Author Topic: Is Fibromyalgi Really Ehlers Danlos Syndrome (EDS)  (Read 3139 times)


Ryan Ryder

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When to Suspect (EDS - document for patients)
« Reply #1 on: August 08, 2015, 07:31:15 PM »
When to Suspect

(EDS - document for patients)

"Fibromyalgia (FM). Many patients diagnosed as having Fibromyalgia really have JHS, since many symptoms are very similar, with recurrent pain, “trigger points”, chronic fatigue and with normal laboratory tests. It is my opinion that FM is part of the JHS disease, since all patients with FM that I see fulfill the Brighton criteria that is diagnostic for JHS. Wed call these pains “fibromyalgic pains of JHS”

-Dr. Bravo

Dr Bravo's When To Suspect document:

http://oreds.org/uploads/3/1/5/0/3150381/drbravowhentosuspect.pdf

-RR
« Last Edit: August 22, 2015, 02:29:53 AM by Ryan Ryder »

Emmerson Elliot

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Re: Is Fibromyalgi Really Ehlers Danlos Syndrome (EDS)
« Reply #2 on: August 21, 2015, 11:37:20 PM »
Are you able to find and post that French translation here please Ryan?

and other articles on EDS/ Fibro cross-diagnosis if you find them please?

They are very important

thanks
Emmerson

Ryan Ryder

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Fibromyalgia: an unrecognized Ehlers-Danlos syndrome hypermobile type?
« Reply #3 on: August 22, 2015, 01:49:17 AM »

Fibromyalgia: an unrecognized Ehlers-Danlos syndrome hypermobile type?


FIBROMYALGIE : un syndrome d’Ehlers-Danlos de type hypermobile qui s’ignore ?

"Fibromyalgia: a hypermobile form of EDS we ignore/miss?


Hermanns-Lê T1, Piérard GE, Angenot P.

Rev Med Liège

January 2013

FULL article text in FRENCH
(English translation has been completed.)

.........................................
Fibromyalgia: an unrecognized Ehlers-Danlos syndrome hypermobile type?

Hermanns-Lê T1, Piérard GE, Angenot P.

Rev Med Liège

January 2013

Abstract (in English)

Some patients suffering from fibromyalgia present with clinical signs and alterations in the histopathology, immunohistochemistry and ultrastructure of the dermis similar to the Ehlers-Danlos syndrome, hypermobile type (EDSH). Some types of fibromyalgia possibly represent an undiagnosed EDSH.

PMID: 23444824 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed/23444824

................................................


English Translation Aug 8, 2015

Fibromyalgia: an unrecognized Ehlers-Danlos syndrome hypermobile type?

Hermanns-Lê T1, Piérard GE, Angenot P.

Rev Med Liège

January 2013

Introduction

Fibromyalgia is characterized by chronic muscle and joint pain, associated with fatigue, anxiety and depression as well as by problems with cognition, sleep and digestion (1,2). It’s found especially among young and middle-aged women. Diagnosis depends on a group of precise pain (tender) points (PD) that respond to finger pressure (11/18 points required minimum), and more recently diagnosis is comprised equally of a severity score of symptoms (SS) including fatigue, trouble waking in the morning, and cognitive trouble (at least 7/18 PD, 5/9 SS, or 3-6/18 PD and 9 SS). The etiology (cause) of fibromyalgia remains unknown to this day.

The Hypermobile Type Ehlers-Danlos Syndrome (HEDS or SEDH in French) is characterized principally by ligamentous laxity (a Beighton score of > 5/9) and skin that is velvety or somewhat stretchy. One of these 2 criteria suffices to confirm the diagnosis according to the Villefranche nosology (3), which defines the major and minor criteria of EDS and its different types (4,5). SEDH (HEDS) is complicated often by joint complaints, poly-algia, frequent bruising, fatigue and proprioceptive and digestive troubles. It presents as a genetic connective tissue disorder, of which the extra-articular anomalies are well known but the mutations are poorly known.

Patients and Methods

Between August 2008 and July 2012, we examined 52 fibromyalgia patients, some of whom had been suffering for 20 years. They were seen by Physiotherapists and neurologists for an EDS study. The majority (49/52) were women. Their ages ranged from 15 to 63 years. Upon clinical examination, ligamentous laxity was present and pain severity was variable at the time of the exams. The Beighton Score ranged from 0 to 7 but, in the majority of cases, the score was 4 or 5/9, the minimum for a typical EDS (form). However, we found a velvety or moderately extensible (stretchy) skin and a number of other secondary criteria. Often, personal and family history revealed join laxity or problems with sprains or subluxations and easy bruising.

