Letter to the Editor (Submitted on September 2003, not accepted)

Journal of Rheumatology

Fibromialgia is not a diagnosis : Comment on the editorial by Crofford and

Clauw ¹ and the comment by Ehrlich

To the Editor:

I agree with G. Ehrlich ², that Fibromialgia is not a good diagnosis. My impression is that Fibromialgia includes patients with Psicogenic Rheumatism and patients with Benign Joint Hypermobility Syndrome (BJHS). Psicogenic Rheumatism patients describe their pain using words charged with a big emotional tone. For them “the pain is terrible”, “excruciating”, “incapacitating”, “difficult to bear”. They use expressions as “like something that erodes my flesh”, “like worms in my muscles”. The localization of the pain is vague and not precise, contrary to the well localized tender points. BJHS is usually undiagnosed in most parts of the world. To me, there is no coincidence that the “tender points sites” described for Fibromialgia, are exactly the areas of entesis, tendinitis and bursitis, which are recurrent problems in patients with BJHS. Fitzcharles ³ studied 76 patients referred to the rheumatologist as Fibromialgia. Of this only 29 had true Fibromialgia and the rest had other diagnoses, including 8 cases of probable BJHS.

I have been very interested in patients with BJHS for the last 3 years as they constitute 34% of my private rheumatological practice, in Santiago. Chile. I agree with R. Grahame ⁴, in London, that BJHS is probably the main cause of muscle skeletal pain, plus many other signs and symptoms derived from the fragility of tissues, due to an Hereditary Alteration of the Collagen Fibers. Rheumatologists, Orthopedic surgeons and Primary care physicians need to be aware of this condition (BJHS) and in order to make the diagnosis they need a high index of suspicion and to know and use the Brighton Criteria ⁵.

Further more, Chronic Fatigue related to depression as seen in Fibromialgia is a symptom noted by the patient upon awakening. The fatigue that many of these patients experience at mid-day or after standing or walking slowly, is most likely related to Disautonomia, secondary to BJHS. Here a vasovagal imbalance coupled with the genetic alteration of the collagen fibers of the veins, tends to produce Orthostatic Hypotension with fatigue, dizziness and even syncopal episodes. Most of these patients have cold intolerance and tendency to have chronically low blood pressure. Many have told me that they feel like "my batteries became discharged at midday" and they feel better after lying down. Many of them complain of swollen hands or feet, specially in AM or after long trips by plane. Disautonomia can easily be confirmed with a Tilt Test. I wonder how many "Chronic Fatigue patients with the diagnosis of Fibromialgia" would have a positive Tilt test, which would to me indicate that they have Disautonomia, probably secondary to BJHS and probably not Fibromialgia.

Barron has published a study ⁶ showing that joint hypermobility is more common in children with Chronic Fatigue Syndrome than healthy controls.

Interestingly enough in Spain, Bulbena ⁷ has related Anxiety to hyper-laxicity of joints. This is easy to understand, since these patients have poor quality of life, due to recurrent pain and lack of sympathy and understanding of their problems by their relatives and physicians . Because of this, they get multiple tests, see multiple physicians and at the end they are angry and frustrated . This sounds to me , very much like what we usually call Fibromialgia. As in Fibromialgia, in BJHS we also lack specific laboratory tests to confirm the clinical diagnosis, but BJHS can be diagnosed with certainty, when using the Brighton Criteria. The treatment, so far, is not too effective either.

BJHS appears to have a genetic basis, which Fibromialgia has not. The frequency of this condition has increased significantly lately, probably due to mutations of the genes. It is frequently associated with congenital malformations and is for this reason that we have proposed the hypothesis ⁸ that probably these mutations are caused by the lack of folic acid and/or the use of medicines that reduce folic acid (Triamterene, Sulfazalazine, anti-epileptic drugs, etc) during the peri-conception period and the first trimester of pregnancy. Is well known that the lack of folic acid interferes in the genesis of DNA. Methotrexate is effective by reducing folates and is also teratogenic.

I would suggest to either restrict the term Fibromialgia to patients with Psicogenic Rheumatism or drop the name altogether, because it appears to be inappropriate, when used with the present ACR criteria. In any case, I think rheumatologists need to make an effort to identify and diagnose the very frequent cases of BJHS.

References :

1.- Crofford LJ, Clauw DJ. Fibromialgia: Where are we a decade after the American College of Rheumatology classification criteria was developed? Arthritis Rheum 2002; 46: 1136-38

2.- Ehrlich GE. Fibromialgia is not a diagnosis: comment on the editorial by Crofford and Clauw. Arthritis Rheum 2003; 48 (1): 276; author reply 277.

3.- Fitzcharles MA, Boulos P. Inaccuracy in the diagnosis of Fibromialgia syndrome: analyses of referrals. Rheumatology (Oxford Feb; 42 (2): 263-7

4.- Grahame R. Editorial: Time to Take Hypermobility Seriously (in Adults and Children). Rheumatology 2001; 40: 485-491.

5.- Grahame R. Brighton Diagnosis Criteria for the Benign Joint Hypermobility Syndrome. Br J Rheumatol 2000 ; 27 : 1777-79.

6.- Barron, DF, Cohen BA, Geraghty MT, Violandr, Rowe PC. Joint hypermobility is more common in children with chronic fatigue syndrome than in healthy controls. J Pediatr 2002; 141:421-5 Sep 6.

7.- Bulbena A, Martin-Santos R. Laxitud articular y trastorno de angustia. Monografías de Psiquiatría 1994; 6: 20-25.

8.- Bravo JF, Arteaga MP, Coello L. Utility of bone cintigraphy in the study of Hereditary Disorders of the Connective Tissues (HDCT). Alasbim J 6(22): October 2003. http//www.alasbimnjournal.cl/revistas/22/bravo esp.html

Jaime F. Bravo, MD

Rheumatologist. Clínica Arauco. Santiago. Chile

Former-member of the Denver Arthritis Clinic. Denver. Colorado

Date: September 8, 2003

Fax: 56-2-206-1614

E-Mail address: Jaime.bravos@gmail.com