Salla disease

To the Editor:

In a recent issue of CHEST (November 1998), Judson and Strange[1] rationally discussed that sarcoidosis caused bullous emphysema through the three cases of pulmonary sarcoidosis.

Although bullae may occur in subjects with otherwise healthy lungs, most bullae develop in smokers with centrilobular emphysema. Thus, cigarette smoking is considered to be an utmost risk factor in the development of bullous emphysema. However, the mechanism and cause of bullous emphysema have not fully been elucidated. Therefore, the current comprehensive report is important.

Giant bullae can occur in patients with sarcoidosis and advanced stages of the disease, and appear to be bullous emphysema or vanishing lung syndrome.[2-5] Although emphysema increases in frequency with age and is found most frequently in patients in the seventh decade, bullous emphysema is predominantly found in young patients. Further, the surgical procedures, including lung volume reduction surgery, are particularly beneficial for those who have bullous emphysema as well as bullous sarcoidosis.[5-8] Sarcoidosis should be carefully considered in the differential diagnosis of bullous emphysema in young patients with chronic airflow limitation (CAL), in addition to the other hereditary disorders, including [[Alpha].sub.1]-antitrypsin deficiency, Fabry's disease, Salla disease, cutis laxa, and Ehlers-Danlos syndrome.[5] There is no doubt that sarcoidosis is a cause of bullous emphysema in young patients with CAL; however, the pathogenesis of large bullae in patients with sarcoidosis remains to be determined.

Interestingly, bullous emphysema can be found in dogs, cats, and pups. Hoover and colleagues[9] suggested that congenital weakness of the bronchial cartilage and lung torsion might be involved in the development of bullous emphysema in the pup. This may, in part, support the authors' hypothesis that endobronchial lesions of sarcoidosis in bronchi and bronchioles cause remodeling of the bronchial trees and airways. Further studies and reports are needed to clarify the pathology and etiology of bullous sarcoidosis.

Correspondence to: Shinji Teramoto, MD, FCCP, Department of Geriatric Medicine, Tokyo University Hospital, 7-3-1 Hongo Bunkyo-ku, Tokyo, Japan 113-8655

REFERENCES

[1] Judson MA, Strange C. Bullous sarcoidosis: a report of three cases. Chest 1998; 114:1474-1478

[2] Miller A. The vanishing lung syndrome associated with pulmonary sarcoidosis. Br J Dis Chest 1981; 75:209-214

[3] Beim J, Hoofstein V. Aggressive cavitary pulmonary sarcoidosis. Am Rev Respir Dis 1991; 143:428-430

[4] Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis. AJR Am J Roentgenol 1985; 144:513-528

[5] Teramoto S, Fukuchi Y. Bullous emphysema. Curr Opin Pulm Med 1996; 2:90-96

[6] Teramoto S, Fukuchi Y, Nagase T, et al. Quantitative assessment of dyspnea before andafter bullectomy for giant bulla. Chest 1992; 102:1362-1366

[7] Pena CM, Cosgrove DM, Eng P, et al. Bullectomies for bullous sarcoidosis. Cleve Clin J Med 1993; 60:157-160

[8] Keller CA, Naunheim KS, Osterloh J, et al. Histopathologic diagnosis made in lung tissue resected from patients with severe emphysema undergoing lung volume reduction surgery. Chest 1997; 111:941-947

[9] Hoover JP, Henry GA, Panciera RJ. Bronchial cartilage dysplasia with multifocal lobar bullous emphysema and lung torsions in a pup. J Am Vet Med Assoc 1992; 201:599-602

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