Appeal type: Medical necessity
Decision: Upheld

Diagnosis: Diffuse cutaneous systemic sclerosis
Treatment: Truxima (rituximab) 500MG (milligrams)/50 ML (milliliters) Vial
The insurer denied: Truxima (rituximab) 500MG (milligrams)/50 ML (milliliters) Vial
The denial is upheld

The patient is an adult female with a diagnosis of diffuse cutaneous systemic sclerosis diagnosed in 2 years ago. She was hospitalized that same year with an NSTEMI (non-ST-elevation myocardial infarction). She has previously been on methotrexate, which was later discontinued, and she was started on mycophenolate mofetil. She was started on Tocilizumab in last year. She has also been on hydroxychloroquine in the past. She was diagnosed with myocarditis in early this year with symptoms of fatigue and a cardiac MRI (magnetic resonance imaging). She had an endomyocardial biopsy which showed unremarkable endomyocardial tissue and no evidence of myocarditis or amyloidosis. She had a cardiac PET (positron emission tomography) scan in this year, which was consistent with myocarditis with an ejection fraction of 45 to 50%(percent). She had a right heart catheterization in early this year, which showed a normal pulmonary arterial pressure. The patient is currently on the maximal dose of Mycophenolate mofetil at 1500 mg (milligrams) twice a day and tocilizumab 162 mg (milligrams) weekly. She has also received 2 rounds of IVIG (intravenous immune globulin). Her rheumatologist wants to stop tocilizumab and start Truxima (rituximab). 
Truxima (rituximab) 500 milligram/50 milliliter Vial was denied as the patient does not have documentation of diagnosis of non-Hodgkin's lymphoma, chronic lymphocytic leukemia, granulomatosis with polyangiitis, microscopic polyangiitis, rheumatoid arthritis, or another FDA (Food and Drug Administration) approved indication for the requested medication. 
She is currently on mycophenolate mofetil for her skin thickening and tocilizumab for her inflammatory arthritis, but these have not been effective for treatment of myocarditis. Myocarditis is a life-threatening complication of scleroderma and if not promptly treated with rituximab could result in prolonged, extensive hospitalization and even death.

The proposed treatment is not medically necessary. 

Rituximab is not medically necessary for this patient as it is not in accordance with the nationally accepted standards of medical practice; it is clinically inappropriate and not considered effective 
for patient's illness.

This patient has involvement of multiple organs including the heart causing myocarditis. This is a relatively rare finding in these patients. Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. While there is no established treatment practices for myocarditis associated with SSc (scleroderma), immunosuppressive agents are typically used in a manner comparable to that for the treatment of interstitial lung disease associated with SSc (scleroderma). This patient has already tried and failed methotrexate, mycophenolate mofetil and tocilizumab. There is some recent systemic review (Reference 1, 2) that indicate treatment with azathioprine and. Reported regimens in lymphocytic myocarditis case reports and case series include various immunosuppress agents, including corticosteroids, azathioprine, cyclosporine, cyclophosphamide, thymoglobulin, methotrexate, and IM (intramuscular) with IVIG (intravenous immune globulin).

There is minimal data on Rituximab in Systemic sclerosis associated myocarditis. This patient has not tried azathioprine, cyclosporine, cyclophosphamide, which have more supporting data than Rituximab. (Reference 3,4) Therefore, Rituximab is not considered necessary for this patient.