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Relapsing polychondritis (RP) is a chronic multisystemic disease characterized by recurrent episodes of cartilage inflammation throughout the body. The lower respiratory tract is involved in 20% to 50% of patients and results in significant morbidity. Effective medical therapies and airway interventions are available in experienced centers; however, no single treatment is curative, and the prognosis of RP with airway disease remains overall guarded.

Despite recent advances in medical treatment, pulmonary arterial hypertension (PAH) continues to be associated with high morbidity and mortality. While the diagnosis is established via a right heart catheterisation, current non-invasive measures of disease severity and response to treatment used in clinical practice are the 6-min walk distance and the World Health Organization functional class. Although both parameters correlate with disease severity and prognosis, they have significant limitations. A major shortcoming in assessing PAH is lack of standardised, non-invasive, objective parameters that function as biomarkers to help assess the severity and prognosis of disease and to follow patients' response to treatment. In this article, we will review current knowledge on potential biomarkers associated with diagnosis, prognosis and response to treatment of PAH. Most biomarkers are either being evaluated for potential use in clinical practice, or being used as research tools.

OBJECTIVE: To assess the prevalence and characteristics of airway involvement in relapsing polychondritis (RP). METHODS: Retrospective chart review and data analysis of RP patients seen in the Rheumatology Clinic and the Complex Airway Center at Beth Israel Deaconess Medical Center from January 2004 through February 2008. RESULTS: RP was diagnosed in 145 patients. Thirty-one patients had airway involvement, a prevalence of 21%. Twenty-two patients were women (70%), and they were between 11 and 61 years of age (median age, 42 years) at the time of first symptoms. Airway symptoms were the first manifestation of disease in 17 patients (54%). Dyspnea was the most common symptom in 20 patients (64%), followed by cough, stridor, and hoarseness. Airway problems included the following: subglottic stenosis (n = 8; 26%); focal and diffuse malacia (n = 15; 48%); and focal stenosis in different areas of the bronchial tree in the rest of the patients. Twelve patients (40%) required and underwent intervention including balloon dilatation, stent placement, tracheotomy, or a combination of the above with good success. The majority of patients experienced improvement in airway symptoms after intervention. One patient died during the follow-up period from the progression of airway disease. The rest of the patients continue to undergo periodic evaluation and intervention. CONCLUSION: In this largest cohort described in the English language literature, we found symptomatic airway involvement in RP to be common and at times severe. The nature of airway problems is diverse, with tracheomalacia being the most common. Airway intervention is frequently required and in experienced hands results in symptom improvement.