The Fibromyalgia Spectrum - Part of the Big Picture in Understanding Fibromyalgia by Mark J. Pellegrino, MD 08-04-2007
Today I’m convinced Fibromyalgia is indeed a broader condition with various subsets - and a Fibromyalgia Spectrum model is helpful in organizing and educating patients, writes Dr. Mark J. Pellegrino, MD, a Fibromyalgia expert specialized in Physical Medicine and Rehabilitation.
This article - excerpted with permission from Dr. Pellegrino’s highly praised and reader-friendly book, Fibromyalgia: Up Close and Personal - explains the spectrum of conditions the doctor has observed in caring for more than 20,000 patients in his clinical practice. Dr. Pellegrino has been a Fibromyalgia patient himself since childhood.
As a senior resident at The Ohio State University in 1988, I gave a lecture on Fibromyalgia at the Physical Medicine Grand Rounds. One of my lecture slides was entitled Fibromyalgia, A Spectrum of Conditions? I discussed how Fibromyalgia appears to be a broader condition with specific subsets. Fibromyalgia was in that area between normal and disease the gray area. Some of the subsets were closer to normal, involving regional pain only, or milder symptoms without numerous associated conditions. Some subsets were closer to abnormal, with some features of connective tissue or rheumatic diseases, but were not quite there.
Today I’m convinced Fibromyalgia is indeed a broader condition with various subsets. I believe this information is helpful in explaining why everyone's symptoms are different even though they all have Fibromyalgia. This chapter addresses how the Fibromyalgia spectrum is part of the big picture in understanding Fibromyalgia.
Fibromyalgia Is a Distinct Medical Entity, and Appropriately So
We have long recognized, however, that many conditions overlap it, and various conditions exist that can lead to secondary Fibromyalgia. Dr. Muhammad Yunus, MD, [a professor and FM specialist at the University of Illinois College of Medicine] has developed the concept of Dysregulation Spectrum Syndrome (DSS) to describe how conditions overlap. Dr. Yunus describes DSS as representing various associated conditions that share similar clinical characteristics and pathologic mechanisms with Fibromyalgia. Ten conditions are in the DSS umbrella: Fibromyalgia, Chronic Fatigue Syndrome, Irritable Bowel Syndrome, tension headaches, migraine headaches, primary dysmenorrhea, periodic limb movement disorder, restless leg syndrome, temporomandibular pain syndrome, and myofascial pain syndrome. He predicts other entities will be added to this list in the future.
According to Dr. Yunus, Conditions in DSS Share a Number of Characteristics:
1. Patients with different conditions sharing similar profiles.
2. Common shared symptoms, such as pain, poor sleep, fatigue, and female predominance.
3. Hypersensitivity to pain.
4. No diagnostic pathology that can be measured.
5. Shared psychological complaints such as depression and anxiety.
6. Shared common genetic factor likely.
7. Common neurohormonal dysfunctions.
8. Treatments directed at the central nervous system leading to improvement.
9. TMJ [temporomandibular joint] dysfunction.
I have discussed the Fibromyalgia spectrum with my patients to help them understand the various subsets possible. I do not see Fibromyalgia as a member of a bigger family, but as the main condition. It is the founding father and keeps its name. If Fibromyalgia is the founding father, then the various overlapping conditions and subsets become the children. The name Fibromyalgia remains, but different subsets have unique characteristics and together they become the Fibromyalgia spectrum. This diagram shows the concept of the Fibromyalgia spectrum. The Fibromyalgia entity partially overlaps with the normal entity on one side and the disease entity on the other side. Within the Fibromyalgia entity are 8 subsets. The first subset is in the most normal portion of Fibromyalgia, and the 8th subset is in the most diseased portion of Fibromyalgia. Each number represents a distinct subset with distinct characteristics.
