The Autoimmune Connection: Essential Information for Women on Diagnosis, Treatment, and Getting On With Your Life (52 page)
Authors: Rita Baron-Faust,Jill Buyon
Fellow Travelers—Fibromyalgia, Chronic Fatigue, Endometriosis, and Interstitial Cystitis
I was diagnosed with juvenile rheumatoid arthritis when I was in eighth grade. But I’ve been fortunate in that I only seem to get bad flares every few years. I was always able to stay active in some way; I’ve even run marathons. When I have a flare, the pain and fatigue are very bad . . . it’s hard to do my job . . . I can’t write or type, I can’t open a car door, or even turn the key in the ignition . . . but I would take different drugs and it would get better. Even with those problems, I had started to think of my RA as kind of predictable. But then I started to develop pain in other areas of my body. Not in the joints, but in the muscles around them . . . and at very specific points like my shoulders, elbow, or knees. And I got this overwhelming aching fatigue, like I had the flu . . . I couldn’t seem to think straight . . . I just wanted to sleep all the time, but I never really felt rested. This was unlike any of the RA flares I’d ever had, and none of the drugs I normally took seemed to help it. My rheumatologist kept saying it was my RA getting worse, but the blood tests did not show any signs of a flare and my joints were not affected. My doctor finally diagnosed me with fibromyalgia.
C
ATE
, 30
Fibromyalgia
is
not
an autoimmune disease. However, it occurs so often with autoimmune diseases, particularly rheumatoid arthritis (RA), Sjögren’s syndrome, and systemic lupus erythematosus (SLE), and has such a major impact on so many women’s lives, that we felt it should be included in this book.
Symptoms of fibromyalgia can overlap with autoimmune connective tissue diseases, and it’s often difficult to tell whether it’s an RA or SLE flare or not. Additionally, new insights have been gained into the nature of this disorder—its characteristic signs, its possible causes, and its effects on women with autoimmune diseases.
Fibromyalgia isn’t a new problem by any means. In the 1880s it was called
neurasthenia
,
1
a vague ailment attributed to “weakness or exhaustion of the nervous system” that sent women to their beds with aches and pains, unexplained fatigue, and depression (and maybe a swig of “Lydia Pinkham’s Vegetable Compound,” a popular cure-all containing 20 percent alcohol). But it wasn’t until 1975 that two Canadian researchers studying rheumatoid arthritis patients described the distinct symptoms—including sleep disorders, diffuse musculoskeletal pain, and tenderness at specific points around the body—that characterize fibromyalgia.
2
It was first called
fibrositis
, meaning “muscle inflammation,” but studies later showed there was no inflammation in the muscles of patients with the disorder.
Today, the constellation of symptoms known as
fibromyalgia syndrome (FMS)
is recognized as a distinct medical disorder involving pain amplification or hypersensitivity, with myriad dimensions.
The American College of Rheumatology (ACR) first established formal diagnostic criteria for FMS in 1990,
3
updating those criteria in 2010,
4
making further tweaks in 2011.
5
The newer ACR criteria include high levels of fatigue, unrefreshing sleep, and cognitive problems (trouble thinking or remembering things) plus chronic pain in multiple areas.
The biggest change is the way fibromyalgia is diagnosed. The 1990 criteria called for examination of 18 “tender points” (see illustration), specific points of the body sensitive and painful to the touch. This has been replaced by findings of persistent musculoskeletal pain in areas
correlating
to those tender points. There’s also a severity scale (SS) score for other symptoms (see
page 422
), which requires more careful attention to how patients
experience the disorder. Mood, which can vary and is very difficult to quantify, is not considered in the criteria.
2011 ACR guidelines for FMS correlate the 1990 ‘tender points’ with localized areas where there is pain and sensitivity.
Source: American College of Rheumatology
The ACR says the old system missed 25 percent of fibromyalgia patients, while the newer criteria accurately identify more than 90 percent of patients. A simple six-item self-report FMS questionnaire is also being studied.
