Reprint Article - Chronic Fatigued Often Paired With Ehlers-Danlos Syndrome

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Chronic Fatigued Often Paired With Ehlers-Danlos Syndrome

Bruce Jancin, Denver Bureau

[Pediatric News 33(3):40, 1999. © 1999 International Medical News Group.]

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Denver -- Chronic fatigue syndrome appears to be common among patients with

Ehlers-Danlos syndrome, a connective tissue disorder characterized by joint

hypermobility, hyperelastic skin, and connective tissue fragility.

Orthostatic intolerance is seen in both conditions, presumably because

connective tissue abnormalities predispose to excessive venous pooling

during upright posture. This explanation makes the observed association

biologically plausible, Dr. T. Geraghty said at the annual meeting

of the American Society of Human Genetics.

According to other investigators, as many as one in nine people may have a

mild variant of the connective tissue disorder. Other manifestations of the

disorder may include acrocyanosis when limbs are in a dependent position,

wide papyraceous scarring over bony prominences, ocular lesions, easy

bruisability, and other vascular abnormalities.

It's too early to say how often the two conditions coexist, however, said

Dr. Geraghty of s Hopkins University, Baltimore.

Dual-diagnosis patients may obtain symptomatic improvement using medications

that address orthostatic intolerance by reducing venous pooling. Moreover,

establishing the diagnosis of Ehlers-Danlos syndrome has added benefits,

including a heightened awareness of potentially serious complications,

improved detection of affected relatives, and avoidance of unnecessary

diagnostic and surgical procedures.

Dr. Geraghty reported on 10 patients averaging 16 years of age who were

evaluated both in the Hopkins chronic fatigue syndrome clinic and the

genetics clinic. All met prevailing diagnostic criteria for both disorders.

Diagnosis of chronic fatigue syndrome preceded that of Ehlers-Danlos by a

median of 42 months in 9 of 10 patients.

The medical literature has historically underemphasized the easy

fatiguability of patients with Ehlers-Danlos, as well as their common

complaints of widespread muscle and joint pain. Indeed, all 10 patients in

this series complained of varying degrees of pelvic, joint, and limb pain in

the absence of articular swelling or erythema. Nine had a history of

recurrent joint dislocations, requiring surgery in three cases, Dr. Geraghty

noted.

All patients had abnormal results on orthostatic testing. Based upon the

results of an upright tilt-table test or a 10-minute standing test, eight

patients had postural tachycardia syndrome, six had neurally mediated

hypotension, and four had both.

Several prior studies in patients with chronic fatigue syndrome have shown

application of military antishock trousers results in symptomatic

improvement, with prevention of orthostatic tachycardia and hypotension.

It's logical that symptoms of chronic fatigue and orthostatic intolerance in

patients with Ehlers-Danlos syndrome might respond to vasoconstrictors that

reduce gravitational venous pooling, but this hypothesis awaits testing in

randomized, controlled trials.

Before using these agents in patients with Ehlers-Danlos syndrome, however,

the presence of an enlarged aortic root should be ruled out. It's not a

common abnormality -- it was present in 1 of 10 patients in this series --

but it predisposes to a feared complication of the connective tissue

disorder: dissecting aortic aneurysm, he said.