Appeal type: Experimental/Investigational
Decision: Overturned

Diagnosis: Systemic lupus erythematosus, inflammatory arthritis, malar rash, photosensitivity, oral ulcers and Raynaud's phenomenon
Treatment: Sildenafil 20mg (milligrams)
The insurer denied: Sildenafil 20mg (milligrams)
The denial is overturned

This patient is an adult female with history of systemic lupus erythematosus (SLE). She has a history of a positive ANA (antinuclear antibody), inflammatory arthritis, malar rash, photosensitivity, oral ulcers and Raynaud's phenomenon. She is currently treated with Benlysta infusions for her SLE (systemic lupus erythematosus) along with hydroxychloroquine. She was currently treated with sildenafil 20mg (milligrams) daily for her Raynaud's until recently discontinuing for a surgery and since then has had worsening Raynaud's symptoms. She has previously tried and failed calcium channel blockers nifedipine and amlodipine for her Raynaud's. The request for sildenafil was initially denied coverage by the health plan due to the diagnosis submitted not being an FDA (United States Food and Drug Administration)-approved indication for the requested product, and therefore the patient does not meet criteria for approval. The reason the provider is appealing the determination is because the patient has previously responded well to sildenafil.

The health plan should cover the requested Sildenafil.

The patient has secondary Raynaud's due to her systemic lupus erythematosus. Patients with secondary RP (Raynaud's phenomenon) are more likely to have severe attacks, and disease management is more likely to be based upon pharmacologic agents, while continuing non-pharmacologic measures. The goals of therapy are to improve quality of life and to prevent ischemic tissue injury. 
Literature supports the addition or substitution of other pharmacologic agents, such as topical nitrates or phosphodiesterase (PDE) 5 inhibitors (sildenafil), if calcium channel blockers (CCB), such as nifedipine or amlodipine which the patient has previously tried, are insufficient. In patients who do not respond adequately to a CCB (calcium channel blocker) alone, experts suggest the addition of a PDE (phosphodiesterase) inhibitor (e.g., sildenafil). In patients in whom a PDE (phosphodiesterase) inhibitor is not available, effective, or well-tolerated, experts suggest the use of topical nitrates. 
In patients who have obtained some but inadequate benefit from a CCB (calcium channel blocker) and who tolerate that therapy, experts suggest the addition of a PDE (phosphodiesterase) type 5 inhibitor and continue the CCB (calcium channel blocker). The efficacy of sildenafil was best examined in a meta-analysis of six randomized controlled trials that included 244 patients with secondary RP (Raynaud's phenomenon). There was a modest but significant benefit of PDE (phosphodiesterase) inhibitors on Raynaud's Condition Score (RCS), as well as on the frequency and duration of RP (Raynaud's phenomenon) attacks. PDE (phosphodiesterase) inhibitors reduced the frequency of RP (Raynaud's phenomenon) attacks by approximately 0.5/day compared with placebo, which is comparable to the reduction reported in another meta-analysis assessing the efficacy of CCBs (calcium channel blockers) in systemic sclerosis (SSc)-related RP (Raynaud's phenomenon) (0.6/day). In patients who do not reach the therapeutic goal or who are unable to tolerate a CCB (calcium channel blocker), a PDE (phosphodiesterase) type-5 inhibitor, or a topical nitrate, alone or in combination, then experts use one of the other oral agents such as an alpha-adrenergic receptor blocker or an angiotensin II receptor antagonist for which there is some evidence of benefit. Most studies of these agents do not provide solid evidence of efficacy due to small sample sizes, limited duration of study, or a lack of control data. Given this data, it is recommended that sildenafil be approved to better treat this patient's RP (Raynaud's phenomenon) and to prevent digital ischemia.