6
While it’s not 100 percent accurate, experts say the updated ACR criteria can help distinguish symptoms of RA, SLE, and other autoimmune diseases from fibromyalgia and may improve treatments for both.
The list of FMS-associated symptoms is long and very general—so it’s quite easy to
self-diagnose
. In addition, there are no clinical or laboratory tests that can confirm the diagnosis. This is why fibromyalgia has been, and remains, a controversial and difficult diagnosis.
Fibromyalgia typically strikes women between the ages of 20 and 40, possibly caused by abnormalities in the processing of pain signals by the central nervous system. Some studies have suggested that it may be triggered by injuries, infections, or autoimmune diseases.
Between 2 and 4 percent of women in the general population may be affected, two to seven times more women than men
7
depending on which set of criteria is used.
Up to 25 percent of women with autoimmune diseases, including rheumatoid arthritis, lupus, and Crohn’s disease, may meet updated ACR criteria for fibromyalgia.
Fibromyalgia symptoms also overlap with other autoimmune “fellow travelers” such as
chronic fatigue syndrome
,
irritable bowel syndrome (IBS)
, and
interstitial cystitis (IC)
.
The underlying causes of fibromyalgia are still unknown. Some experts believe that some stressor or trauma—be it a physical injury, immune overstimulation (as in autoimmune disease), hormonal alterations (such as being hypothyroid), infections, extreme emotional stress, or a combination of factors—triggers disturbances in the central nervous system of susceptible women, leading to oversensitivity to even low levels of pain. It’s been likened to having the “volume turned up” to pain and other stimuli.
Fibromyalgia can run in families, primarily among women, mostly affected by pain amplification. “We don’t have a single candidate gene, and it’s probably a combination of genetic factors, but the evidence is that there’s a predisposition to this disorder,” remarks Laurence A. Bradley, PhD, professor of
medicine in the division of Clinical Immunology and Rheumatology at the University of Alabama at Birmingham. “There may be multiple disruptions in the function of structures in the central nervous system involved in pain transmission and modulation, along with dysregulation of neurotransmitters involved in pain processing and stress hormones.”
However, without genetic factors or biomarkers that may signal enhanced risk, there’s no way to make reliable predictions at this time as to who is especially vulnerable to FMS, with or without a family history, Dr. Bradley stresses.
Brain activity in processing pain seems to be different in people with FMS. “We identified resting state abnormalities in cerebral blood flow in two brain structures involved in processing pain in patients with fibromyalgia, compared to healthy people. And in non-patients who report pain, we also saw low levels of blood flow in one of those structures,” says Dr. Bradley. Many people with fibromyalgia appear to have abnormalities in the
autonomic nervous system
(which governs breathing, heart rate, and blood pressure, among other things), causing symptoms like
orthostatic hypotension
(dizziness when going from a sitting to standing position).
Brain chemicals that may be affected in FMS include
serotonin
(which helps regulate mood, sleep, and appetite) and
norepinephrine
(a stress hormone). Studies have also found levels of
substance P
, a chemical that helps transmit pain signals, to be up to three times higher in the spinal fluid of fibromyalgia patients than levels in healthy individuals.
8
A small study at the Cedars-Sinai Medical Center in Los Angeles also found increased levels of inflammatory cytokines stimulated by substance P in blood samples from 56 people with fibromyalgia, compared to 36 healthy people.
9
One cytokine,
interleukin-8 (IL-8)
promotes pain, and another,
interleukin-6 (IL-6)
, induces hypersensitivity to painful stimuli, fatigue, and depression. (IL-6 is also elevated in people with lupus and rheumatoid arthritis.) Researchers believe that these inflammatory cytokines may play a role in increased pain sensitivity.
“The evidence is not very consistent, but there is some question as to whether there may be abnormalities in some of the cytokines that promote inflammation in people with fibromyalgia. However, there is no reliable evidence so far that there is an immune system problem in fibromyalgia, and certainly not an autoimmune problem,” says Dr. Bradley, who has studied the disorder for years.
