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A2606 - Nontuberculous Mycobacterial Pulmonary Infections in Men: The Lord Windermere Syndrome?
Author Block: M. Holt, S. H. Kasperbauer, A. Levin, W. Drummond, G. A. Huitt, C. L. Daley; National Jewish Health, Denver, CO, United States.
Rationale: The phenotype of nontuberculous mycobacterial (NTM) lung disease, occurring in the absence of predisposing factors, has been defined by the study of female-predominant cohorts. This phenotype comprises right middle lobe (RML)- or lingula-preponderant nodular bronchiectasis, increased stature, reduced BMI, and increased frequencies of scoliosis, pectus excavatum and mitral valve prolapse. The goal of the current study is to describe the phenotype of NTM lung disease in men without predisposing factors. Methods: A search of the National Jewish Health Research Database was conducted with the following parameters: male sex; never-smoker status; presence of NTM lung disease (ICD10 code); absence of HIV and cystic fibrosis (ICD10 codes); and availability of a computed tomography (CT) chest. The search identified 103 cases since the inception of the Electronic Medical Record (EMR) in 2008. Following review of the EMR and CT chest data, 53 cases of ATS/IDSA-defined NTM lung disease were included in the analysis. The pattern and extent of disease, and the presence of pectus excavatum (Haller Index > 2.5) were evaluated on CT chest. Scoliosis was evaluated on postero-anterior chest x-ray (Cobb angle > 10°) and kyphosis on lateral chest x-ray (Cobb angle > 40°). If x-rays were unavailable, CT topograms were used. Continuous variables are summarized as: mean (standard deviation). Results: Patients were predominantly Caucasian (88%, n=51), aged 66 (11) years with an FEV1 of 79.6 (22.3) % predicted. Average height was 176 (9) cm, weight was 74.1 (11.1) kg and BMI was 24.0 (4.0) kg/m2. Common comorbidities were chronic rhinitis/sinusitis (49%), gastroesophageal reflux disease (49%), and asthma (23%). A single disease-causing CFTR gene mutation was present in 4 (10%, n=40) patients and a heterozygous alpha-1-antitrypsin phenotype in 5 (11%, n=47) patients. In the 35 patients not already receiving vitamin D supplementation, 17 had serum vitamin D concentrations less than 30ng/mL (49%). The most common radiographic pattern of disease was nodular bronchiectasis without cavitation (79%). Lobar-preponderant disease most commonly involved the lingula, RML and/or right upper lobe. The frequencies of pectus excavatum, scoliosis and kyphosis were 19%, 11% and 75%, respectively. Conclusions: Men with NTM lung disease in the absence of predisposing factors exhibit phenotypic similarities to their female counterparts, including a predominance of nodular bronchiectatic disease. The frequencies of radiographic pectus excavatum and scoliosis were lower than expected and kyphosis was highly prevalent. Further investigation will include control group comparison.