[Marked airway constriction due to relapsing polychondritis]

Area of Science:

• Rheumatology
• Internal Medicine

Background:

• Relapsing polychondritis (RP) is a rare, systemic autoimmune disease characterized by progressive cartilage destruction.
• Airway involvement, particularly tracheal stenosis, is a serious complication associated with poor prognosis in RP.

Observation:

• A 40-year-old woman presented with symptoms including dry cough, sore throat, fever, auricular swelling, and conjunctival redness.
• Chest CT revealed tracheal narrowing due to enlarged mediastinal soft tissues, raising suspicion for RP.
• Cartilage biopsy confirmed the diagnosis of relapsing polychondritis through pathological examination.

Findings:

• Initial treatment with prednisolone was ineffective in alleviating the patient's symptoms.
• A combination therapy of methylprednisolone pulse therapy and oral diaminodiphenylsulfone led to gradual symptom resolution.
• Histopathology demonstrated cartilage dissolution and lymphocytic infiltration, consistent with RP.

Implications:

• This case underscores the importance of early diagnosis and aggressive management of relapsing polychondritis, especially when airway compromise is suspected.
• Combined immunosuppressive therapy, including methylprednisolone pulse and diaminodiphenylsulfone, can be effective in managing severe RP cases.
• Close monitoring for airway obstruction is critical due to the risk of sudden death in patients with relapsing polychondritis.