Continuing Education Activity

Tietze syndrome is a rare, atypical cause of anterior chest pain, characterized by localized tenderness and non-suppurative swelling, typically associated with the 2nd or 3rd costal cartilages unilaterally. This benign and self-limiting condition can cause significant discomfort, and healthcare professionals play a crucial role in its evaluation and management.

The activity explores the clinical presentation, diagnostic criteria, and current treatment options for patients with this condition. There is an emphasis on highlighting the pivotal role of the healthcare team in effectively managing patients with Tietze syndrome.

Objectives:

• Identify the clinical signs and symptoms associated with Tietze syndrome, including localized chest pain and swelling over the costosternal or costochondral junctions.

• Differentiate Tietze syndrome from other conditions that present with chest pain, such as cardiac issues or musculoskeletal disorders, through a thorough clinical assessment and diagnostic evaluation.

• Implement evidence-based interventions and management strategies for individuals diagnosed with Tietze syndrome, focusing on pain relief and improving functional outcomes.

• Collaborate with the interprofessional healthcare team, including physical therapists, radiologists, and rheumatologists, to ensure a comprehensive and coordinated approach to the management of Tietze syndrome.

Introduction

Tietze syndrome is a rare, nonsuppurative inflammatory condition characterized by chest pain and swelling at the costochondral junction.[1] Tietze syndrome is a painful but benign condition characterized by localized swelling, most commonly unilaterally at the 2nd and 3rd costosternal or costochondral joints. However, the sternoclavicular joint can also be involved.

Etiology

The exact etiology of Tietze syndrome remains unclear. Some studies have postulated that multiple microtraumas to the anterior chest wall may trigger the development of Tietze syndrome. Tietze syndrome may occur more frequently in conditions like psoriatic arthritis. Other potential suggested causes may include falls, car accidents, and conditions contributing to coughing, such as sinusitis or laryngitis. Chronic, excessive coughing, vomiting, trauma or impact to the chest, viral or bacterial infections, or surgery to the thoracic area may precede the development of the disorder in some cases.[2][3] 

A recent case report suggests that Tietze syndrome can also develop after a recent asymptomatic Covid-19 infection. Results from studies propose that Tietze's syndrome may not constitute a distinct condition and is linked to the involvement of the anterior chest wall in spondyloarthritides and sternocostoclavicular hyperostosis.[4]

Epidemiology

The precise prevalence and incidence of Tietze syndrome are unknown. Biological sex, ethnicity, geography, or occupation do not provide any insights into occurrence rates. This condition appears to affect males and females in approximately equal proportions. Individuals aged under 40 years experience the condition more frequently than those over 40. Instances of the condition are infrequent with individuals aged 40 years and above, although there have been reported cases.[3][5][6]

Pathophysiology

Characterizing Tietze syndrome involves describing a localized, painful, swollen mass that typically lacks rubor or erythema and does not exhibit pustulation. The predominant association is with the cartilage of either rib 2 or rib 3, with around 70% of patients experiencing the condition localized to one side. However, this condition has also been identified at the sternoclavicular and xiphisternal joints.[7] 

Discussions are ongoing regarding the possibility that this disease process is inflammatory and may be part of a more comprehensive seronegative pathology.[3] The symptoms are often self-limiting, but Tietze syndrome recurrences are possible.

Histopathology

The costal cartilage histopathology is notable for increased vascularity and hypertrophy of the peripheral cartilage, indicating proliferation occurring. The perichondrium appears to be unaffected. Other findings include mucopolysaccharide-containing clefts that may undergo calcification.[8]

History and Physical

Patients typically present complaining of acute chest pain with no history of trauma. Because Tietze syndrome typically occurs in a younger population, patients experiencing this condition are commonly otherwise healthy. Patients with Tietze syndrome often describe the pain as dull and aching at rest. During movement and positional changes, individuals often describe the feeling as sharp, with pain radiating to the neck, arms, and shoulders. On exam, the pain is sharp and stabbing directly over the swollen area and can radiate to the shoulder and proximal arm. A practitioner can sometimes palpate a firm, spindle-shaped swelling overlying the cartilage of the affected rib. The localized pain can be mild or potentially severe. The condition typically affects upper ribs only, specifically T1 through T4, where the 2nd and 3rd ribs are the most commonly affected levels.

Heat and overt erythema are not usually associated with the swelling. There can be some discoloration and erythema present. At times, patients may experience or complain of a feeling of warmth in the affected area. Symptoms of Tietze syndrome may start suddenly but can also develop gradually. The patient's discomfort is often positional and made worse with the movement of the ipsilateral arm, twisting of the torso, coughing, sneezing, and deep breathing.[2]

Cardiac, pulmonary, and neurological exams are typically benign concerning Tietze syndrome. Any cardiopulmonary abnormal findings discovered upon exam should receive further evaluation to rule out other conditions.

Evaluation

After completing a thorough workup of life-threatening or more common diseases, healthcare professionals make a diagnosis of Tietze syndrome through a process of exclusion. Perform an electrocardiogram on all patients presenting with acute chest pain. Lab results for this syndrome are non-specific. Biopsy of the costal cartilage can lead to a more prompt diagnosis if obtained early in the disease progression. Ultrasound is the most effective modality to diagnose Tietze syndrome as this test can quickly demonstrate soft tissue swelling at the site of inflammation. Nuclear magnetic resonance is another useful diagnostic tool that accurately identifies inflammation-related alterations in the neighboring fat tissue and bone marrow. These inflammatory changes can lead to compression and joint narrowing of the affected articulation.[2] Laboratory workup may also show elevated inflammatory markers such as ESR or CRP.

