Oscar Rystiggo (rozanolixizumab-noli) (PG190) Form

Generalized myasthenia gravis (gMG) is a chronic autoimmune neuromuscular disorder characterized by muscle weakness and fatigue. It is caused by the production of autoantibodies that target components of the neuromuscular junction, such as the acetylcholine receptor (AChR) or muscle-specific tyrosine kinase (MuSK). The condition leads to a breakdown in communication between nerves and muscles, resulting in weakness and fatigue of voluntary muscles. Symptoms of gMG can vary but commonly include weakness of the eye muscles (ocular myasthenia), drooping eyelids (ptosis), blurred or double vision (diplopia), changes in facial expressions, difficulty swallowing, and shortness of breath. The severity of gMG is often classified using the Myasthenia Gravis Foundation of America (MGFA) Clinical Classification, which categorizes the disease into five main classes (I-V) based on signs, symptoms, and degree of impairment. This classification helps guide treatment decisions and assess disease progression. 1 Rystiggo (rozanolixizumab-noli) is a prescription medicine indicated for the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-AChR or anti-MuSK antibody positive. It is administered through subcutaneous infusion and is designed to target the underlying autoimmune mechanisms of gMG. Definitions "Generalized myasthenia gravis (gMG)" is a chronic autoimmune neuromuscular disorder characterized by fluctuating weakness of voluntary muscles. "Anti-acetylcholine receptor (anti-AChR) antibodies" are autoantibodies directed against the nicotinic acetylcholine receptor found at the neuromuscular junction. "Anti-muscle specific tyrosine kinase (anti-MuSK) antibodies" are autoantibodies directed against the muscle-specific tyrosine kinase protein. "Myasthenia Gravis Foundation of America (MGFA) Clinical Classification" is a system that categorizes disease severity into five main classes (I-V) with subclasses based on signs, symptoms, and degree of impairment. "Myasthenia Gravis Activities of Daily Living (MG-ADL)" is an 8-item patient-reported questionnaire that assesses daily functions often impacted by myasthenia gravis. Total score ranges from 0 to 24, with a higher score indicating more disability. A positive change in the score indicates worsening and a negative change indicates improvement. "Quantitative Myasthenia Gravis (QMG)" is a comprehensive 13-item scale specifically designed to accurately assess the severity of myasthenia gravis. It evaluates various aspects such as endurance, fatigability, and fluctuations in symptoms. The scale assigns scores ranging from 0 to 39, with higher scores indicating a more severe manifestation of the disease. A positive change in the score indicates worsening and a negative change indicates improvement. 2 Medical Necessity Criteria for Initial Authorization The Plan considers Rystiggo (rozanolixizumab-noli) medically necessary when ALL of the following criteria are met: 1. Prescribed by or in consultation with a neurologist or neuromuscular disease specialist; AND 2. The member is 18 years of age or older; AND 3. The member has a confirmed diagnosis of generalized myasthenia gravis (gMG) AND documentation of ALL of the following: a. Positive serologic test for anti-acetylcholine receptor (anti-AChR) OR anti-muscle specific tyrosine kinase (anti-MuSK) antibodies; and b. Myasthenia Gravis Foundation of America (MGFA) Clinical Classification Class II to IVa (see Appendix, Table 1); and c. Baseline Myasthenia Gravis-Activities of Daily Living (MG-ADL) total score of at least 3 (with at least 3 points from non-ocular symptoms); AND 4. IF anti-AChR antibody positive, the member is unable to use, limited by toxicity, or has adequately tried and failed or experienced insufficient response to at least TWO standard therapies for gMG, such as: a. Cholinesterase inhibitors (eg, pyridostigmine); and/or b. Corticosteroids (e.g., prednisone) or inability to taper steroids below a reasonably acceptable level without return of symptoms; and/or c. Immunosuppressive therapies (e.g., azathioprine, cyclosporine, mycophenolate mofetil, cyclophosphamide, tacrolimus); AND 5. Will not be used concomitantly with other immunomodulatory biologic therapies (e.g., efgartigimod alfa, rituximab, ravulizumab, zilucoplan, etc.); AND 6. Prescribed at a dose and frequency that is within FDA approved labeling OR is supported by compendia or evidence-based published dosing guidelines for the requested indication. If the above prior authorization criteria are met, the requested product will be authorized for 16- weeks. Medical Necessity Criteria for Reauthorization Reauthorization for 6 months will be granted if the member has recent (within the last 3 months) clinical chart documentation demonstrating ALL of the following criteria: 1. Prescribed by or in consultation with a neurologist or neuromuscular disease specialist; AND 2. Documentation of positive clinical response to therapy, such as ANY of the following: 3 a. Improvement in Myasthenia Gravis-Activities of Daily Living (MG-ADL) OR Quantitative Myasthenia Gravis (QMG) score from baseline; and/or b. Achievement of minimal symptom expression or pharmacological remission; and/or c. Lack of relapses or reduced frequency/severity of relapses compared to baseline; AND 3. Ongoing therapy is required to maintain disease stability and control; AND 4. There is no unacceptable toxicity or adverse reaction to therapy, such as: a. Serious infections (e.g. serious respiratory or urinary tract infections); and/or b. Severe hypersensitivity reactions; and/or c. Severe immunosuppression; and/or d. Other intolerable side effects or reactions; AND 5. Will not be used concomitantly with other immunomodulatory biologic therapies (e.g., efgartigimod alfa, rituximab, rozanolixizumab, ravulizumab, zilucoplan, etc.); AND 6. Prescribed at a dose and frequency that is within FDA approved labeling OR is supported by compendia or evidence-based published dosing guidelines for the requested indication. Experimental or Investigational / Not Medically Necessary Rystiggo (rozanolixizumab-noli) for any other indication or use is considered not medically necessary by the Plan, as it is deemed to be experimental, investigational, or unproven. Non-covered indications include, but are not limited to, the following: ● Chronic Inflammatory Demyelinating Polyradiculoneuropathy ● Fibromyalgia ● Leucine-Rich Glioma Inactivated 1 Autoimmune Encephalitis ● Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease (MOG-AD) ● Primary Immune Thrombocytopenia (ITP) ● Thrombocytopenia 4