Systemic Sclerosis: when insurers say no, reviewers often say yes

Where the denial was overturned

Findings: The physician reviewer found that Nature of Statutory Criteria/Case Summary: The parent of a patient has requested authorization and coverage for Actemra ACTPen subcutaneous pen injector. In a study, pediatric patients with systemic sclerosis treated with Actemra showed significant improvements and Actemra was well tolerated. Similarly, a case report of a pediatric patient with multi-organ systemic sclerosis treated with Actemra monotherapy showed clinical remission without adverse events. In this case, the patient has a history of severe and refractory systemic sclerosis characterized by cutaneous lesions, synovitis, and multiple areas of fasciitis in the chest, abdomen, and pelvis with findings indicative of deep soft tissue involvement and progressive disease despite standard immunosuppressive therapy. The literature supports the use of Actemra in this clinical scenario.

Nature of Statutory Criteria/Case Summary: A patient has requested authorization and coverage for Botox injections. Findings: The physician reviewer found that evidence supports the use of botulinum toxin as an effective treatment for Raynaud’s phenomenon secondary to systemic sclerosis, particularly in cases refractory to conventional therapy. Studies have demonstrated that Botox improves blood flow, reduces pain, and promotes healing in ischemic digits. In this case, the patient has severe sclerodactyly and Raynaud’s phenomenon refractory to treatment with Myfortic and tadalafil and causing significant functional impairment. Overall, the medical literature supports the use of Botox in this patient’s clinical setting. Therefore, the requested Botox injections are medically necessary for the treatment of this patient.

Where the denial was upheld

Nature of Statutory Criteria/Case Summary: An enrollee has requested authorization and coverage for a positioning pillow and compression socks.Scleroderma/systemic sclerosis is a rare condition that typically affects young to middle-aged women. It is characterized by hardening and tightening of the skin and connective tissues that is caused by an overproduction and accumulation of collagen. The exact cause is unknown, but the condition is felt to be largely autoimmune in nature (Cutolo, M., et al.). The disease manifestations are heterogenous, affecting multiple organ systems. Edema is one of the hallmarks of the early phase of scleroderma/systemic sclerosis. This edema, which is nonfibrotic and interstitial, is caused by pathology in the endothelium resulting in vascular leak, underscoring the importance of targeting treatment towards the primary disease (Bruni, C., et al.).

Nature of Statutory Criteria/Case Summary: An enrollee has requested authorization and coverage for Gammaked at a hospital-associated infusion center.Findings: Two out of three physician reviewers found that the requested Gammaked at a hospital-associated infusion center is not likely to be more beneficial than any available standard treatment for the patient's medical condition.At this time, case series and small trials have suggested potential benefit from intravenous immunoglobulin (IVIG) for skin fibrosis as well as other symptoms, including gastrointestinal tract manifestations, of systemic sclerosis. Clinical trials are being planned, but the mechanism of action for antifibrotic activity remains unclear. Several small, uncontrolled studies have evaluated the effect of intravenous administration of immune globulin on dermal fibrosis, each with reported improvement.

These outcomes come from California’s external review program — an independent physician panel whose decision binds the insurer. Every state has an equivalent, and internal appeals succeed even more often. If your care for systemic sclerosis was denied, the published record says the denial is worth fighting.