A skin biopsy was performed on an area protected from the sun, armpit or buttock to exclude changes in connective tissue from sun exposure. Part of the biopsy was for histopathological exam of connective tissue of the skin and dermal dendrocytes type 1 (DD-1), marked by anti-Factor XIIIa antibody (6). Another part was reserved for ultrastructural study under transmission electron microscope.

Ultrasctructural Observations

Half of the cases presented dermatopathologic changes to the collagen and/or elastic fibers and a decrease in the number of DD-1, which were often hypoplastic. Ultrastructural examination showed in all cases, abnormalities of connective tissues common to EDS: collagen fibril bundles of variable diameter, spaced irregularly. Other fibrils had a circular or flower-shaped cross-section (fig. 1). They were sometimes twisted along their longitudinal axis (length), or arranged in swirled plies. The elastic fibers had a jagged outline to their aspect, presence of micro-calcifications, and an increase in osmophilic elements along the amorphous (extracellular? JG) matrix.

Conclusion

A recruitment bias is possible in our study, since our patients were preselected and seen by an EDS study. There are however numerous symptoms common to HEDS and FM. These consist of polyalgias, fatigue and proprioceptive problems… Joint hypermobility is reported in FM (1). In several recent studies conducted at FM patient homes, nearly 46.6 – 64.2 % of patients presented with hypermobile joints, versus 22-28.8 % in the control groups, characterized by a significantly lower Beighton score (7-9).

In our series of FM patients, some presented a Beighton score insufficient to evoke a type of EDS, but adequate for Benign Joint Hypermobility Syndrome (BJHS) (10). We share the opinion of some authors that consider BJHS to really be another form of HEDS (11-15). Further, joint hypermobility can be masked by pain, arthritis or the sequelae from prior surgery. According to these arguments, the relationship between FM and HEDS is questionable. Further studies should be conducted to explore this hypothesis.

Recognition of structural changes of collagen fibrils is not a definitive diagnosis criterion. These anomalies rather indicate a kinship indicator between diseases that have a common impact or overlap with fibrillogensis of collagen. It should be immediately noted that to date genetics remains non-contributory and the complexity of reported anomalies in different types of EDS is quite large. Every clinical form can be in effect associated with several diverse genetic anomalies.

In conclusion, certain dogmatic aspects of the nosology may not stand up to scrutiny and so are likely to be amended in coming years. Expect conceptual changes that will lead to better care for patients. In this example discussed here, consider how the FM patient presenting first to a psychiatrist with various subjective complaints, or (variously) to a pathology lab, likely genetic, can have a profound impact on the perception of the disease by the patient and their team.

