Background/Purpose

Joint hypermobility syndrome (JHS) is a chronically disabling disorder manifested as a widespread musculoskeletal pain and/or fatigue, in the presence of generalized joint hypermobility. It is often masquerading as Fibromyalgia or Chronic fatigue syndrome. It is a condition that is often overlooked by clinicians.

Methods

Currently in our clinic, we have observed 25 patients between 22 and 58 yr who were diagnosed with JHS using the Brighton Criteria (1). All of them presented with one or more of these symptoms: chronic fatigue, generalized musculoskeletal pain (arthralgias and myalgias), pre-syncope, palpitations, dizziness caring the diagnosis of fibromyalgia. They do not have any Marfanoid features or certain types of Ehlers-Danlos syndrome or skin involvement.       

Out of 25 patients 16 (64%) had a positive diagnostic tilt table test consistent with the diagnosis of postural orthostatic tachycardia syndrome (POTS), 6 (24%) have not had a tilt table test done yet (financial, non compliance, etc) and 3 (12%) had a negative tilt table test.

  

Patient	Age	Gender	*Symptoms of Autonomic dysfunction	Tilt table test
1	22	F	+	+
2	39	F	+	+
3	38	F	+	+
4	28	F	+	+
5	32	F	+	Not done
6	40	F	+	–
7	37	F	+	+
8	28	F	+	+
9	32	F	+	+
10	48	F	+	–
11	36	F	+	+
12	32	F	+	+
13	38	F	+	+
14	39	F	+	+
15	36	F	+	+
16	35	F	+	–
17	35	F	+	Not done
18	26	F	+	+
19	33	F	+	Not done
20	22	F	+	+
21	36	F	+	Not done
22	33	F	+	+
23	55	F	+	+
24	58	F	+	Not done
25	28	F	+	Not done

* Patient presented with Symptoms of Autonomic dysfunction like, orthostatic hypotension, palpitation, dizziness, passing out, fatigue.

Results

We have noticed that most of these patients are young female with a mean age of 35 with a presentation consistent with fibromyalgia or chronic fatigue syndrome, however further work up showed presence of autonomic dysfunction clinically and confirmed by tilt table test in 64%. By applying standard of care treatment for POTS with fludrocrotison, increasing water and salt intake, using Ted Hose stocking, they showed improvement in their clinical presentation. 

Conclusion

We conclude that there is a selective group of patients with JHS who have autonomic dysfunction which can contribute to the clinical presentations of these patients. These patients frequently are misdiagnosed as having Fibromyalgia or Chronic fatigue syndrome.

We have acronymed this entity “HyPOTS”.  

Potential manifestations of autonomic dysfunction include cardiac dysrhythmias, postural orthostatic tachycardia syndrome, orthostatic hypotension and orthostatic intolerance. Mechanisms leading to such phenomena in JHS patients may include weakened vascular tissue elasticity and impaired peripheral vasoregulation as a consequence of adrenoceptor or neuronal abnormalities (6, 7).

The pathophysiological basis for these symptoms needs to be further explored.

IRB approval for chart review was gained from University Hospital Case Medical Center.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/joint-hypermobility-syndrome-and-postural-orthostatic-tachycardia-syndrome-hypots/