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Published on: November 4, 2010
Relapsing polychondritis: reversible airway obstruction is not always asthma
Michael J Segel1, Simon Godfrey, Neville Berkman
1Institute of Pulmonology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Abstract:
Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous and other proteoglycan-rich tissues. Respiratory tract involvement is a common cause of morbidity and mortality in RP. We describe a patient whose clinical features at onset of disease were typical of asthma. Later, the patient developed symptoms and signs characteristic of RP. Tracheobronchomalacia necessitated airway support by stenting. The possibility that airway obstruction in the initial stages of RP is due to airway inflammation and that early, aggressive immunosuppressive treatment of RP may delay or prevent irreversible cartilaginous destruction and airway collapse are discussed.
Insights
Relapsing polychondritis (RP) can initially mimic asthma, leading to delayed diagnosis. Early immunosuppressive treatment may prevent severe airway damage in this rare cartilaginous disease.
Area of Science:
- Rheumatology
- Pulmonology
- Rare Diseases
Background:
- Relapsing polychondritis (RP) is a rare autoimmune condition affecting cartilage.
- Respiratory tract involvement in RP frequently leads to significant morbidity and mortality.
Observation:
- A patient presented with initial symptoms consistent with asthma.
- The patient later developed characteristic signs and symptoms of relapsing polychondritis.
- Tracheobronchomalacia developed, requiring airway stenting for support.
Findings:
- Airway obstruction in early RP may be attributed to inflammation.
- The case highlights the potential for RP to initially manifest with asthma-like symptoms.
Implications:
- Early diagnosis and aggressive immunosuppressive therapy for RP are crucial.
- Prompt treatment may prevent irreversible damage to cartilage and airway collapse.
- This case underscores the importance of considering RP in patients with persistent airway issues.
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