1. Wolfe F.— The American College of Rheumatology preliminary diagnostic criteria for fibromyalgia and measurement of symptoms severity. Arthritis Care Res, 2010, 62, 600-610.
2. Jahan F, Nanji K, Qidwai W, Qasim R.— Fibromyalgia syndrome: an overview of pathophysiology, diagnosis and management. Oman Med J, 2012, 27, 192-195.
3. Beighton P, De Paepe A, Steinmann B, et al.— EhlersDanlos syndrome : revised nosology, Villefranche, 1997. Am J Med Genet, 1998, 77, 31-37.
4. Hermanns-Lê T, Piérard GE.— Le syndrome d’EhlersDanlos. Un centenaire revisité. Skin, 2000, 3, 138-141.
5. Hermanns-Lê T, Piérard GE.— Ultrastructural alterations of elastic fibres and other dermal components in Ehlers-Danlos syndrome of the hypermobile type. Am J Dermatopathol, 2007, 29, 370-373.
6. Hermanns-Lê T, Piérard GE.— Factor XIIIa-positive dendrocyte rarefaction in EHlers-Danlos syndrome classic type. Am J Dermatopathol, 2001, 23, 427-430.
7. Ofluoglu D, Gunduz OH, Kul-Panza E, Guven Z.— Hypermobility in women with fibromyalgia syndrome. Clin Rheumatol, 2006, 25, 291-293.
8. Sendur OF, Gurer G, Bozbas GT.— The frequency of hypermobility and its relationship with clinical findings of fibromyalgia patients. Clin Rheumatol, 2007, 26, 485-487.
9. Ting TV, Hashkes PJ, Schikler K, et al.— The role of benign joint hypermobility in the pain experience in juvenile fibromyalgia : an observational study. Pediatr Rheumatol Online J, 2012, Epub June 15.
10. Simpson MR.— Benign joint hypermobility syndrome : evaluation, diagnosis, and management. J Am Osteopath Assoc, 2006, 106, 531-536.
11. Graham R.— Joint hypermobility and genetic collagen disorders : are they related? Arch Dis Child, 1999, 80, 188-191.
12 Graham R. — Heritable disorders of connective tissue. Balliere’s Clin Rheumatol, 2000, 14, 345-361.
13. Adib N, Davies K, Graham R, et al.— Joint hypermobility syndrome in childhood. A not so benign multisystem disorder? Rheumatology (Oxford), 2005, 44, 744-750.
14. Zweers MC, Kucharekova M, Schalkwijck J.— Tenascin-X: a candidate gene for begnin joint hypermobility syndrome and hypermobility type Ehlers-Danlos syndrome? Ann Rheum Dis, 2005, 64, 504-505.
15. Hermanns-Lê T, Reginster MA, Piérard-Franchimont C, et al.— Dermal ultrastructure in low Beighton score memebers of 17 families with hypermobile-type Ehlers-Danlos syndrome. J Biomed Biotechnol, 2012, 878107.

-RR
« Last Edit: August 22, 2015, 02:09:07 AM by Ryan Ryder »

Ryan Ryder

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Hypermobility in women with fibromyalgia syndrome
« Reply #4 on: August 22, 2015, 01:52:58 AM »
Hypermobility in women with fibromyalgia syndrome

Demet Ofluoglu
Osman Hakan Gunduz
Evren Kul-Panza
Zeynep Guven

Clinical Rheumatology (Impact Factor: 1.77

06/2006

ABSTRACT

The purpose of this study was to evaluate the relationship between hypermobility and fibromyalgia syndrome (FS) in women. Ninety-three women with FS who met the American College of Rheumatology criteria for FS and 58 healthy women without FS were included in this study. All women were examined for hypermobility by blinded observers using the Beighton criteria. The mean age was 43.5+/-9.9 (21-68) and 40.2+/-11.1 (21-63) years in the FS and control groups, respectively, and the two groups were statistically similar (p>0.05). The mean Beighton total score was 4.7+/-2.1 and 2.9+/-2.4 in the FS and control groups, respectively (p<0.0001). The frequency of joint hypermobility was 64.2% in the FS group and 22% in the control group. In accordance with the Beighton criteria (p<0.05), we found that the joint hypermobility ratio was significantly higher in patients with FS than in subjects without FS. Additionally, we evaluated the correlation between the total Beighton score and the age and number of trigger points. There were negative correlations between the total Beighton score and the age (r=-0.42, p<0.001) and number of trigger points (r=-0.24, p=0.03) in all patients. Hypermobility syndrome is more common in women with FS than in those in the control group. Therefore, the relationship between hypermobility and FS should be taken into consideration in the diagnosis and follow-up of women, especially those with widespread pain.

http://www.researchgate.net/publication/7537343_Hypermobility_in_women_with_fibromyalgia_syndrome

-RR
« Last Edit: August 22, 2015, 02:30:20 AM by Ryan Ryder »

Ryan Ryder

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Fibromyalgia - A Perspective on Muscle Dysfunction and Effective Treatments
« Reply #5 on: August 22, 2015, 02:05:23 AM »
Fibromyalgia - A Perspective on Muscle Dysfunction and Effective Treatments

Fibro Friends (blog)

A patient authored resource on Chronic Fatigue and Fibromyalgia Syndromes and effective treatments.

