Tietze’s Syndrome: Causes, Tests, and Treatments
Stephanie Kenrose
Smashwords Edition
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Copyright 2010 Stephanie Kenrose
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Table of Contents
In 1992, I had never heard of Tietze’s syndrome. If you'd have asked me what it was, I might have said it had something to do with mosquitoes (Tietze’s syndrome is sometimes confused with the sleeping sickness caused by the tsetse fly and referred to incorrectly as tsetse’s syndrome).
In the spring of 1992, I was taken to the ER with symptoms of a heart attack . I was 26 years old. A crushing feeling enveloped my chest like an elephant was sitting on top of me; the pain was like nothing I had ever experienced. The panic that accompanied the pain was overwhelming--luckily, it wasn’t a heart attack, but I left the ER that evening without knowing the cause of my pain. Months later, I still didn’t have a diagnosis.
“There’s nothing wrong with you,” an impatient chest specialist told me after two months of trying to diagnose the excruciating pain in my chest. I’d had tests for heart problems, blood disorders, stomach disorders…all to no avail. “You should consider seeing a psychiatrist,” he told me. I took him up on it, briefly, but a year later, I still had the pain (and a prescription for Valium).
The large amounts of ibuprofen I was consuming daily caused acid reflux that burned by esophagus and added more pain to my chest area. I went to a different doctor to ask him for prescription strength Zantac. I was a new patient, so he performed a thorough exam, listening to me as I asked him not to tap my chest. “Where does it hurt?” he asked. I pointed to my ribs. “Everywhere.” He tapped my costal cartilage gently. “Here?” he asked. I nodded. “You’ve got Tietze’s Syndrome,” he said.
I’d never heard of it. The doctor explained that he’d only heard of it in passing (he was fresh out of medical school), and that all he knew about it was that it was a rare disorder, characterized by crushing pain when the chest is pressed on. Tietze’s syndrome, he explained, was like costochondritis; only unlike costochondritis, with Tietze's syndrome the rib cartilage swells and the condition can be chronic--sometimes lasting decades.
Tietze's syndrome was named after a German surgeon named Alexander Tietze (1864-1927), who discovered the disease in 1921. The physician studied at Breslau (Wroclaw), obtaining his doctorate in 1887 and eventually practicing at the Allerheiligen-Hospital. He wrote an excellent textbook on emergency surgery which was published in 1927, and he contributed numerous papers on surgical topics. Ninety years after its discovery, the rare disorder named after him has had only a few hundred cases reported in the medical literature worldwide.
Many sources of information will tell you that Tietze's syndrome is the same as costochondritis . On the contrary, the two conditions are different.
Tietze's syndrome is characterized by inflammation and swelling in the costochondral (rib) cartilage, which causes pain that varies from very mild to so severe that it can mimic a heart attack . Costochondritis and Tietze’s syndrome affect the same region of the chest--the cartilage that connects the ribs to the breast bone—but that’s about where the similarity ends.
Tietze’s causes inflammation, tenderness, and swelling. According to the Mayo clinic, swelling is absent with costochondritis . Note that you may not be able to feel the swelling—for example, if the Tietze’s syndrome is affecting the first costal joint, it may be hidden behind your clavicle.
Tietze’s syndrome affects men and women equally, but according to Wright’s Radiology of the Chest and Related Conditions, Tietze’s syndrome usually occurs in the 20-40 year-old age range bracket. On the other hand, costochondritis tends to affect women over the age of 40, although anyone can get it. One case highlighted in the literature tells of a two-year-old boy who had a painful swelling in one of his left ribs that was diagnosed as Tietze’s syndrome: luckily for the boy, the swelling died down after a few weeks.
According to Harrison's Rheumatology, costochondritis affects the third, fourth, or fifth joints down in between the breast bone and ribs (you can feel these joints with your fingers). Tietze's syndrome tends to affect only one joint in 80% of cases, although the pain may radiate out, making it nearly impossible for a sufferer to tell which rib is causing the pain.