“Nevertheless, there are individuals with autoimmune diseases such as rheumatoid arthritis who develop fibromyalgia as a secondary condition,” he adds.
Psychological factors also come into play. The lifetime incidence of mood disorders like depression and anxiety in people with fibromyalgia ranges from 40 to 70 percent, but that may be an overestimate. “When we compare people being treated for fibromyalgia with non-patients, nonpatients tend to have lower scores on questionnaires that measure depression or other kinds of psychological distress, and they often feel they’re more able to use their internal resources to cope with their pain than the people who are treated in the clinic,” comments Dr. Bradley.
The main symptoms of fibromyalgia—fatigue, unrefreshing sleep, cognitive problems (dubbed “fibro fog”), and chronic pain in multiple areas of the body—are frustratingly nonspecific.
While many women with fibromyalgia also have painful connective tissue diseases like lupus or rheumatoid arthritis, in many cases no physical joint, bone, or tissue damage or inflammation can be found.
Fibromyalgia pain often waxes and wanes, shifts from one area of the body to another, and is often accompanied by numbness or tingling. Some women complain that they “ache all over,” while others report pain only in certain regions of the body, like the lower back and hip. In fact, many women may initially be diagnosed with lower back problems or an overuse injury like tennis elbow (
lateral epicondylitis
) or heel pain (
plantar fasciitis
).
Other problems common in fibromyalgia include headaches, dry eyes, jaw pain (
temporomandibular joint syndrome, TMJ
), noncardiac chest pain, heartburn, painful periods, urinary frequency and urgency (
irritable bladder
), and pelvic pain. Women with FMS may also have other, more regional pain-related syndromes, such as
irritable bowel syndrome
, interstitial cystitis, endometriosis, and vulvar pain syndromes (
vulvodynia
and
vulvar vestibulitis
, a chronic irritation, rawness and pain in the vulva. labia, or the
vulvar vestible
, a crescent shaped opening around the vaginal opening).
“Many of these symptoms and syndromes cluster together. For example, it’s much more likely for someone with chronic pain to have irritable bowel syndrome or TMJ syndrome than someone who doesn’t have chronic pain,”
observes noted FMS authority Daniel J. Clauw, MD, a professor of medicine in the division of rheumatology and director of the Center for the Advancement of Clinical Research at the University of Michigan, Ann Arbor.
A majority of women with fibromyalgia report severe fatigue; at least half of those who meet the criteria for FMS will also meet diagnostic criteria for chronic fatigue syndrome (CFS), says Dr. Clauw. “While the defining symptom of CFS is fatigue, you also have to have pain-based symptoms.”
In FMS, fatigue and pain are worse after physical activity, and women may also experience major problems with concentration and memory. Other symptoms can include weight gain (or loss); intolerance to heat, cold, and loud noises; and problems with vision or hearing. Women may also complain of “allergic” symptoms, ranging from drug reactions and multiple chemical sensitivities to respiratory symptoms like runny nose and nasal congestion.
Symptoms of fibromyalgia may come on after viral infections, such as
Epstein-Barr virus
(infectious mononucleosis),
Lyme disease, parvovirus
, or
Q fever
, all of which have a pain component. “Two to 3 percent of people who develop these infections will never completely recover and end up with fibromyalgia or chronic fatigue syndrome,” says Dr. Clauw. There’s also a subset of people who develop hypothyroidism and have chronic widespread pain. And even after their thyroid hormone is replaced and they are made euthyroid, they never regain their baseline state of health and have something indistinguishable from fibromyalgia.”
Certain medications, like the cholesterol-lowering drugs called
statins
, can provoke chronic widespread muscle pain, which persists even after the drug is stopped. Symptoms mimicking fibromyalgia can also occur when women are being tapered off high-dose corticosteroids. However, regular pain medicines (like aspirin, ibuprofen, or acetaminophen) don’t help the pain of fibromyalgia, and that’s often what brings a woman to her doctor’s office.
