A 63-year-old man with recurrent pulmonary infections: a case of Swyer-James Macleod syndrome.
Swyer-James Macleod syndrome was described for the first time in 1953 by Paul R. Swyer and G.C.W. James in a six-year-old boy with recurrent right lung bronchopneumonia and bronchitis who had unilateral emphysema and secondary deficiency of pulmonary blood supply. (1) Subsequently W.M. Macleod reported a series of nine cases, aged 18 to 41, with abnormal transradiancy of one lung. (2) It is an uncommon cause of recurrent pulmonary infections. A case of Swyer-James Macleod syndrome diagnosed at age 63 in a patient with recurrent respiratory infections and history of childhood pertussis is presented.
A 63-year-old African American man presented to the pulmonary clinic with complaints of slowly progressive shortness of breath and recurrent pneumonia over the previous 20 years. He was recently hospitalized for acute worsening of shortness of breath, cough productive of scanty yellowish sputum, and was treated for bronchitis with steroids and antibiotics. He had no history of hemoptysis, night sweats, fever, chills, weight loss, nausea, vomiting or heartburn. He reported occasional chest tightness. Past medical history was significant for pertussis during childhood, more than 10 episodes of pneumonia over the past 20 years which were treated as an outpatient, hyperlipidemia and hypertension. He had no allergies. He smoked one pack of cigarettes per day for five years and quit 35 years before. There was no history of alcohol or recreational drug abuse. He was born in Nigeria and moved to the United States 30 years prior. The patient was a married, retired teacher with three children. His medications included inhaled fluticasone-salmeterol, tiotropium, and albuterol; losartan; amlodipine; hydrochlorthiazide; and gemfibrozil.
Patient was an average build, well-nourished man in no apparent distress. Vitals signs were heart rate 75 beats per minute, temperature 97.2[degrees]F, respiratory rate 14 per minute, blood pressure 140/95 mm of Hg. Trachea was midline. Jugular venous pressure was normal. There was no stridor. Chest expansions were asymmetric, with decreased expansion of the right hemithorax, decreased breath sounds on the right upper lung zone and crackles in the right, mid to lower lung zones. Cardiac examination was normal. There was no peripheral edema, clubbing, or cyanosis.
Biochemical and hematologic tests, including arterial blood gases, were within normal range. Pulmonary function testing revealed very severe reduction in forced expiratory volume at one second ([FEV.sub.1]), air trapping and severe decrease in diffusing capacity (Table 1). Chest radiograph (Figure 1) showed hyperlucent right lung with decreased vascular markings and a small hilar shadow. CT (Figure 2) showed a hypoplastic pulmonary artery, bronchiectasis, hyperlucency on the right side and no pulmonary embolism. Ventilation-perfusion radionuclide scan (Figure 3) showed matched ventilation and perfusion defects in the right lung.
[FIGURE 1 OMITTED]
The patient was diagnosed with Swyer-James Macleod syndrome and continued to be treated with bronchodilators and inhaled corticosteroids. At three month follow-up visit his symptoms were improved and he required no further courses of antibiotics. He received influenza and pneumococcal vaccinations.
The proposed pathogenesis of Swyer-James Macleod sydrome is bronchiolitis obliterans caused by respiratory infections in early childhood leading to inflammation, fibrosis and obstruction of the terminal and respiratory bronchioles, possibly preventing normal development of the alveolar bud (Figure 4). The syndrome is an acquired cause of unilateral emphysema. The combination of fibrotic obliteration of the capillary vascular bed in the interalveolar septa and decreased ventilation decreases pulmonary artery blood flow to the affected segments leading to pulmonary artery hypoplasia on the involved side. (1,3) Organisms that have been associated with this syndrome are adenovirus, measles, bordetella pertussis, mycobacterium spp, influenza A and mycoplasma. Several authors have suggested that adenovirus is the most common causative agent. The differential diagnosis on chest radiograph includes unilateral agenesis or extrinsic pulmonary artery occlusion from tumor, pulmonary embolism, bronchial obstruction (central or extrinsic) causing compensatory emphysema, bronchial atresia, pseudo hyperlucent lung in patients after mastectomy, absence or atrophy of pectoral muscles, and shoulder girdle. (4)
[FIGURE 2 OMITTED]
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Presentation varies from asymptomatic to a severe respiratory impairment. Patients usually present with recurrent respiratory tract infections, chronic cough with expectoration, dyspnea, and hemoptysis. The diagnosis can be made in infancy or early childhood, but in asymptomatic individuals can be delayed until adulthood. (11)
[FIGURE 4 OMITTED]
Chest radiograph classically shows hyperlucency of the affected lung, decrease in vascular markings and small hilar shadow. Expiratory radiographs may reveal air trapping in the affected lung and contralateral mediastinal shift. On the contrary, in unilateral agenesis of the pulmonary artery, the hilar shadow is absent and there is contralateral hyperlucent lung. (4) CT imaging is the diagnostic modality of choice as it can confirm the diagnosis and exclude other diagnostic possibilities. On CT the affected lung appears hyperlucent, with air trapping more evident on expiration. (5) There is also decreased size of the parenchymal vasculature, bronchiectasis and a hypoplastic ipsilateral pulmonary artery. (6) Ventilation-perfusion radionuclide scans show matched ventilation and perfusion defects. (7-9) A pulmonary angiogram is seldom required to confirm hypoplasia of the main pulmonary artery and of its branches. (4) Pulmonary function testing shows obstruction and hyperinflation in the majority of patients, but a restrictive component can be observed in patients with more pronounced hypoplasia of the affected lung. Elevated neutrophils and CD8+T lymphocytes as well as a decreased CD4/CD8 ratio have been reported in two patients where a bronchoalveolar lavage was performed, suggestive of ongoing active inflammation. (10)
The prognosis depends on the extent of bronchiectasis and severity of ventilatory impairement. Treatment involves medical therapy aimed at treating infections, especially in patients with bronchiectasis. Inhaled bronchodilators and inhaled corticosteroids are commonly used and may improve symptoms. (7) Surgical resection of the affected area or pneumonectomy may be required in patients with severe bronchiectasis causing recurrent pneumonia and/or recurrent severe hemoptysis. (7,11)
* Swyer-James Macleod syndrome is a rare acquired cause of unilateral hyperlucent lung.