Costochondritis is usually short-lived, whereas Tietze’s syndrome can last for years, turning into a chronic condition. One half of Tietze’s syndrome patients have pain that lasts more than one year.
My doctor prescribed Vioxx , which I took for nine months to alleviate the pain. The drug worked wonders, but thankfully I was not one of the estimated 100,000 people who suffered a heart attack due to Vioxx!
That is how my year-long journey into understanding Tietze’s syndrome happened. I wasn’t crazy or stressed: I had a legitimate disease. Two decades later, I still have Tietze's syndrome, but I've learned ways to manage it.
The fact that it took a full year for doctors to diagnose my condition is no longer puzzling to me. Tietze's syndrome is a rare condition (there are only a few hundred reported cases in the medical literature going back to 1921), and Tietze’s syndrome is often confused with other disorders of the chest--even by physicians. These include costochondritis and slipping rib syndrome —which occurs when one of the ribs slips out of place, stretching the ligaments. In Tietze's syndrome, a rib does not slip out of place: the rib stays in place but the costal cartilage swells.
Tietze’s syndrome is sometimes confused with costosternal chondrodynia but the two conditions are exact opposites! Costosternal Chondrodynia often affects several ribs. It rarely affects the second costal joint. Some researchers note that most cases of Tietze’s syndrome occur only in the second costal joint. There are only a few similarities between Tietze’s syndrome and Costosternal Chondrodynia: an unknown origin of the disease, the disease’s benign (not harmful) nature, and the fact that both disease can last for years.
You may also find Tietze’s syndrome called many other names—it may be impossible for you to figure out which disease you have unless you have a diagnostic (such as an ultrasound) performed. Some other names that Tietze's might be called (or mistaken for!):
Chest wall Syndrome
Condropathia Tuberosa
Costal Chondritis
Costochondritis
Costochondrial Junction Syndrome
Costochondritis
Cyriax's syndrome
Slipping Rib Syndrome
Parasternal chondrodnia
Peristernal perichondritis
Thoracochondralgia
Tsetse Disease
Tietze’s Disease
Xiphoidalgia
Tietze’s syndrome was a frustrating disease in the beginning; if only I had had a correct diagnosis, so much stress and anguish would have been avoided! Understanding what this disease is—and what it isn’t—is the first step to effective treatment.
Doctors are well educated. However, Tietze's syndrome is so rare that even your doctor might miss the diagnosis (he's going to be trying to decide between about three dozen different possibilities for your pain, and Tietze's is way down on that list). Two general practitioners and two specialists missed my diagnosis of Tietze's syndrome; it took one year for me to finally get diagnosed--and that diagnosis made it a lot easier to deal with. This chapter will show you how to tell if you have Tietze's syndrome--a benign inflammation of the ribs--or something more sinister, like Pleomorphic T-cell lymphoma , a rare cancer.
Before continuing, make absolutely sure that your physician has ruled out pain caused by heart problems or other serious illness.
Tietze’s Syndrome Symptom Checklist
Do you have intense pain in the rib cartilage area? That's at the junction between your sternum and ribs. The pain could be so severe you might even mistake it for a heart attack . The pain may radiate, and you may not even be sure of the central location for the pain--just that it's somewhere in or on your chest.
Do you have pain in the chest area when rolling over in bed, or trying to get out of bed? This is caused by pressure from the ribs squeezing the inflamed breast plate cartilage. You might feel this pain when otherwise twisting, turning or bending.
Does the pain radiate from the chest to the arms and shoulders? (The pain might be so severe it could feel like it is coming from everywhere).
Is the pain worse when breathing? Sometimes even a little movement of the rib cage will cause intense pain.
Lightly press down on the cartilage area between your sternum (breast plate) and ribs. Is it tender and painful in at least one of the first three ribs?
