Appeal type: Experimental/Investigational
Decision: Overturned

Diagnosis: Diffuse eosinophilic fasciitis.
Treatment: Xeljanz.
The insurer denied coverage for Xeljanz.
The denial is overturned.

This is an adult female with a history of hypereosinophilic fasciitis. The patient has tried and failed prednisone, methotrexate, CellCept and mepolizumab. The treating provider has requested therapy with Xeljanz.

Per Wright, N, A., Mazori, D, R., Patel, M., Merola, J, F., Femia, A, N., Vleugels, R, A., (2016), "It is important to note that the JAK (Janus kinase inhibitors) tofacitinib, ruxolitinib, and baricitinib have all demonstrated efficacy in the treatment of idiopathic hypereosinophilic syndrome, eosinophilic esophagitis, and eosinophilic fasciitis."

Per Kim, S. R., Charos, A., Damsky, W., Heald, P., Girardi, M., & King, B. A. (2018), "Morphea is a fibrosing skin disease with a spectrum of presentations ranging from localized indurated plaques to circumferential involvement of limbs that impairs mobility.1 Eosinophilic fasciitis (EF) is often considered a severe form of morphea.1 Treatment of severe sclerosing diseases, such as generalized deep morphea (GDM) and EF, can be challenging. Methotrexate and prednisone are therapeutic mainstays but are not effective in all cases.1 A recent retrospective study showed that ruxolitinib, a Janus kinase (JAK)1/2 inhibitor, was effective in treating sclerodermatous cutaneous graft-versus-host disease.2 Another series found the benefit of JAK inhibitors in the treatment of hypereosinophilic syndrome (HES) with cutaneous involvement.3 Based on these data and mechanistic studies of sclerosis from animal models, we hypothesized that JAK inhibitors, such as tofacitinib, would be effective for GDM and EF."

Per Solimani, F., Meier, K. and Ghoreschi, K., (2019), "Increasing evidence confirming the beneficial role of systemic or topical JAKi (Janus kinase inhibitors) in the treatment of these disorders is accumulating. Since the JAK/STAT (signal transducer and activator of transcription) signaling pathway plays a crucial role for many cytokines (Figure 2), a variety of inflammatory dermatological disorders may benefit from this new class of immunomodulators. For instance, lichen sclerosus et atrophicans (LSA) is an inflammatory disorder of skin and genital mucosa associated with severe pruritus and scaring. Many patients do not respond adequately to topical steroids (165). Due to its frequent recalcitrant course, there is an urgent need of new therapeutics for LSA. Inflammatory factors like IFN (Interferons), CXCL10 (chemokine receptor), and CCR5 (chemokine receptor) are highly expressed in lesional skin, suggesting that LSA is a Th1 (T cell helper 1) -dominated disorder (166). Oral or topical application of JAKi could stop the inflammatory circuit in LSA. Case reports on positive effects of JAKi exist for LSA and for patients with other sclerosing skin diseases like morphea, eosinophilic fasciitis, or systemic sclerosis (167, 168)."
There are no FDA (Food and Drug Administration) /standard treatment options for this rare disease. However, given the previous failed medications for this patient, and in view of the paucity of options, Xeljanz is likely to be beneficial, is an appropriate next step in therapy, and should be approved for this patient.

The carrier's denial of Xeljanz should be overturned.