Underlying Causes of POTS
It is important to look for possible underlying causes of postural orthostatic tachycardia syndrome (POTS). Finding and treating those medical problems can allow better management of POTS symptoms better.
Common Comorbidities with POTS
Ehlers-Danlos Syndrome (EDS)
Eighty percent of people with Ehlers-Danlos Syndrome have POTS (but not the other way around). Most people with POTS have Type III EDS which is also called the hypermobility type. There is currently no known gene for this type of EDS, so diagnosis is made using family history and clinical evaluation. To meet the criteria for EDS Type III, patients must meet at least one of the major diagnostic criteria and two minor criteria.
- Joint hypermobility, which usually is confirmed by a score of five or more on the nine-point Beighton scale including:
- Soft or velvety skin with normal or slightly increased extensibility. A skin flap greater than 1.5 cm indicates increased extensibility.
- Absence of skin or soft tissue fragility, which is suggestive of other types of EDS.
- Molluscoid pseudotumors
- Surgical complications, such as incisional hernia, wound splitting, or sutures tearing through tissues and failing to hold.
- Family history of similar features without significant skin or soft tissue fragility in a pattern consistent with autosomal dominant inheritance
- Recurrent joint dislocations or subluxations
- Chronic joint or limb pain
- Easy bruising
- Functional bowel disorders (functional gastritis, irritable bowel syndrome)
- Neurally mediated hypotension or postural orthostatic tachycardia
- High, narrow palate
- Dental crowding
Mast Cell Activation Syndrome (MCAS)
Mast cell activation syndrome (MCAS) is also commonly associated with POTS and can present in a wide variety of ways. Mast cells are normally found throughout the body, and are most famous for their role in allergy and inflammation. In the case of mast cell activation syndrome, the mast cells are frequently releasing too much histamine. Symptoms of mast cell activation syndrome include:
- Recurrent abdominal pain
- Nasal congestion
- Chest tightness
Blood and urine tests can be run to confirm this diagnosis including blood tryptase levels and N-methyl histamine and prostaglandin D2 among others in a 24 hour urine sample. Allergists or immunologists are most likely to understand, diagnose, and treat MCAS.
Perhaps most importantly, people with mast cell activation syndrome will see an improvement in the above symptoms when they take a combination of Histamine-1 (Zyrtec, Benedryl) and Histamine-2 (Pepcid-AC) antagonists. Talk with your doctor about trying this regimen for a week to see if your symptoms improve.
Click here for scientific articles about mast cell activation syndrome.
Other Possible Underlying Causes of POTS
Here is a list of some common underlying causes for POTS.
- Adrenal deficiency - Addison's disease, adrenal fatigue
- Anemia, deficiency in folic acid
- Antiphospholipid Syndrome (Hughes Syndrome)
- Cervical stenosis
- Chiari malformation
- Chronic Fatigue Syndrome/Myalgic Encephalomyelitis
- Crohn’s Disease and Ulcerative Colitis
- Delta Storage Pool Deficiency
- Diabetes and Pre-Diabetes
- Ehlers-Danlos Syndrome
- Epstein Barr
- Mast Cell Activation Disorders
- Median Arcuate Ligament Syndrome
- Mitochondrial Diseases
- Nitric Oxide Deficiency
- Norepinephrine Transporter Deficiency
- Nutcracker Syndrome
- Sjogren’s Syndrome
- Thyroid Disease
- Tumors – pheochromocytoma, neuroblastoma, pelvic ganglioneuroma
- Physical Trauma, Surgery, Pregnancy
- Vitamin Deficiencies – B1, B3, B6, B9 and B12