IVIG for Neuromuscular Disorders

CIDP is an immune-mediated neuropathy in which damage to myelin, the protective sheath of the nerves, causes progressive weakness of the arms and legs over months or years, often with numbness and tingling that begin in both hands and feet. It is usually chronic and often requires long-term treatment.

Evaluation by a neurologist typically includes a neurological examination, including reflexes, nerve conduction studies with electromyography (EMG), and blood and urine tests.

Treatment options include corticosteroids, plasmapheresis (plasma exchange) and immune globulin. Therapies FDA-approved for adults with CIDP and dispensed by MedRx:

• Intravenous immune globulin: Gammagard Liquid, Gamunex-C, Panzyga, Privigen
• Subcutaneous immune globulin: Hizentra, as maintenance therapy to prevent relapse, started after intravenous immune globulin. See SCIG at home.
• Subcutaneous FcRn blocker: Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase)

GBS is an acute immune-mediated polyneuropathy. Weakness and abnormal sensations typically begin in the feet and legs and ascend to the arms and upper body over days to weeks. Like CIDP, it results from damage to myelin. Severe cases can cause respiratory compromise and paralysis requiring hospital care, including mechanical ventilation.

Plasmapheresis or IVIG is usually started promptly after diagnosis. IVIG is not FDA-approved for GBS; its use in GBS is off-label.

MMN is a rare immune-mediated neuropathy that affects motor nerves only. It causes weakness without sensory loss, typically beginning in the hands and often asymmetric.

Gammagard Liquid, given intravenously, is FDA-approved as maintenance therapy for adults with MMN.

Myasthenia gravis is a chronic autoimmune disease in which antibodies against receptors at the neuromuscular junction impair signal transmission from nerve to muscle. It causes fatigable weakness of voluntary muscles: weakness that worsens with activity and improves with rest. The ocular, facial and bulbar muscles (those used for eye movement, chewing, speaking and swallowing) are most often affected; respiratory, neck and limb muscles can be involved.

FDA-approved for generalized MG and dispensed by MedRx:

• Vyvgart (efgartigimod alfa-fcab), intravenous, and Vyvgart Hytrulo, subcutaneous, for adults
• Rystiggo (rozanolixizumab-noli), subcutaneous infusion, for adults who are anti-acetylcholine receptor (AChR) or anti-muscle-specific tyrosine kinase (MuSK) antibody positive
• Soliris (eculizumab), intravenous, for patients 6 years and older who are AChR antibody positive. Soliris is available only through an FDA-required REMS program, and MedRx is certified to dispense it.

IVIG is not FDA-approved for MG; its use in MG is off-label.

Dermatomyositis, an inflammatory myopathy, is covered on the page on autoimmune skin diseases.

1. The prescriber's office sends a referral: for IVIG or SCIG, the Immunoglobulin Referral Form (PDF), with recent clinical notes and laboratory results, by fax, or a call.
2. MedRx's intake team verifies insurance benefits and obtains prior authorization when the plan requires it.
3. Medication and supplies are delivered to the patient's door at no charge. Next-day delivery is available; timing varies by location.
4. A skilled, trusted and licensed nurse from an accredited nursing agency administers the infusion and monitors vital signs and adverse reactions; for SCIG, the nurse trains the patient or caregiver.
5. A MedRx pharmacist is available 24 hours a day, 7 days a week. MedRx's pharmacy team includes Ig Certified Pharmacists (IgCP), who hold a specialty certification in immune globulin therapy.

MedRx dispenses both on-label and off-label prescriptions written by the treating physician, who determines the therapy. See IVIG at home for more on home IVIG.

Health plans commonly require prior authorization for these therapies. MedRx's intake team verifies coverage and works with the prescriber's office on the documentation before the first delivery. Coverage of off-label use varies by plan.

Where out-of-pocket costs are a barrier, MedRx helps patients find and apply for copay assistance, grants and other financial aid. The manufacturers of these medicines also run patient support programs, named on each medication page.

• Immune globulin: Gamunex-C, Gammagard Liquid, Privigen, Panzyga, Hizentra and Octagam.
• Other medicines for myasthenia gravis: Vyvgart, Rystiggo and Soliris.

Each has its own page: what it is approved for, how it is given, and how to start. See every medication we fill.

Read more about home infusion nursing and biologic infusions at home.