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 fascitis. The patient has tried and failed prednisone, methotrexate, Cellcept and mepolizumab. The treating provider has requested therapy with Xeljanz.

Scientific evidence in peer-reviewed literature supports a result of improvement in health outcome.

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 (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)."

The patient has hypereosinophilic fascitis, which is a rare disease. Treatment options are based on small case series and small studies. There are no FDA (Food and Drug Administration) approved drugs for this rare condition.

The patient's disease has been shown refractory to multiple therapies, and Xeljanz has shown to be helpful in these cases and is an appropriate next step in this patient's therapy.

Xeljanz does not have FDA approval for this diagnosis.

This patient a good candidate for Xeljanz.

The adverse risks are not increased over standard treatment/services.

The patient's disease is progressing and affecting the mobility in the upper extremities and activities of daily living (ADLs). The patient has failed the standard therapies. If not treated appropriately, the disease could continue to progress with irreversible damage. Xeljanz has been shown to be helpful in these refractory cases

Xeljanz should be approved for 12 months.

The carrier's denial of Xeljanz should be overturned.