Hyperpots?

[KellyA]

I have lupus and relapsing polychondritis, Sjogrens, raynauds, large fiber neuropathy, and some stuff we're still trying to figure out. AI stuff is managed, but not controlled, with flares about every 6 months. From reading articles and talking to others, I think I have hyperpots and likely MCAS. I'm on bystolic for my BP and HR, which is working pretty well. I was on a Ca channel blocker for over a year with my first cardiologist. It didn't do a thing, and he refused to change it. Said we needed to address the AI stuff, and he didn't mess with that. Love my new cardio!

Anyway, I've had high BP when standing for about a year and a half, since my worst flare. Always high HR, that rises >30 on standing. Was about 135/90-85 supine, 160/110-125 standing before meds, now about 120/70-70 supine and 135/85-90 standing. I also have night sweats, chills/cold sweats, cold/clammy hands and feet, pooling on standing (legs get darker), tremors in my hands, flushing, GI issues (especially after eating), and probably some others I'm forgetting. Docs like to blame everything on my weight and meds, especially prednisone. Though shrug when I tell them I have had the same symptoms with a 40 pound range, on/off and higher/lower doses of meds.

I had autonomic testing on Friday and I'm not sure what to think. They were all normal, except the valsalva maneuver, which was lower than normal. He said he was surprised because of my logs and symptoms, but now doesn't think I have POTS. My norepinephrine levels were 590 supine and 1150 standing. He said that all he looks at is the percentage it rises; he says doubling on standing is normal. From everything I've read, both my numbers are high enough to strongly suggest hyperPOTS. What is the current, official line on diagnosis? I also had an abnormal swallow study that I'm waiting to hear the final interpretation of from my GI (liquids and pills get hung up in my esophagus, but not chewed foods or pudding, worse with cold liquids. No obvious strictures, and I think higher than the entrance to the stomach where you would think achalasia, maybe 3/4 down the esophagus). He said the valsalva maneuver indicated an issue with the vagus nerve, which might also be the cause of my GI problems. I'm also wondering if, although he said I have essential tremors and a family history (though much older), if maybe it's actually the high NE levels making my hands shake. He put me on propanolol and it didn't do a thing.

Anyway, what are your thoughts? I'm in Washington state, and my neurologist is one I found on both this site and Dysautonomia International. I'm just worried about discounting what all my symptoms and logs have pointed to--hyperpots, because of 2 hours of one day. I was off a bunch of my meds, but maybe not long enough? It was freezing in the room, could that have influenced any of the test with my raynauds? Also, what are the differences in accuracy between the TTT, which is at 70°, and truly standing while testing? And how important is it to diagnose hypovolemia? Is there really any treatment?

I'm glad I found this group! I've learned a lot from reading your posts!

[RecipeForDisaster]

I just want to tell you that I reached a diagnosis "dead end" because of a "normal" TTT as well. I did not have norepinephrine measured and there is zero detail in my results, only one BP measurement for instance! I also think that the stress and rush of getting to the city, parking, finding the place, etc. made my numbers look better. I always have higher BP when stressed and I do know that my BP was under 90 for a good portion of the test anyway. I was feeling faint but didn't pass out. Apparently that is okay?

I am seeing three new doctors in the next two months and don't know what to expect. Honestly, I don't have a notion of what I think I have, I just know something is definitely wrong and it really seems autonomic. You are not alone!

[kalamazoo]

I messaged you!

[haugr]

590 is a really high for a supine norepinephrine measurement.  Have you by chance been tested for a pheochromocytoma?

[KellyA]

Thanks for letting me know I'm not alone. He said the NE test and the other catecholamines was to rule that out. From him and my reading, I don't have it because everything else was negative, and I think with pheo the NE are more like quadruple on standing or more.

[SarahA33]

Hi Kelly - Welcome to the forum! Maybe a second opinion is needed? Here is the diagnostic criteria from a recent paper by Dr. Raj, a POTS specialist -

 

Table 1

Diagnostic criteria for postural tachycardia syndrome

• Increase in heart rate ≥30 bpm within 10 min of upright posture (tilt test or stand) from a supine position

◦ Heart rate increase must be >40 bpm in patients <20 years

• Associated with a constellation of symptoms that are worse with upright posture and that improve with recumbence

• Chronic symptoms that have lasted for >6 months

• In the absence of other disorders or medications that are known to predispose to orthostatic tachycardia Central Hyperadrenergic POTS Although the elevated sympathetic tone in POTS is often secondary to another pathophysiological mechanism, such as neuropathy or hypovolemia, it can also be the primary underlying problem [19]. Furlan et al. reported increased sympathetic tone in patients with POTS [52]. Resting sympathetic nerve activity, measured by microneurography, is elevated. A spectral analysis index of sympathetic function and plasma norepinephrine are sometimes higher at supine rest and increase more with HUT in POTS [52, 53]. Plasma norepinephrine levels should be determined in patients with POTS while in steady state in the supine and upright positions (at least 10 min in each position). When measured during upright posture, norepinephrine is elevated in many patients with POTS (>3.55 nmol/L (600 pg/mL) and sometimes >5.91 nmol/L (1000 pg/mL)), consistent with the sympathetic neuronal activation elicited by standing in these patients [19]. This is sometimes used as a criterion for the “hyperadrenergic subtype” of POTS. An exaggerated sympathetic vasoconstrictor response during the recovery and overshoot phases of the Valsalva maneuver may also reflect a hyperadrenergic state in patients with POTS [2, 19]. These patients may have orthostatic hypertension (increase in systolic blood pressure >10 mmHg on standing) and complain of tremor, anxiety, and cold, sweaty extremities