Symptoms and Emergency Warning Signs

Recognizing the symptoms of relapsing polychondritis (RP) is a vital part of managing the condition. Because RP is a systemic (body-wide) disease, symptoms can appear in many different areas, from your ears to your heart ^[1]. Understanding what is ‘normal’ for your condition versus what requires immediate or urgent action can help you navigate life with more confidence.

Common Signs of Inflammation

Most people with RP will experience inflammation in the cartilage of the ears, nose, or joints. Routine flares (like a mildly red, painful ear without a fever) are often managed with your rheumatologist and are not usually considered medical emergencies ^[2].

• Ear Inflammation (Auricular Chondritis): This is often the first sign of RP. Your outer ear may become red, hot, swollen, and very painful to the touch ^[2]. A suggestive sign of RP is that the inflammation often spares the earlobe. Because the lobe is made of fat and skin rather than cartilage, it remains its normal color while the rest of the ear is bright red ^[3]. However, bacterial infections can also spare the earlobe, so if the ear is accompanied by fever, drainage, or rapidly spreading redness, seek prompt assessment rather than assuming it is an RP flare.
• Nasal Inflammation (Nasal Chondritis): You may feel pain or tenderness at the bridge of your nose ^[4]. Over time, repeated inflammation can weaken the cartilage, leading to a visible flattening of the nasal bridge known as a saddle-nose deformity ^[5].
• Joint Pain (Polyarthritis): RP can cause migratory joint pain, meaning it moves from one joint to another. It most often affects the hands, knees, and ankles, causing swelling and stiffness ^[6].
• Systemic Symptoms: Many patients experience extreme fatigue, low-grade fevers, and unexplained weight loss during active flares ^[7][1].

Eyes, Ears, and Balance

The tissues in your eyes and inner ears are also susceptible to the immune system’s mistakes in RP.

• Eye Involvement: You may experience redness, pain, or blurred vision. This can be caused by scleritis (inflammation of the white part of the eye) or uveitis (internal eye inflammation) ^[8].
• Audiovestibular Symptoms: Inflammation can affect the inner ear, leading to sudden hearing loss, tinnitus (ringing in the ears), or vertigo (a spinning sensation/dizziness) ^[9].

Emergency and Urgent Warning Signs

While most RP symptoms can be managed with your rheumatologist, certain manifestations involve critical structures like your airways, large blood vessels, vision, or hearing. These require prompt medical intervention.

1. Call 911 (or your local emergency number) for:

• Airway Crisis: The cartilage that keeps your windpipe (trachea) and voice box (larynx) open can become inflamed or weakened. If the airway narrows (stenosis) or becomes floppy (malacia), it can lead to a life-threatening blockage ^[10][11]. Seek emergency care for:
  • Stridor: A high-pitched, gasping, or whistling sound when you breathe.
  • Shortness of Breath: A feeling of choking, air hunger, or being unable to speak in full sentences ^[12].
  • Visible Effort: Drawing in the skin around your neck or ribs when trying to breathe, or blue lips ^[13].
• Cardiovascular Crisis: RP can cause inflammation of the aorta (the body’s main artery). While an aortic aneurysm is usually monitored and managed with planned surgery, a dissection (a tear in the artery wall) is a surgical emergency ^[14][15]. Seek emergency care for:
  • Sudden, Severe Pain: Intense “tearing” or “ripping” pain in the chest, upper back, or between the shoulder blades ^[15].
  • Unexplained Fainting: Sudden loss of consciousness or severe lightheadedness ^[16].
  • Signs of Heart Failure: Sudden, severe breathlessness, especially when lying flat, or rapid swelling of the legs ^[17].

Note for Emergency Responders: If you have known airway narrowing, it is helpful to carry a medical alert card. Standard intubation (placing a breathing tube) can be more difficult in RP patients, and specialized equipment or techniques may be required ^[18][19].

2. Seek Same-Day URGENT Care for:

• Vision Threats: Severe eye pain, extreme light sensitivity (photophobia), or any new vision loss can indicate necrotizing scleritis or uveitis, requiring urgent ophthalmology care to protect your sight.
• Hearing Threats: Sudden hearing loss or severe vertigo requires urgent assessment.
• New Hoarseness: A sudden change in your voice or a total loss of voice ^[19]. If hoarseness is accompanied by breathing difficulty, treat it as an emergency.
• Suspected Infection: A hot, swollen ear accompanied by fever, drainage, or a wound.