited data is available showing some benefit of methotrexate combined with systemic corticosteroids . Abatacept (Orencia) is a recombinant fusion protein that competitively binds to CD80 or CD86 receptors, hence selectively inhibits T-cell activation .
With reference to the work of Stausbøl-Grøn et al.  and our previous pilot work , we conducted a follow-up study to assess the efficacy of abatacept (Orencia), a selective T cell costimulation modulator, in patients with morphea subtypes, where Th-17 subtypes of effector CD4 +T cell has been proposed in the pathogenesis .
Six Caucasian patients with established morphea subtypes according to the Mayo Clinic Classification  and with no contraindication to abatacept were included in this prospective open-label study. Descriptive statistics were reported as median, SD and IQR for non-parametric calculations or number and percentages as appropriate.
This was an open-label, prospective study highlighting the excellent response and good safety profile of abatacept in all patients. Methotrexate in combination with systemic steroid was the only therapy used prior to abatacept in 3 patients at median dose of 17.5 mg/week (median duration of 3 months). 4 of the 6 patients received subcutaneous form of abatacept at dose 125mg/week with the exception of 2 of the 6 patients with severe disease who also received abatacept infusions at standard dose of 10mg/kg body weight as the induction therapy prior to the subcutaneous form. All patients on abatacept received concomitant treatment with methotrexate and low dose glucocorticoid that was discontinued in 3 patients after a mean duration of 12.9 months at median dose of 15mg/week (median duration of 10 month) due to gastrointestinal upset, recurrent urinary tract infection and deranged liver function tests respectively and this cohort showed excellent response using abatacept as monotherapy. Patient demographics and details of outcomes are shown in Table 1. No adverse event was reported with abatacept treatment at a median duration of 29 months.
Abatacept was well tolerated in all 6 patients with excellent clinical outcome. These case series highlights abatacept (Orencia) as a promising therapeutic option for severe or resistant morphea subtypes.