Tietze Syndrome: A Rare Inflammatory Disorder

Tietze syndrome is a rare, benign inflammatory disorder that causes swelling in one or more of the upper ribs at the costochondral junction — where the ribs attach to the sternum. Symptoms can develop rapidly or gradually and can spread to other parts of the upper body. The cause is unknown.

Signs and Symptoms

The main symptom of Tietze syndrome is pain in one or more of the upper four rips, usually the second or third rib. The pain can be mild to severe. It is dull, sharp, or aching and may spread to the neck, shoulder, and arm. Swelling occurs in the cartilage connecting the rib to the sternum, and the area may be red and feel warm.

Causes

Experts are unsure what causes Tietze syndrome. Some researchers believe that it results from a series of microtraumas that damage the ribs, leading to the inflammation. Frequent coughing or vomiting or something more sudden, like an impact from a car accident or surgery to the thoracic area, can cause these microtraumas.

At-Risk Populations

Because it is so rare, the prevalence of Tietze syndrome is unknown. Researchers believe that it affects males and females equally. It is unlikely to affect infants, children, and the elderly and most often occurs in the 20s and 30s. Most cases happen sporadically, and there does not seem to be a genetic component.

Diagnosis of Exclusion

Tietze syndrome is a diagnosis of exclusions, which means that it is diagnosed in people with chest pain and cartilage swelling, but other conditions should be ruled out first. Other conditions have similar symptoms, and a diagnosis of Tietze syndrome can only be made after a thorough physical exam and testing.

Possible Symptoms

In addition to pain and inflammation, other symptoms of Tietze syndrome may include a low-grade fever, psoriasis exacerbations, and blisters on the hands and feet. Some lab values may also help diagnose Tietze syndrome, including elevated white blood cells, C-reactive protein, and erythrocyte sedimentation rate. These are all indicative of inflammation.

Physical Exam

On physical exam, Tietze syndrome usually presents with one side of the thorax swollen and palpable tenderness over the affected joint, typically the second or third rib. Usually, the doctor assesses this by applying gentle pressure with one finger to the chest wall to locate the precise location of the pain.

Imaging

Because Tietze’s syndrome is so rare, researchers suggest imaging tests for a correct diagnosis. Ultrasound is the most common to reveal swelling, but specialized scans like nuclear magnetic resonance imagining (MRI) can show inflammatory changes that occur without impacts on the bone and cartilage. Detailed imaging is an effective way to differentiate between Tietze’s syndrome and other conditions.

Treatment

There have not been many studies about treating Tietze’s syndrome. Common interventions include conservative pain management, like NSAIDs and other analgesics. If the pain does not respond to medication, steroids and numbing agents can be injected into the joint, but this is rare. The pain usually resolves within two weeks of treatment and may disappear on its own.

Costochondritis

Tietze syndrome is commonly confused with costchondritis, a common condition that presents similarly. These terms are sometimes used interchangeably, but they are two separate conditions. The distinguishing factor between costochondritis and Tietze syndrome is swelling. Swelling does not occur in costochondritis. The latter is also more likely to affect those over age 40.

Other Conditions

Other conditions that can cause symptoms similar to Tietze syndrome can be more serious. They include fibromyalgia, ankylosing spondylitis, coronary heart disease, and pneumonia. In rare cases, malignant lymphoma may cause chest pain and swelling similar to Tietze syndrome, demonstrating why lab work and imaging are necessary to get an accurate diagnosis.