These questions are the most common about Tietze's and costochondritis. The hallmark for Tietze's syndrome is that in addition to the pain described above, usually only one rib is affected--the second or third rib down. There are rare exceptions--for example, if the swelling is in the first joint, it may be hidden under your clavicle and you won't be able to feel the swelling. Feel the connections around your other ribs to see what's normal for you. If you feel swelling at the first, second, or third junctions, you most probably have Tietze's syndrome. However, make sure you see a doctor: there are a couple of cancers that can cause one costal junction to be swollen and painful; skin cancer that has invaded the chest area and Pleomorphic T-cell lymphoma is one example.
The chances of a tumor causing your pain are about as slim as contracting Tietze's syndrome itself (only a few hundred cases have ever been documented in the medical literature). However, the same tests that check for Tietze's syndrome (an MRI or CT-scan) will also be able to definitively tell if you have a tumor.
Tietze's syndrome (unlike costochondritis) can turn into a chronic condition lasting years, or even decades. Why should you get a diagnosis instead of waiting for the pain to go away? If your doctor tells you that you have "costochondritis ," you might expect the pain to disappear in a few weeks. If it doesn't, you should insist on a test to rule out other causes .
I am fairly certain that a virus caused my Tietze’s syndrome. How do I know this? Not because of any body of literature, but because of a bizarre series of coincidences that started with a trip to Ireland and ended with karmic retribution and a divorce.
In February, 1992, I traveled to Ireland on a ferry from Hollyhead to Dublin, a wave-riding roller coaster that took a nauseating two hours. I was on my way to a dog show, with a fellow dog breeder named Gwen. We knew each other from dog shows we attended every few weeks, and shared a love for Siberian huskies, so we had a lot to talk about. Six weeks after I returned home from the trip, I was sitting on a friend’s couch watching Star Trek: The Next Generation when a crushing pain enveloped my chest. My friend must have seen my pale face, because she asked “Are you OK?” I wasn’t okay. I was in so much pain, I could hardly speak. I’ve given birth naturally, and childbirth paled in comparison to the searing pain I was experiencing in my chest. My friend rushed me to the ER, and I had the sickening, strange feeling that I was not going to make it out of the ER. After I described my symptoms (crushing chest pain), I had a heart-attack work up, and ex-ray to check for blood clots, and finally, after the major tests were negative, a dose of Valium that rendered me stupid enough to actually enjoy shopping at Wal Mart on the way home (driven there by my friend of course).
I took six weeks off of work. Sometime around week five, my ex-husband, John came down with the disease and I began to suspect a virus. The final clue to the origin of my disease came about six months later; I realized that I hadn’t seen Gwen (the lady who had accompanied me to Ireland) in many months. In fact, no one had seen nor heard of her since the Ireland trip. I called her on the phone and asked her if she had any plans to go to any dog shows in the near future. She said no. “I came down with a disease,” she said. “I’m in so much pain…I can’t do anything.”
After speaking to Gwen on the phone, I discovered we had the same ailment. And like me, she had come down with it a few weeks after our return from Ireland. Add that to my ex-husband coming down with the disorder six weeks after I returned home, and we had out answer: our ailment was caused by a virus.
Although researchers have yet to figure out all of the causes of Tietze’s Syndrome, they have some pretty good ideas, from as benign as coughing to as frightening as cancer. More than one of the following conditions in combination may cause Tietze’s Syndrome and it’s often hard or even impossible to pinpoint an exact cause.
Trauma , Recurrent Microtrauma, or Intercostal hematoma. A fast, unexpected movement could cause a pool of blood—a bruise—to form in the costal cartilage. Bruises (hematomas) in the cartilage joining the ribs to the breast bone (the intercostal area) have been seen in baseball players, but it could be cause by something unexpected like shaking a rug.
Recurrent episodes of micro trauma to the chest wall: boxing, karate and other martial arts, severe vomiting and/or coughing, are examples of where the chest could experience continuous trauma, but it could be as simple as lifting heavy objects in an awkward fashion over time.