The Eight Subsets of the Fibromyalgia Spectrum Are:
1. Predisposed state
2. Prodromal [preceding] state
3. Undiagnosed Fibromyalgia
4. Regional Fibromyalgia
5. Generalized Fibromyalgia
6. Fibromyalgia with particular associated conditions
7. Fibromyalgia with coexisting mild disease
8. Secondary Fibromyalgia reactive to disease.
An individual can move up this spectrum from a lower numbered subset to a higher numbered subset, but once in a particular subset, she/he does not return to a lower numbered subset. One can achieve a remission, but stays in that subset. In other words, there is no going back.
Let's review the features of each subset.
Subset 1: Predisposed State The individual is asymptomatic. Clinical Fibromyalgia is not present in this state. The individual is at risk for developing Fibromyalgia due to hereditary factors, which may include one or both parents with Fibromyalgia or a rheumatic/connective tissue disease, or a sibling or first-degree relative with Fibromyalgia.
Subset 2: Prodromal State Prodromal means preceding, or the state leading to the condition. Clinical Fibromyalgia is still not present. There is no widespread pain or painful tender points. The individual is not asymptomatic, however. Associated conditions common with Fibromyalgia may be present in this stage, such as headaches, restless leg syndrome, fatigue, or irritable bowel syndrome. Pain may be present at times, but intermittently (not chronic, persistent pains). Even though the individual may have one or more associated condition(s), widespread persistent pain is not present, so therefore Fibromyalgia is not yet present. Typical Fibromyalgia pain must be present before we can diagnose clinical Fibromyalgia, no matter how many associated conditions may be present, but those who have numerous associated conditions are at risk.
Subset 3: Undiagnosed Fibromyalgia Chronic pain is now present, either regional or generalized in nature. This is the point of no return. The person has painful tender points which may or may not meet the American College of Rheumatology-defined 11 of 18 criteria. The person in this stage usually has milder symptoms and has not yet seen a doctor or been officially diagnosed with Fibromyalgia. If this individual were to see a knowledgeable physician, that diagnosis would be made.
Subset 4: Regional Fibromyalgia Individuals in this stage have been diagnosed with Fibromyalgia, but not generalized. Chronic pain is limited to one or a few areas such as the upper body or the low back. The symptoms may wax and wane. Usually, this subset is triggered by a trauma. I believe myofascial pain syndrome is part of this regional Fibromyalgia, and both terms are essentially synonymous. Myofascial pain syndrome has become familiar through the work of the late Dr. Janet Travell, MD, and Dr. David Simons, MD. Myofascial pain syndrome is defined by painful muscles and the presence of triggerpoints and taut bands of muscle fibers which are ropey and painful when palpated. An involuntary shortening of the fibrous muscle band can create a local twitch response. Some of those who work with myofascial pain syndrome will argue that it is a separate distinct entity from Fibromyalgia. I disagree. The similarities between myofascial pain syndrome and Fibromyalgia are far greater than their differences. They both have trigger points, tender points, ropey muscles, sympathetic nerve dysfunction, ATP abnormalities, peripheral and central mechanisms, regional and generalized versions, and associated conditions. Sound familiar? The treatments are essentially the same. As our clinical experience has evolved and our knowledge and research have become more refined, I think it is clear that myofascial pain syndrome is a part of the overall Fibromyalgia spectrum. Individuals with regional Fibromyalgia, over time, often develop generalized Fibromyalgia. Or they can remain in this stage indefinitely. Identifying the regional stage early and treating it can definitely help to prevent progression.
Subset 5: Generalized Fibromyalgia Individuals in this stage have widespread pain and tender points. They will usually meet the American College of Rheumatology-defined 11 of 18 criteria, but as previously explained, one can still have generalized Fibromyalgia with fewer tender points. Various associated conditions seen with Fibromyalgia can be present sleep disorder, irritable bowel syndrome, depression, fatigue, and so on. These associated conditions are not taking on a life of their own, so to speak, but are part of the whole and managed with the overall Fibromyalgia treatment. Regional Fibromyalgia can progress to this subset. Various causes of generalized Fibromyalgia include genetic factors, trauma, infections, and more, but secondary Fibromyalgia from a primary disease is not included in this subset.