October 19, 2011

Dr. Byron Hyde, one of the premier medical doctors and researchers on chronic fatigue syndrome and related disorders defines fibromyalgia as "a pathological hyper-elasticity of the peripheral vascular system or anything that causes this pathological expansion". A subset of people with fibromyalgia have an excessive amount of elasticity in their connective tissue due to a genetic disorder called Ehlers-Danlos Syndrome that manifests itself in lax or hypermobile joints, ligaments and skin. For the majority of people with fibromyalgia hyper-elasticity is caused by dysautonmia - a term describing malfunction of the autonomic nervous system that controls heart rate, blood pressure, digestive tact peristalsis, and most importantly blood volume, which is typically low.

http://fibrofriends.typepad.com/fibro_friends/2011/10/f.html

-RR
« Last Edit: August 22, 2015, 02:11:14 AM by Ryan Ryder »

Ryan Ryder

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Ehlers Danlos Syndrom
« Reply #6 on: August 22, 2015, 02:19:23 AM »
Ehlers Danlos  Syndrome

NeuroImmune Alliance


"World-renowned EDS expert, rheumatologist Professor Rodney Grahame (University College London) points out that, in America, almost 650,000 cases of Ehlers-Danlos Syndrome (EDS) are missed ANNUALLY, based on studies that suggest almost 95% of cases presenting to clinics are missed, most often diagnosed with other things (Fibro/ME/csf, POTS/OI etc.)."

http://www.neuroimmunealliance.org/ehlers-danlos-syndrome.php

-RR

Ryan Ryder

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Keynote: Hypermobility, Fibromyalgia, and Chronic Pain
« Reply #7 on: August 22, 2015, 02:51:02 AM »
Keynote: Hypermobility, Fibromyalgia, and Chronic Pain

Rodney Grahame, CBE, MD, FRCP, FACP

EDNF 2012 Conference Cincinnati

August 2012

Objectives:

1. Demonstrate the connection between hypermobility, fibromyalgia, and
chronic pain in EDS patients.

2. Discuss intervention methods.


Link to Section Objectives pdf:

http://ednf.org/sites/default/files/Grahame_Keynote_ObjectivesCME.pdf


Link to Slide Show Presentation:

http://ednf.org/sites/default/files/GRAHAME_R_EDNF_2012_V5.pdf


Link to 2012 Annual Conference:

http://www.ednf.org/2012-annual-conference#annual_conference-page_1-5

-RR


Ryan Ryder

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Fibromyalgia: Time To Be a Secondary Diagnosis?
« Reply #8 on: August 23, 2015, 03:50:29 PM »
Fibromyalgia: Time To Be a Secondary Diagnosis?

Blog:

EDS Info (Ehlers-Danlos Syndrome)
Research and articles related to living with Chronic Pain from EDS & Fibromyalgia

October 9, 2014

..........

Original Article:

Editor's Memo: Fibromyalgia: Time To Be a Secondary Diagnosis?

By Forest Tennant, MD, DrPH

Practical Pain Management

First published on:

August 1, 2013

............

Blog Post:

This article presents a novel theory: “The most common cause of fibromyalgia is probably positional cervical cord compression.”

Fibromyalgia has undergone a most interesting metamorphosis over the past 30 years. Initially, most of us believed it to be a psychological phenomenon with no organic basis.

The first transition was when it became recognized as a myofacial disease with trigger points (fibrositis). In 1990, the American College of Rheumatology Research Classification Criteria (ACR RCC) for fibromyalgia was published. The criteria required two components for fibromyalgia: a patient’s history of chronic widespread pain for at least 3 months, and ≥11 of 18 painfully tender soft tissue sites on physical examination

Late in the 1990s, the concept of a central, autonomic hyperfunctional state with neurotransmitter deficiencies and lack of restorative sleep were proposed as the causative factors. The resulting hyperactive autonomic nervous system manifested as tachycardia, hyperthermia, fatigue, depression, and pain “all over” as opposed to specific trigger points. This led to a revision of ACR RCC criteria for fibromyalgia, resulting in the 2010 ACR Fibromyalgia Diagnostic Criteria (2010 ACR FDC).2 The 2010 ACR FDC viewed the 1990 ACR RCC as the gold standard, but did not require tender-point examination.

Without the requirement of specific trigger points, it seems that just about every patient with generalized pain, insomnia, and depression is now given the diagnosis of “fibromyalgia.”

In my opinion, the latest (and most likely) final chapter in the fibromyalgia odyssey is that it is not a primary disease at all, but a secondary or symptomatic manifestation of an underlying, causative disorder. I believe, primarily based on my own clinical experience and the excellent work of the National Fibromyalgia Research Association and the National Fibromyalgia Partnership, Inc., that it is time to recognize fibromyalgia as being secondary to an inciting or underlying disease process.