* It is caused by respiratory infection in early childhood leading to bronchiolitis obliterans and hypoplasia of the pulmonary artery.
* Chest radiography shows unilateral hyperlucent lung, a small hilar shadow and decreased vascular markings on the affected side.
* CT shows air trapping, more evident during expiration.
* Management includes antibiotics to treat recurrent infections. Surgery may be necessary for recurrent pneumonia and severe hemoptysis.
(1.) Swyer PR, James GC. A case of unilateral pulmonary emphysema. Thorax 1953;8:133-136.
(2.) Macleod WM. Abnormal transradiancy of one lung. Thorax 1954; 9:147-153.
(3.) Marchevsky AM, Guintu R, Koss M, et al. Swyer-James (MacLeod) syndrome with placental transmogrification of the lung: a case report and review of the literature. Arch Pathol Lab Med 2005; 129:686-689.
(4.) Gottlieb LS, Turner AF. Swyer-James (Macleod's) syndrome. Variations in pulmonary-bronchial arterial blood flow. Chest 1976;69:62-66.
(5.) Moore AD, Godwin JD, Dietrich PA, et al. Swyer-James syndrome: CT findings in eight patients. AJR Am J Roentgenol 1992;158:1211-1215.
(6.) Lucaya J, Gartner S, Garcia-Pena P, et al. Spectrum of manifestations of Swyer-James-MacLeod syndrome. J Comput Assist Tomogr 1998; 22:592-597.
(7.) Salmanzadeh A, Pomeranz SJ, Ramsingh PS. Ventilation-perfusion scintigraphic correlation with multimodality imaging in a proven case of Swyer-James (Macleod's) syndrome. Clin Nucl Med 1997; 22:115-118.
(8.) Kobernik R, Savaser AN, Rossdeutscher R, et al. Scintigraphic images of Swyer-James syndrome. Eur J Nucl Med 1988;14:217 219.
(9.) O'Dell CW, Taylor A, Higgins CB, et al. Ventilation-perfusion lung images in the Swyer-James syndrome. Radiology 1976; 121:423-426.
(10.) Bernardi F, Cazzato S, Poletti V, et al. Swyer-James syndrome: bronchoalveolar lavage findings in two patients. Eur Respir J 1995;8:654-657.
(11.) McKinney LA, Mathai MG, Byrd RP Jr, Roy TM. An unusual presentation of a rare disorder: Swyer-James-Macleod's syndrome. Clin Pulm Med 2002;9:105-108.
Neeraj R. Desai, MD; Jaime Palomino, MD; Hafez Hayek, MD; Jay Shames, MD; and Francesco Simeone, MD
Drs. Desai, Palomino, Hayek, shames, and Simeone are with the Section of Pulmonary Diseases, Critical Care and Environmental Medicine, Tulane University Health Sciences Center in new Orleans.
Table. Pulmonary function test. Pre Bronchodilator Actual % Predicted FVC (L) 2.00 50 [FEV.sub.1] (L) 0.98 32 [FEV.sub.1]/FVC (%) 49 63 TLC (L) 7.90 113 RV (L) 5.34 234 RV/TLC (%) 68 205 [D.sub.L]CO (mL/min/mmHg) 15.51 48 [D.sub.L]CO/VA (mL/min/mmHg/L) 3.37 73 Post Bronchodilator Actual % Predicted % Change FVC (L) 2.20 55 10 [FEV.sub.1] (L) 1.23 40 25 [FEV.sub.1]/FVC (%) 72 14 TLC (L) RV (L) RV/TLC (%) [D.sub.L]CO (mL/min/mmHg) [D.sub.L]CO/VA (mL/min/mmHg/L) FVC = forced vital capacity; FEV = forced expiratory volume at one second; TLC = total lung capacity; RV = esidual volume; [D.sub.L]CO = diffusing capacity of the lung for carbon monoxide; and VA = measurement of alveolar volume.
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|Author:||Desai, Neeraj R.; Palomino, Jaime; Hayek, Hafez; Shames, Jay; Simeone, Francesco|
|Publication:||The Journal of the Louisiana State Medical Society|
|Article Type:||Case study|
|Date:||Nov 1, 2010|
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