Fibromyalgia . Fibromyalgia is a chronic, disabling disorder that occurs in about 2% of the population, and is seen more in women that in men. The cause of fibromyalgia is not known, but significantly higher levels of substance P (a neurotransmitter responsible for transmitting pain signals) have been found in the spinal fluid of patients with fibromyalgia. The inflamed costochondral margins that are commonly associated with Tietze’s syndrome are the same as two of the pressure points associated with fibromyalgia; it’s therefore important that all of a patient’s symptoms are taken into account when making a diagnosis of Tietze’s.
Arthritis and related diseases. Rarely, patients with psoriatic arthritis have reported Tietze’s syndrome; psoriatic arthritis is a condition where arthritis is accompanied by red patches of inflamed skin. Other arthritis related diseases (i.e. ankylosing spondylitis, reactive arthritis and Reiter's disease) might also produce Tietze’s syndrome.
Ulcerative colitis or Crohn's disease . Patients with inflammatory bowel disease such as ulcerative colitis and Crohn’s disease often report costochondritis and Tietze’s syndrome.
Cancer . An Italian study led by R. Cocco found that several patients who were diagnosed with Tietze’s syndrome actually had tumors. The patients all had the classic signs of Tietze’s syndrome—swelling of the costal cartilage, pain--and they were all treated with a standard treatment for the disease: anti-inflammatory drugs and an injection of cortisol. When symptoms didn't improve, the patients sought answers: three of the cases were later found to have Hodgkin's disease and one person had non-Hodgkin's lymphoma. All four patients made a complete recovery after the cancer was diagnosed.
Heredity factors. No one knows exactly what causes Tietze’s syndrome, but it’s thought there could certainly be a hereditary factor. It could run in families—perhaps because of a genetic abnormality.
Radiation Therapy. If you have had radiation therapy to the chest or breast area, there is a chance you will experience Tietze's syndrome sometime down the road (it could be years after the therapy).
Syphilis. Some studies of Tietze's syndrome have linked syphilis to outbreaks of Tietze's syndrome.
Stress. There's no definitive research to suggest that stress causes Tietze's syndrome, but it can make an underlying condition like fibromyalgia worse.
Viruses. A virus caused my Tietze’s syndrome; could it have caused yours?
Tietze's syndrome is a rare disease by itself. However, there are a few unusual cases of Tietze's syndrome reported in the medical journals. It’s unlikely that your Tietze’s syndrome is caused by one of these very rare occurrences.
Breast Implants . One breast cancer survivor who had reconstructive surgery (with large breast implants) ended up suffering from Tietze's syndrome due to the large implants rubbing against her ribs.
Skin Cancer . One unfortunate patient, diagnosed with Tietze's syndrome, found out that although his symptoms were identical to Tietze's syndrome, further diagnostics revealed he actually had skin cancer which had invaded his inner chest.
Malignant Tumor. There's at least one person out there who is glad he didn't accept a diagnosis of Tietze's syndrome. One patient presented with typical symptoms of Tietze's syndrome--pain in one left costal cartilage. After a biopsy, it was revealed he had Pleomorphic T-cell lymphoma: cancer. While an MRI or CT scan could have caught the tumor , X-rays could not.
E-Coli. One case of E-coli in a diabetic woman led to a severe case of costochondritis . Although different, Tietze's syndrome is closely related to costochondritis, so it isn't so farfetched to think that E-Coli could occasionally cause the disease.
There are several tests that can be undertaken to determine whether you have Tietze's syndrome or not. Why test? Because testing can aid you with treating your disease and will rule out other causes , like fibromyalgia and cancer. Your doctor may recommend one of the following techniques to rule out other disorders:
Computed tomography (sometimes known as a CT or CAT scan) is one of the most exact ways to diagnose Tietze's syndrome.
Xeroradiography (a technique commonly used for mammograms) allows a doctor to see a complete picture of the chest wall and rule out other cause of your pain, such as tumors.