Teprotumumab, sold under the brand name Tepezza, is a medication used to treat adults with thyroid eye disease, a rare condition where the muscles and fatty tissues behind the eye become inflamed, causing the eyes to bulge outwards.The most common side effects are muscle spasm, nausea, hair loss, diarrhea, fatigue, high blood sugar, hearing loss, dry skin, altered sense of taste and headache. Teprotumumab should not be used if pregnant, and women of child-bearing potential should have their pregnancy status verified prior to beginning treatment and should be counseled on pregnancy prevention during treatment and for six months following the last dose.It is a human monoclonal antibody developed by Genmab and Roche for tumour treatment but was later researched by River Vision Development Corporation and Horizon Therapeutics to be used for ophthalmic uses. It binds to IGF-1R.Teprotumumab was approved for use in the United States in January 2020. The U.S. Food and Drug Administration (FDA) considers it to be a first-in-class medication. Medical use
In a multicenter randomized trial in patients with active Graves ophthalmopathy teprotumumab was more effective than placebo. In February 2019, Horizon announced results from a Phase III confirmatory trial evaluating teprotumumab for the treatment of active thyroid eye disease (TED). The study met its primary endpoint, showing more patients treated with teprotumumab compared with placebo had a meaningful improvement in proptosis, or bulging of the eye: 82.9 percent of teprotumumab patients compared to 9.5 percent of placebo patients achieved the primary endpoint of a 2 mm or more reduction in proptosis (p<0.001).
Paroxysmal hypertension is episodic and volatile high blood pressure, which may be due to stress of any sort, or from a pheochromocytoma, a type of tumor involving the adrenal medulla.Patients with paroxysmal hypertension who test negative for pheochromocytoma are said to be suffering from a clinical entity called "pseudopheochromocytoma." This disorder is due to episodic dopamine discharge and has been observed predominantly in hypertensive women whose presentation mimicked pheochromocytoma, but with subsequent testing being negative for malignancy. In patients with pseudopheochromocytoma, dopamine was found to be significantly increased post-paroxysm. The paroxysm is said to be similar to the hypertensive episodes described by Page in 1935, and has been colloquially referred to as "Pages Syndrome". These episodes can occur after diencephalic stimulation.The exact cause of pseudopheochromocytoma is unknown, though it is thought to be related to episodic dopamine discharge. External stressors that may contribute include pain, anxiety, or emotional trauma. Therefore, treatment of pseudopheochromocytoma is aimed at psychological support and intervention with antidepressants, but also treatment with alpha and then beta blockers in resistant cases. References
Further reading
Kuchel O, Buu NT, Larochelle P, Hamet P, Genest J (1986). "Episodic dopamine discharge in paroxysmal hypertension. Pages syndrome revisited". Archives of Internal Medicine. 146 (7): 1315–20. doi:10.1001/archinte.1986.00360190079011. PMID 3718127. Seck SM, Ka EF, Niang A, Diouf B (2009). "Pseudopheochromocytoma: An uncommon cause of malignant hypertension". Indian Journal of Nephrology. 19 (3): 122–4. doi:10.4103/0971-4065.57111. PMC 2859479. PMID 20436734. Mann SJ (2008). "Severe paroxysmal hypertension (pseudopheochromocytoma)". Current Hypertension Reports. 10 (1): 12–8. doi:10.1007/s11906-008-0005-2. PMID 18367021. S2CID 36584198.