Name that syndrome: JHS or EDS-HM?
I think joint hypermobility syndrome is actually Ehlers-Danlos syndrome-hypermobile type. ("Joint Hypermobility Syndrome Underdiagnosed," July 1, 2006, p. 28).This condition has been around since 1946, defined by doctors such as Peter Byers, Victor McKusick, and Peter Beighton (yes, the same doctor who created the "Beighton score" mentioned in your article). Its nosology was redefined in 1997 at the Villefranche conference.
There actually is no such thing JHS. I know that this is a controversial statement. I invite your readers to visit the Ehlers-Danlos National Foundation Web site, www.ednf.org, and to review the hypermobile type classification. They will quickly note that the description in your article is actually EDS and not JHS.
Mark E. Lavallee, M.D.
South Bend, Ind.
Dr. Rodney Grahame replies:
Dr. Lavallee's letter is both refreshing and pertinent. Most clinicians in the field would certainly agree that JHS and Ehlers-Danlos syndrome-hypermobility type (EDS-HM, formerly known as EDS type III) are, for all intents and purposes, one and the same. The only problem is that until the genetic cause of each condition is established, we can only surmise.
As to how two seemingly identical conditions could assume such different-sounding names, EDS-HM originated from a clinical genetic perspective, whereas JHS developed from a rheumatologic background.
It is not correct to say that JHS does not exist. It has been well established in the rheumatologic literature for nearly 40 years, although evidence of its preeminent importance as a source of morbidity has only very recently been accumulating. It is defined by a set of validated criteria, termed the Brighton criteria.
The choice of name is of more than academic interest. The use of EDS-HM sometimes alarms patients, but it makes doctors sit up and take notice. The term JHS is sometimes interpreted as referring to a trivial disorder, which some doctors dismiss, to their patients' dismay.
The name used for this syndrome has pragmatic implications, too. In Denmark, for example, patients with a diagnosis of EDS-HM are eligible for Social Security benefits, but those labeled has having JHS are not.
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| Title Annotation: | LETTERS; joint hypermobility syndrome; Ehlers-Danlos syndrome-hypermobility type |
|---|---|
| Publication: | Internal Medicine News |
| Article Type: | Letter to the editor |
| Geographic Code: | 1USA |
| Date: | Aug 1, 2006 |
| Words: | 400 |
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