The symptoms of fibromyalgia and autoimmune disorders overlap, including joint and muscle pain, fatigue, and morning stiffness, as well as a feeling that the hands or feet are swollen. Some women may have symptoms similar to
Raynaud’s phenomenon
(see
page 131
), but their entire hand turns pale or red, instead of just the fingers. Facial flushing and a mottled red rash on the legs (
livedo reticularis
) are also common in fibromyalgia, and women may initially be misdiagnosed with lupus. FMS often coexists with autoimmune diseases.
The 2010–2011 ACR classification criteria for fibromyalgia include three major symptoms—
fatigue, unrefreshing sleep, and cognitive problems
(trouble thinking or remembering things)—plus a variety of other nonspecific symptoms (see
page 417
) and persistent pain in at least seven areas of the body:
5
- Shoulder girdle (shoulder blades and collar bone)
- Left or right hip (buttock and area where the hip joint meets the upper thigh bone)
- Left or right jaw
- Upper back/lower back (right or left)
- Upper arm/lower arm (right or left)
- Upper leg/lower leg (right or left)
According to the ACR criteria, the number of painful areas adds up to a
widespread pain index (WPI)
. Symptoms are assessed with a severity score of 5 or above (on a scale of 1 to 12). Or, both the WPI and the SS must add up to 9 or above to meet the criteria.
The Fibromyalgia Network (
www.fmnetnews.com
) has adapted the ACR criteria into a self-assessment form, which may be valuable to bring to a doctor visit (see
Appendix A
).
If you, or your doctor, suspect you have fibromyalgia, you’ll undergo a thorough physical examination to exclude neurological, orthopedic, and rheumatological causes for your symptoms.
More acute, short-term pain may be due to an injury or disease flare, and may need a more extensive workup. Physical signs of inflammation like swollen joints (
synovitis
) or muscle weakness are not signs of fibromyalgia. But inflammatory and noninflammatory mechanisms can both cause fibromyalgia symptoms, so having a response to anti-inflammatory medications doesn’t automatically rule out FMS. Since one in three lupus patients also have fibromyalgia, often doctors attribute symptoms to SLE and try to treat them with prednisone. However, lupus patients don’t usually have the overall pain sensitivity seen in fibromyalgia.
There are no diagnostic tests or blood markers for fibromyalgia.
Your doctor may order a complete blood count; blood tests for liver, kidney, and thyroid function, and a sedimentation rate or level of C-reactive protein to detect inflammation. Unless your pain has come on suddenly or there’s evidence that you may have an autoimmune disease, testing for markers such as rheumatoid factor (RF) or antinuclear antibodies (ANAs) is usually not done.
If you have dizziness when going from a prone to standing position, your blood pressure will be measured as you do this to see if you have a sudden drop in pressure (
orthostatic hypotension
, or
orthostatic instability
), a common symptom in fibromyalgia. If you’re having pain and stiffness in weight-bearing joints, x-rays or other imaging may be done to look for evidence of arthritis, either rheumatoid arthritis or
osteoarthritis
(the wear-and-tear kind that breaks down cartilage in the joints).
Cate’s story continues:
My husband was fine through my RA flares, but he doesn’t understand this.
It really has put a strain on our relationship. He keeps saying, “Why do you need to sleep so much? Why can’t you do things? Why are you upset so easily? Can’t you take something for the pain?” It’s very frustrating. I know if my RA got bad, there are drugs I could take to help it. But nothing really seems to make fibromyalgia better . . . I’ve had antidepressants . . . I’ve taken bigger doses of pain medications. The only thing that really seems to help is exercise. So I just force myself to go out and walk or run. It’s hard, and I can’t exercise as much as I used to, and that gets me down sometimes, much more so than having RA. But I refuse to give in to this. Since I’ve had RA for so long I understand what’s going on in my body. But fibromyalgia? This, I just don’t understand.