Relapsing Polychondritis (RP)

The tube may not fix the obstruction - In RP the trachea and bronchi collapse because the cartilage rings holding them open have been destroyed, so the obstruction sits at and below the level of an endotracheal tube rather than above it. Passing a tube through the cords can leave the malacic segment still collapsing, and a tube tip that ends above the diseased segment stents nothing. Know from imaging where the floppy segment is before you induce, and have a plan for a collapse the tube cannot reach - a longer tube, a rigid bronchoscope, and ENT or thoracic in the room.

Map the airway before induction - Dynamic exploratory CT is the study that shows RP airway involvement: air trapping, expiratory collapse, airway wall thickening, and luminal narrowing. Pulmonary function testing adds the degree of air trapping and the lung volumes. Read those, not just the Mallampati - the airway exam at the bedside tells you nothing about a segment two-thirds of the way down the trachea.

Expect a narrower lumen than the patient's size predicts - Luminal narrowing and wall thickening are part of the disease, and laryngeal chondritis puts the tight spot below the cords where you cannot see it. Have a range of smaller tubes and a video laryngoscope open before the first attempt rather than sending for them after a tube will not pass.

Sedation is not the safer option - Collapse in a malacic airway is driven by transmural pressure, not by who is holding the laryngoscope. Sedation that drops respiratory drive and airway tone can precipitate the same obstruction without a secured airway to rescue it. If you sedate, keep it light and be set up to convert to a definitive airway at any point.

Emergence is the dangerous end - Respiratory failure from airway collapse is the most frequent cause of death in RP, and the loss of tone at emergence is exactly when a malacic segment closes. Extubate fully awake and sitting up, with the difficult-airway cart and the same rescue plan you used at induction still in the room, and set a low threshold for a monitored bed instead of routine PACU discharge.

Definitive rescue is mechanical - When the airway in RP collapses, what reopens it is mechanical: airway dilation, stenting, tracheostomy, or laryngotracheal reconstruction. Steroids treat the inflammation but will not reopen a collapsed segment in the moment. On a high-risk case, agree beforehand on who performs which of those and where the rigid scope is sitting.

Nose and ears are damaged cartilage - Nasal chondritis affects about a quarter of patients and produces the saddle-nose deformity, so avoid nasal intubation and nasogastric tubes where the case allows it. Auricular chondritis is present in about 90% and the inflamed pinna is painful - keep mask straps, circuit tubing, and head rings off the ear when you position and pad.

Steroids and immunosuppressants come with the patient - Severe RP with laryngeal or tracheobronchial involvement is treated with IV methylprednisolone 1 g daily for three days followed by oral prednisone 1 mg/kg, plus an immunosuppressant - cyclophosphamide 1 to 2 mg/kg, methotrexate, azathioprine, cyclosporine, or infliximab. Ask what they are on and when they last took it, plan stress-dose coverage against their maintenance dose, and treat them as immunosuppressed for line and airway asepsis.

Document the eyes before you start - Ocular involvement runs 20% to 60% and includes episcleritis, scleritis, keratitis, and uveitis. Tape and lubricate deliberately, and record any pre-existing eye findings preoperatively so a postoperative complaint is not attributed to positioning.

Look for the disease behind the disease - Over 30% of RP patients carry a coexisting autoimmune or hematologic condition, and RP overlaps clinically with rheumatoid arthritis, ANCA-associated vasculitis, polyarteritis nodosa, and Behcet syndrome. Check the CBC and the problem list for a second diagnosis before you assume the cartilage is the whole story.

Relapsing polychondritis (RP) is a rare autoimmune attack on cartilage itself. Antibodies against type II, IX, and XI collagen, together with CD4+ T cells that recruit monocytes and macrophages, drive release of matrix metalloproteinase-3 and cathepsins L and K, which digest the proteoglycan matrix. Each flare leaves chondrocyte apoptosis, focal calcification, and fibrosis behind, so cartilage is progressively replaced by floppy scar.

That is what makes it an airway disease. The cartilage rings that hold the trachea and bronchi open are targets, and once they are destroyed those airways collapse (tracheobronchomalacia), presenting as cough, hoarseness, and wheeze. Auricular cartilage is involved in about 90% of patients with the lobe spared, joints in 50% to 75%, eyes in 20% to 60%, and nasal cartilage in about 25%. Respiratory compromise from airway collapse is the most frequent cause of death.