Unusual life-threatening Rosai-Dorfman disease of the trachea: role of NF-κB.

Rosai-Dorfman disease (RDD) is a rare non-neoplastic histioproliferative disorder characterised by painless lymphadenopathy, low fever, high erythrocyte sedimentation rate, leucocytosis and hypergammaglobulinaemia. Overactivity of nuclear factor κB (NF-κB) is linked with inflammatory, cancerous and autoimmune diseases. The first case is described of an unusual life-threatening RDD of the trachea with no lymphadenopathy at risk of suffocation in a 39-year-old Chinese woman. A diagnosis of RDD was made following CT scans, thoracotomy and histological examination. Gel shift assay revealed an essential role for NF-κB overactivity in RDD. The patient remains well with no evidence of progression without treatment. Histological confirmation should be sought in all cases as the clinical manifestation of RDD is similar to asthma or lung carcinoma.

[Impulse oscillometry for estimation of airway obstruction].

OBJECTIVE: To explore the application of impulse oscillometry (IOS) in the estimation of airway obstruction and to evaluate the correlation between spirometry indices and IOS parameters. METHODS: From Nov.2007 to May 2008, spirometry and IOS measurements were performed in 100 participants (male 72, female 28). FEV(1), FVC, FEV(1)/FVC, airway resistance at 5 Hz (R(5)), airway resistance at 20 Hz (R(20)), central resistance (Rc) and peripheral resistance (Rp) of structural parameters interpretation graph, FEV(1)% pred, R(5)% pred, R(20)% pred, and FEV(1)/FVC were analyzed. Correlations between spirometry and IOS parameters were studied. RESULTS: All participants had satisfactory impulse oscillometry results. R(5) and Rp were significantly increased in FEV(1)/FVC < 70% group [(5.3 +/- 2.1) and (6.2 +/- 2.9) cm H(2)OxL(-1)xs(-1) (1 cm H2O = 0.098 kPa)], respectively. There was significant negative correlation between FEV(1) and R(5) and Rp (correlation coefficient was -0.38 and -0.47 respectively, all P < 0.01). There was also negative correlation between FVC and R(5) and Rp (correlation coefficient was -0.28 and -0.37, respectively, all P < 0.01). A significant negative correlation between FEV(1)% pred, FVC% pred, FEV(1)/FVC% pred and R(5)% pred was also observed (correlation coefficient -0.49, -0.39 and -0.43, respectively). CONCLUSIONS: IOS parameters can be used to evaluate airway obstruction. Among IOS parameters, R(5) was the most sensitive, which was also significantly correlated with spirometric parameters.