Recent efforts by these organizations clearly call for recognition that the most common cause of fibromyalgia is probably positional cervical cord compression. Hints of this became known in 1997 when fibromyalgia was noted to occur in high prevalence after neck trauma.5

cervical myelopathy, specifically positional cervical cord compression (PC3), often overlap in patients with fibromyalgia.

The basic premise is that the cervical spinal cord is compressed when patients extend their neck either in a forward flexion or a backward extension position. A spinal cord defect, which may compress the cord in only some positions, may not be seen on standard magnetic resonance imaging (MRI). Proper diagnosis requires that additional dynamic MRI pictures be taken with the neck flexed forward and extended backward. A considerable amount of MRIs and clinical experience now document that repeated cervical neck compression, even though the cord is not damaged, may cause all the manifestations of fibromyalgia including diffuse pain, autonomic arousal, disordered sleep, depression, fatigue, and neurotransmitter changes. Patients who have intermittent cervical cord compression may have a Chiari I malformation or a history of neck trauma, particularly from an auto accident.

The Chiari malformation is typical of EDS, so this theory implies that Fibromyalgia could be caused by EDS, which has long been my suspicion. Any imbalance in function or overload of the body’s systems causes stress that wears down the body’s ability to adapt.  This breakdown of balance could then eventually result in Fibromyalgia symptoms.

My Clinical Experience

I have found that more than two-thirds of patients appear to have a history of cervical neck trauma or the compression-related symptoms described by Dr. Holman when patients extend their neck. I now realize that I have been quite unaware of the critical nature of PC3. After taking detailed histories, I have found that all my other fibromyalgia cases concomitantly started with an infectious disease, usually infectious mononucleosis, head trauma, endometriosis, or a peripheral nerve injury or degenerative process. In these cases, there is always clear evidence of autoimmunity, systemic inflammation, and pituitary-adrenal-gonadal hormone changes.

It is clear to me that any peripheral pain site that can centralize can develop the classic symptoms of fibromyalgia. For my part, I also believe “myofascial syndrome” is in the same boat.

Our new understanding of centralized pain gives us the clue here. Prior to knowing that a painful, peripheral nerve injury and locus can incite glial cell activation, inflammation, central sensitization, and autonomic hyperactivity, it is no wonder that fibromyalgia was initially believed to arise “de novo” in the brain.

This would explain why so many people with EDS are first diagnosed with Fibromyalgia.  Fibromyalgia is often the initial explanation for the widespread pain and fatigue of EDS, and then the EDS diagnosis is found much later – but only in patients who continue searching for a more specific diagnosis, those who suspect their own symptoms don’t quite match those of Fibromyalgia.

Fibromyalgia is only a “syndrome”, a cluster of symptoms without a known cause.  Who knows how many people who are currently diagnosed with it may also have EDS.  An EDS diagnosis doesn’t lead to a cure either, but knowing you have it can help you figure out how to minimize the pain better.

Link to blog post:

https://edsinfo.wordpress.com/2014/10/09/fibromyalgia-time-to-be-a-secondary-diagnosis/

Link to original article:

http://www.practicalpainmanagement.com/pain/editors-memo-fibromyalgia-time-be-secondary-diagnosis

-RR

Ryan Ryder

  • Guest
Skin Ultrastructural similarities between Fibromyalgia and Ehlers-Danlos
Syndrome Hypermobility Type

Published date: January 27, 2016

J Ost Arth 2016

Hermanns-Le T1and Pierard GE 2

Abstract
Fibromyalgia (FM) and hypermobility type Ehlers-Danlos syndrome (EDSH) share a series of common clinical
signs. A clinical distinction between both diseases is occasionally difficult to be established. The physical changes
observed in the mechanical properties of skin and joints do not distinctly distinguish these disorders. In addition,
similar ultrastructural dermal changes are observed in both EDSH and some FM cases. The molecular alterations
remain largely undisclosed in both diseases. As a result, EDSH remains undiagnosed in some FM patients. This
condition deteriorates quality of life and possibly leads to prominent health problems. Key words:
Fibromyalgia; Ehlers-Danlos syndrome hypermobility type; Skin
ultrastructure; Joint hypermobility syndrome; Connective tissue
disorder

http://www.omicsonline.org/open-access/skin-ultrastructural-similarities-between-fibromyalgia-and-ehlersdanlossyndrome-hypermobility-type-joas-1000104.pdf