Appeal type: Formulary Exception
Decision: Overturned

Diagnosis: Nodular scleritis and recurrent bilateral anterior uveitis
Treatment: Acthar Gel
The insurer denied the Acthar Gel.
The denial is overturned.

The patient is a female with a history of nodular scleritis and recurrent bilateral anterior uveitis for whom coverage of Acthar Gel is the subject of review. Coverage was previously denied coverage by the health plan, as current plan-approved criteria do not allow coverage of Acthar Gel unless the patient has a diagnosis of infantile spasms or multiple sclerosis. Alternatives on the plan's formulary are identified as dexamethasone, prednisolone, methylprednisolone, hydrocortisone, and prednisone. The plan requires a trial of 3 alternatives if there are 3 or more covered alternatives.

The patient is identified as having Ankylosing Spondylitis and Crohn's disease which have been causing severe eye inflammation with extreme flare-ups about every 3 months and/or scleritis for the past 14 years despite receiving systemic treatment for her rheumatologic disease. The patient is currently being treated with Humira (adalimumab) and has tried and failed samples of prednisolone, atropine, and Durezol. 

The health plan should cover the formulary exception for Acthar Gel.

Chronic use of formulary systemic steroids would place the patient at unnecessary risk and run counter to the standard of care, as the patient has already moved on to steroid-sparing agents. According to the package insert, H.P. (highly purified) Acthar Gel may be used for ophthalmic diseases. In this case, Acthar is proposed for the treatment of nodular scleritis and recurrent bilateral anterior uveitis that has been refractory to topical steroids (prednisolone, Durezol), immunosuppressive therapy (methotrexate), and biologics (Humira). 

Several case reports are identified in the peer-reviewed medical literature that supports the safety and efficacy of Acthar in the treatment of such non-infectious uveitis. Agarwal et al recently published a case report describing the potential role of subcutaneously administered ACTH (adrenocorticotropic hormone) gel in the management of a patient with non-infectious panuveitis with significant retinal vasculitis, macular edema, and optic nerve inflammation. Therapy with ACTH (adrenocorticotropic hormone) gel resulted in rapid and adequate control of retinal vascular leakage, macular edema, and optic nerve inflammation. 

In a second case report by Ahmad et al, a 16-year-old female with recurrent uveitis associated with poorly controlled juvenile idiopathic arthritis reported remission of uveitis symptoms after receiving RCI (repository corticotropin injection) (80U [units] administered intramuscularly 2 times per week) as additive therapy. 

Sharon et al. reported the clinical course of three patients with bilateral, non-infectious anterior, and intermediate uveitis, treated with ACTH (adrenocorticotropic hormone) gel for 12 months. The authors concluded that subcutaneous adrenocorticotropic hormone gel has shown to be a safe and effective therapy in the management of non-infectious uveitis. 

Nelson et al performed a retrospective medical records review of patients who had a diagnosis of uveitis, received repository corticotropin injection (RCI) in the prior 12 months, and had completed or were receiving RCI (repository corticotropin injection) treatment at the time of data collection. The author concluded that physicians individualized RCI (repository corticotropin injection) therapy among patients who suffered uveitis for several years and when previous therapies were inadequate. Most patients improved after initiating RCI (repository corticotropin injection), most commonly in vision. As uveitis is usually treated with a stepwise approach using corticosteroids, immunosuppressive therapy, and biologics, and this patient has failed or been intolerant to each of these therapies, the medical necessity of Acthar is established in this case.