Subset 6: Fibromyalgia with Particular Associated Conditions People in this group have developed associated conditions that are giving them particular problems which appear as separate entities requiring separate attention. Some of these particular associated conditions include irritable bowel syndrome, [Chronic Fatigue Syndrome], fatigue, tension/migraine headaches, and depression. None of these conditions in themselves have classic disease laboratory markers or cause tissue destruction, yet they may require treatments in addition to the overall Fibromyalgia treatment. Another associated condition is dysautonomia (dysfunction of the small nerves), which can cause abnormalities such as hypoglycemia [low blood sugar], hypotension [low blood pressure], cardiac arrhythmia, irritable bowel syndrome, and vascular headaches.
Subset 7: Fibromyalgia with Coexisting Disease Individuals in this category have a specific disease, and also have Fibromyalgia. The disease doesn’t necessarily cause Fibromyalgia, but can aggravate it if it’s already present. Examples of diseases that can be present and worsen the Fibromyalgia symptoms include: Hormonal problems (hypothyroidism, low estrogen, low growth hormone, and low cortisol) Infectious problems (yeast, parasite or viral infections). Low grade rheumatic or connective tissue disease (lupus, autoimmune disorders, dry eyes syndrome described by Dr. Don Goldenberg, MD, [Chief of Rheumatology at Newton-Wellesley Hospital and Professor of Medicine at Tufts University School of Medicine] may be part of a low grade Sjogren’s syndrome). * Arthritic conditions (cervical spinal stenosis, osteoarthritis, osteoporosis, scoliosis). * Neurological conditions (multiple sclerosis, polio sequelae, neuropathy, head injury residuals). For example, people who have both diabetes and Fibromyalgia will often have more painful Fibromyalgia because the diabetes caused the nerves to be more sensitive. Diabetes is a common cause of neuropathy, or damage to the small nerves, which is painful in itself and even more so with Fibromyalgia. One needs to keep the diabetes under good control to help the pain. * Lung conditions. I see a number of people who have Fibromyalgia along with a lung problem such as emphysema, asthma, chronic bronchitis, or heavy tobacco use. Cigarette smoking can increase Fibromyalgia pain. The nicotine in the smoke causes constriction of the blood vessels, decreasing blood flow, oxygen, and nutrients to the muscles, thereby increasing pain and spasms. Also, carbon monoxide in smoke enters the bloodstream and binds to the hemoglobin molecules in the blood. this blocks oxygen from binding to the hemoglobin, further decreasing oxygen availability to the muscles (and increasing pain). Stop smoking and your muscles will feel better! These diseases exist concurrently with Fibromyalgia but probably do not cause it. Any of these diseases can progress from a mild to a more severe state, and Fibromyalgia worsens as the disease worsens. The physician determines if the disease is coexisting with and aggravating Fibromyalgia (subset 7), or if a disease caused the Fibromyalgia (subset 8).
Subset 8: Secondary Fibromyalgia Reactive to DiseaseIndividuals in this category have secondary Fibromyalgia. They have a primary disease (for example lupus, rheumatoid arthritis) - and Fibromyalgia developed as a result of this disease. People in this subset probably wouldn’t have Fibromyalgia if they never had the primary disease. The primary disease requires treatment, and Fibromyalgia may improve with this treatment. However, the Fibromyalgia often requires its own treatment, and can continue to be a major problem even when the primary disease is treated or is in remission.
Overall - A Useful Explanatory Model I find that the Fibromyalgia spectrum provides a useful clinical model for me when evaluating and treating my patients. It helps me to organize them better! When I diagnose Fibromyalgia, I try to be as specific as possible about what the cause is and what subset it fits. This helps me to better explain Fibromyalgia to the patients and to individualize their treatment programs. Of course, if Ive diagnosed Fibromyalgia it would be subset 4 or greater. The patient wouldn’t be seeking a medical consultation for subsets 3, 2, or 1. If possible, I note the cause. Each subset can have flare-ups or remissions within it, and I note that as well, if appropriate. Subsets 1, 2, and 3 [predisposed state, prodromal state, undiagnosed Fibromyalgia] are useful in appreciating the progression of Fibromyalgia through the spectrum, and can be helpful when advising patients and family members who have specific concerns and questions.
