How Do Doctors Distinguish Active RP From Permanent Damage?

⚠️ URGENT CARE WARNING: Do not wait for a test or doctor’s appointment if you experience red-flag symptoms. Seek emergency medical care for: rapidly worsening breathlessness, noisy breathing (stridor), severe chest pulling, blue lips, or an inability to speak in full sentences. Seek same-day urgent evaluation from an ENT or ophthalmologist for: sudden hearing loss, new severe dizziness/vertigo, severe eye pain, marked light sensitivity, or sudden vision changes.

One of the most challenging aspects of living with relapsing polychondritis (RP) is determining whether a new or worsening symptom is an active flare, an established structural change (damage), or a mix of both.

• Active inflammation means your immune system is currently attacking cartilage, causing swelling and irritation that may respond to immunosuppressive medications or corticosteroids ^[1][2].
• Established structural change refers to cartilage loss, fibrosis (scarring), or deformity left behind after prior inflammation. These changes may not reverse with immunosuppression, though they can often be managed with supportive care, procedures, or rehabilitation ^[3][4].

In reality, these are not always two separate categories. Active inflammation and established damage commonly coexist. Furthermore, symptoms can be caused by unrelated issues like infections, asthma, or medication side effects. Because symptoms like breathlessness or hearing loss can happen in any of these scenarios, doctors must use a careful combination of physical examinations and specialized testing.

Why Blood Tests Are Not Enough

It is a common misconception that a normal blood test means your RP is in remission. While inflammatory markers like C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are elevated in many patients during a flare, they are not perfectly reliable indicators of active disease ^[5].

Research shows that CRP and ESR levels often do not correlate with what is actually happening in the cartilage ^[6]. You can have normal blood tests while experiencing active, dangerous inflammation in your airways or deep cartilage ^[6]. Therefore, normal blood work should never be the sole reason a doctor rules out an active flare.

The Airway: Swelling vs. Structural Collapse

Breathlessness, hoarseness, and voice changes are critical symptoms to evaluate accurately, as the treatments for active airway flares and established airway damage are very different.

• Active Airway Inflammation: During a flare, the lining of the larynx (voice box), trachea (windpipe), or bronchi can become swollen with fluid (edema) and actively inflamed ^[2]. This narrows the airway, causing shortness of breath and wheezing.
• Established Structural Change: Repeated flares can weaken or destroy the rigid cartilage rings that hold your airway open. This leads to tracheobronchomalacia, a condition where the floppy airway collapses when you exhale ^[3][7]. Alternatively, chronic inflammation can leave behind stiff scar tissue, causing a fixed narrowing (stenosis) ^[8].

To investigate the cause, pulmonologists may use:

• Dynamic CT Scans: These are scans taken while you breathe in and forcefully breathe out. They allow doctors to see if the airway is flopping closed when you exhale ^[9][10].
• Pulmonary Function Tests (PFTs): Specialized breathing tests, particularly the flow-volume loop, help doctors measure airflow obstruction, though they cannot always perfectly distinguish between fixed stenosis and dynamic collapse ^[11][9].
• Bronchoscopy: A camera passed into the lungs allows a doctor to look directly inside the airway, but the procedure carries specific risks if your airway is severely obstructed and must be carefully weighed by an experienced team ^[12][10].

No single test provides a definitive answer, and active swelling can occur right alongside structural collapse ^[7].

Evaluating the Ears, Nose, and Hearing

Changes in the ears and nose are classic signs of RP, but it is important to track whether they are active or established.

• Physical Appearance: An active ear or nose flare classically involves pain, warmth, redness, and swelling of the cartilage (often sparing the soft earlobe) ^[1]. Established damage can appear as a saddle-nose deformity (a sunken nasal bridge) or a floppy, deformed ear (pinna) ^[4]. These structural changes can persist even when no active inflammation is present.
• Ultrasound: In specialized centers, doctors may use ultrasound with power Doppler (a tool that shows blood flow) to look at ear cartilage. High blood flow and swelling can suggest active inflammation, but this is an emerging, operator-dependent tool and is not yet standard everywhere ^[13].
• Hearing and Balance: RP can cause inner-ear problems. Sudden sensorineural hearing loss or new severe vertigo requires prompt ENT/audiology assessment, as treatment timing is critical ^[1][14]. While steroids may help some active inner-ear flares, hearing loss can sometimes become profound and permanent despite resolving outer-ear inflammation ^[1]. Doctors compare serial audiograms over time to track whether the hearing deficit is changing or fixed.

Monitoring the Eyes

The eyes can experience both active flares and long-term sequelae (aftereffects). Eye redness, pain, and light sensitivity (photophobia) can result from RP-associated scleritis (inflammation of the white part of the eye) or uveitis ^[15]. However, these identical symptoms can also be caused by urgent infections or acute glaucoma.

Over time, RP or the corticosteroids used to treat it can cause structural eye changes like scleral thinning, corneal scarring, cataracts, or secondary glaucoma (high eye pressure) ^[16][15]. Routine slit-lamp examinations and eye pressure checks help an ophthalmologist monitor these changes, but any new severe pain or vision loss requires immediate evaluation to rule out infection or other threats to sight.

Advanced Imaging for Deep Cartilage

Because much of the body’s cartilage (like the ribs or airways) cannot be seen from the outside, doctors often use advanced imaging.

• CT and MRI: These scans can look for cartilage thickening, increased fluid (high T2 signal on MRI), or contrast enhancement ^[6]. While useful, these findings can be non-specific and may reflect older disease rather than just new, active flares.
• PET Scans (FDG-PET/CT): This scan detects areas of the body that are consuming high amounts of glucose (sugar), which can identify metabolically active cartilage ^[17]. However, it is not a perfect tool ^[18]. Infections and normal bodily processes also consume glucose, leading to potential false positives. PET is generally reserved for complex, specialist-selected cases rather than routine monitoring ^[19].

Treatment Response and Medication Safety

Sometimes, it remains difficult to tell how much of a symptom is from active inflammation versus structural damage. In specific, carefully selected cases, specialists might use a patient’s response to immunosuppression or a corticosteroid adjustment to help guide their understanding of the disease activity ^[2][13].

Never adjust, start, or stop your own corticosteroids or immunosuppressants. A trial of steroids is not a standalone diagnostic test: symptoms might improve for reasons unrelated to RP, non-response does not automatically prove the damage is permanent, and high-dose steroids carry significant risks, including masking serious infections ^[7][1]. Medication changes must always be directed by your specialist team after a thorough evaluation and must never delay emergency care for your airway, eyes, or ears.