Editor's Memo: Fibromyalgia: Time To Be a Secondary Diagnosis?
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.1 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.” So widespread has this condition and symptomatology become that the FDA has approved three agents for the management of fibromyalgia: milnacipran (Savella), pregabalin (Lyrica), and duloxetine (Cymbalta).
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.3,4 Hints of this became known in 1997 when fibromyalgia was noted to occur in high prevalence after neck trauma.5
Practical Pain Management published an article by Andrew Holman, MD, Associate Clinical Professor of Medicine, Division of Rheumatology at the University of Washington in Seattle, which noted that cervical myelopathy, specifically positional cervical cord compression (PC3), often overlap in patients with fibromyalgia.6 For those of us who treat and try to understand fibromyalgia, Dr. Holman’s recent article, “Why You Should Be Thinking About Positional Cervical Cord Compression,” is a must read.4 He provides the history, facts, and convincing evidence that PC3 is the cause of the majority of cases we call 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.4,5 Patients who have intermittent cervical cord compression may have a Chiari I malformation or a history of neck trauma, particularly from an auto accident.5
To help make the diagnosis, Dr. Holman relates that patients with cervical cord compression abhor cervical extension, such as being positioned in a hair dresser’s sink for any significant length of time or in a dentist’s chair looking at the stars or fireworks displays. Also, reading a computer screen over reading glasses or riding a non-recumbent bicycle will bring on symptoms. They may notice a variety of neurologic symptoms quite separate from the pain, such as fatigue and sleep disturbance. Asymmetric grip strength, poor balance (especially with eyes closed), numbness, tingling throughout the body, dizziness, and gait disturbances are common.4
It turns out that Dr. Holman isn’t the only party to recognize that fibromyalgia is secondary to a basic, underlying pain condition and not a primary disease.7-9 Perhaps the most vocal critic has been a young, bright, ahead-of-his-time rheumatologist from Orange County, California.8 To make his point, Jeff Sarkozi, MD, has cynically called fibromyalgia a “disease from nowhere.” Amid great criticism, he points out in his numerous writings that all of his fibromyalgia patients have easily discernible peripheral degeneration of tissue in the form of arthritis, tenosynovitis, bursitis, or fasciitis.
My Clinical Experience
In reviewing my own cases, which have been referred with a diagnosis of fibromyalgia, 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. In fact, patients and some practitioners become so obsessed with the symptoms of generalized pain, depression, sleep disturbance, and autonomic hyperactivity that fibromyalgia erroneously becomes the primary and not the secondary diagnosis. Our new understanding of centralized pain gives us the clue here.9 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. Perhaps it is overdue to also ask whether some other conditions, including vulvodynia, interstitial cystitis, prostatitis, irritable bowel, and myofascial syndrome are true brain diseases that arise “de novo” or did they really start in the periphery and are the result of central glial cell activation, inflammation, and sensitization.9