Let’s Review Some Patient Profiles to Determine the Subset they Fit into in the Fibromyalgia Spectrum
Patient #1 Mary is a 25-year-old receptionist with severe neck and shoulder pain. She had always been very active with aerobics and bicycling and had never had any pain requiring treatment until after a motor vehicle accident when she was rear-ended and suffered a whiplash injury. The pain never went away, and when I saw her I found numerous painful tender points and trigger points with localized spasms in the neck and shoulder muscles. Mary has regional Fibromyalgia (subset 4). She was most likely predisposed to Fibromyalgia, and a traumatic event triggered the development of her regional Fibromyalgia. She leaped from predisposed state (subset 1) to regional Fibromyalgia (subset 4).
Patient #2 Martha is a 30-year-old housewife. She was diagnosed with Fibromyalgia 5 years ago, and she was at a stable baseline with her home program of stretches, exercises,and using a hot tub. In the past year, she has been having increasing pain and fatigue, and difficulty managing her Fibromyalgia. She reports that in the past year she has been getting frequent yeast infections. She is on birth control pills and has had a couple of bladder infections requiring antibiotics in the past year. Her more recent history is otherwise unremarkable. Martha has Fibromyalgia with a coexisting disease - chronic yeast infection (subset 7). Her birth control pills, antibiotic treatment, and perhaps Fibromyalgia have contributed to the chronic yeast infection. In turn, the yeast infection has aggravated her Fibromyalgia. [See also Dr. Pellegrino’s explanation of Candidiasis Yeast Infection and Nutritional Repair. ]
Patient #3 Jamie is a 38-year-old school teacher. She has lupus, diagnosed when she was 13 years old, and has been on various medications since then. She has been in remission for a number of years, but has developed widespread pain. Her sedimentation rate is not elevated to suggest active inflammation. Her clinical exam does not reveal any joint inflammation or active lupus findings, but she does have 16 of 18 painful tender points. Jamie has secondary Fibromyalgia from a disease (subset 8). In her case, the lupus is in remission, but her Fibromyalgia is causing her problems and needs to be treated.
Patient #4 Jamie’s 12-year-old son has been complaining of leg pains. The pains occur at nighttime, and Jamie has to rub the legs and use warm compresses. The pediatrician told her his pains were growing pains. Jamie’s son gets occasional headaches, and sometimes he feels exhausted. He plays many sports, and if he works out a lot his muscles are very sore for several days. On exam, there are no areas of pain or painful tender points. Jamie’s son is probably in a prodromal state (subset 2). He is at risk because his mother has Fibromyalgia and a connective tissue disease, and he has some associated conditions with intermittent pains, but has not developed the persistent widespread pain or painful tender points yet.
Patient #5 Bob is 42 years old and has an awful lot of pain for his age. His pains are more severe than everyday pain, and sometimes he has had to miss work. He is an assembly line worker. He mentions this to his primary care doctor when he is there for his yearly physical. He is examined and found to have 12 of 18 positive painful tender points. Bob had undiagnosed Fibromyalgia (subset 3) until he became official, entering the books with generalized Fibromyalgia (subset 5) after he saw his primary care doctor.
In Conclusion There is much disagreement and controversy among medical professionals and patients about categories and subsets of Fibromyalgia or similar conditions. I'm not attempting to stir the waters with my version of the Fibromyalgia spectrum - rather I'm trying to help you understand the fairly complicated nature of the condition and the different types I see. I find this model useful and practical in my everyday clinical practice.
Remember one of my mottos: Keep things as simple as possible and make sure they make sense!