Radiographs are typically read as usual, while computed tomography may show a slight focal swelling or mild sclerosing of the symptomatic joint.[9]Some recent case reports have actively utilized positron emission tomography, including a computed tomography-fluorodeoxyglucose tracer, to visualize hypermetabolic activity at the symptomatic joint with dense calcification.[10][11] This type of imaging could become a regular tool in the workup of Tietze syndrome. In summary, clinicians should actively obtain imaging and labs when patients present acute chest pain. However, utilizing the results to rule out other potential causes of Tietze syndrome is most effective. Standard magnetic resonance imaging for this condition can show some enlargement or thickening of the affected cartilage and possibly some soft tissue swelling in the area of the affected rib joints.

Treatment / Management

The keystone of Tietze syndrome treatment is conservative therapy and reassurance that this disease process will often resolve with no permanent sequelae, many times within a few weeks. This condition can last months or even up to a year. First-line management is rest and oral or topical anti-inflammatory and analgesic agents. As the underlying etiology is inflammation, typically nonsteroidal medications are likely going to be most effective, more so than acetaminophen or opioids. Clinically recommend up to 10 days' worth of scheduled nonsteroidal medication at an anti-inflammatory dose, provided no contraindications exist. A short course of oral steroids such as prednisone or methylprednisolone could also be considered as an alternative if there are no contraindications.

If rest and nonsteroidal medications do not provide substantial relief, healthcare providers may administer a targeted injection of local anesthetic, steroid, or a combination of both at the site of maximum swelling and observe the site using a sonogram. Some patients have reported benefits with warming pads applied to the affected area. In some persistent, severe cases, clinicians have reported resecting cartilage as a treatment option. Clinicians generally do not recommend this treatment and suggest considering this intervention only on a case-by-case basis.[2]

Differential Diagnosis

Upon the initial presentation of acute chest pain, considering a broad differential diagnosis is crucial since Tietze syndrome is diagnosed by excluding other potential causes. Clinicians frequently misdiagnose this condition as costochondritis because both conditions present with anterior chest wall pain that palpates at the sternochondral and costochondral junctions. Costochondritis is associated with multiple ribs, typically involving ribs 2 through 5. Also, costochondritis is not associated with localized swelling over the affected joints. Ultrasound will not demonstrate significant findings with costochondritis. However, a sonogram actively visualizes inflammation and swelling in diagnosing Tietze syndrome. Physical examination to look for visible swelling can also aid in coming to this diagnosis.

Rule out other pathologies such as acute coronary syndrome, hypertensive crisis, inflammatory processes or infections of the lungs and accompanying pleura, malignancies, chest trauma-associated fractures, rheumatoid or pyogenic arthritis, gastroesophageal reflux disease, or psychogenic disorders.[2][6] Other conditions on the differential diagnosis also include rib fractures and conditions such as lupus and fibromyalgia.

Prognosis

Tietze syndrome is usually a self-limiting disease with a good prognosis. Preventing this condition remains uncertain due to the lack of complete understanding regarding the cause or causes of Tietze syndrome. Most patients report full resolution of symptoms within 1 to 2 weeks with conservative management, but there have been reports of the characteristic swelling and pain lasting for up to one year. Reoccurrences are also possible. Most patients make a full recovery from this Tietze syndrome.

Complications

Complications directly associated with Tietze syndrome are typically rare, with potential risks arising from injections, medications, or surgery. These complications may encompass infection, pneumothorax, adverse reactions to medication, side effects, and scarring.

Postoperative and Rehabilitation Care

Tietze syndrome symptoms typically improve and resolve over time with conservative management, including rest and nonsteroidal anti-inflammatory medications. Avoiding rigorous or strenuous activities that can trigger or cause the recurrence of this condition is essential, especially in the immediate recovery phase. Addressing any condition that may contribute to a patient coughing, sneezing, retching, or vomiting is vital during this phase, as these factors can contribute to a recurrence.

Consultations

Tietze syndrome is a clinical diagnosis of exclusion. If there is any clinical suspicion or family history of a rheumatological condition, consulting a rheumatologist proves helpful in ruling out associated conditions or other factors contributing to a patient's symptoms. This condition may be related to certain rheumatological conditions, such as seronegative spondyloarthritides, and other conditions, such as psoriatic arthritis.

Consulting with a radiologist can be helpful as well. Obtaining a positron emission tomography and computed tomography-fluorodeoxyglucose or other imaging of the area can help confirm this diagnosis and rule out other potential etiologies, such as a rib fracture.

In rare cases of refractory Tietze syndrome, where a patient continues to experience ongoing severe symptoms, consider consulting with a clinician. Consider whether to perform a resection of the affected joints and cartilage.

Deterrence and Patient Education

Advise patients to avoid strenuous activity for 1 to 2 weeks, as there is a chance to exacerbate their discomfort and possibly delay the resolution of their symptoms. Providers should follow these patients closely if there is a concern for worsening symptoms or increasing swelling.

Enhancing Healthcare Team Outcomes

The most essential point regarding Tietze syndrome is the diagnosis of exclusion. This condition is not a deadly disease. To minimize the patient's physical discomfort, mental anguish, wasted time, and expenses, practitioners should be well-versed in the clinical presentation, identify the condition, conduct a comprehensive workup for any acute life-threatening disorders, and educate patients about this generally benign and self-limited condition to provide reassurance.[6] Communication between the interprofessional care team is essential in making an appropriate and timely diagnosis. Exclude other possibilities and provide reassurance. The patient can then usually be managed by their primary care team on an outpatient basis as needed.