Appeal type: Medical Necessity
Decision: Upheld Decision of Health Plan

Nature of Statutory Criteria/Case Summary: An enrollee has requested authorization and coverage for IVIG therapy.The submitted clinical documentation does not support that IVIG therapy  is medically necessary for the patient. There are no visit notes demonstrating how diagnoses of mast cell activation syndrome (MCAS), Ehlers-Danlos syndrome or postural hypotension syndrome (POTS) were made. All of the patients submitted mast cell studies were normal and the records fail to demonstrate evidence of mast cell activation syndrome. MCAS is now defined by robust and generally applicable criteria, including: 1) typical clinical symptoms; 2) a substantial transient increase in serum total tryptase level or an increase in other MC-derived mediators, such as histamine or prostaglandin D(2), or their urinary metabolites; and 3) a response of clinical symptoms to agents that attenuate the production or activities of MC mediators. In this case, there is no evidence of increase in serum tryptase, or any other criteria as noted above.The submitted records also fail to support a diagnosis of POTS. Some patients with POTS present with episodic flushing associated with surges in tachycardia, and have co-existent mast cell activation. They may have abnormal increases in urine methylhistamine (the primary urinary metabolite of histamine) 13, which should ideally be measured from a 4 hour sample at the time of a flushing episode (not a 24 hour sample). This can be associated with dyspnea, headache, lightheadedness, excessive diuresis, and gastrointestinal symptoms such as diarrhea, nausea, and vomiting. These patients often have a hyperadrenergic response to posture, with both orthostatic tachycardia and hypertension. There are many triggers for the flushing, including prolonged standing, exercise, premenstrual cycle, meals, and sexual intercourse. In this case, the patient has a stated history of postural orthostatic hypotension, but the submitted records fail to demonstrate clinical findings to support this diagnosis. There is no discussion of previous therapies to treat POTS or failure of first-line therapies. First-line therapy would include exercise training and increasing plasma volume. Medication therapy that includes IVIG is not first-line therapy for this disorder. The records fail to demonstrate that the patient has failed first-line POTS therapy. Furthermore, while there is a case report of successful treatment with IVIG therapy in a patient with POTS and MCAS, IVIG is not a current recommended therapy for MCAS. Under these circumstances, the requested IVIG therapy is not medically necessary to treat the patients medical condition.Final Result: The reviewer determined that the requested IVIG therapy is not medically necessary for treatment of the patients medical condition. Therefore, the Health Plans denial should be upheld.Credentials/Qualifications: The reviewer is board certified in internal medicine with subspecialty certifications in allergy and immunology and rheumatology, and is actively practicing. The reviewer is an expert in the treatment of the enrollees medical condition and knowledgeable about the proposed treatment through recent or current actual clinical experience treating those with the same or a similar medical condition.