October 2023 Medical Image of the Month: Swyer-James-MacLeod Syndrome

Figure 1. Frontal (A) and lateral (B) topographic images from a non-contrast chest CT show a relative paucity of lung markings in the right hemithorax. There are at least 2 large, cystic-appearing lesions in the right lung, which appears somewhat hyperinflated. Axial (C) and sagittal (D) reconstructions from the CT confirm unilateral areas of emphysematous appearing hyperinflated lung with surrounding atelectasis. The left lung appears relatively normal. Click here to view Figure 1 in an enlarged, separate window.

Figure 2. Hematoxylin and Eosin stained low-power pathological image from right upper lobectomy (A) demonstrates chronic bronchiolitis with features of subtotal obliterative bronchiolitis associated with mild septal fibrosis and prominent emphysematous/cystic change. Elastic trichrome stain of a small airway (B) demonstrates subtotal bronchiolitis obliterans. Click here to view Figure 2 in an enlarged, separate window.

Pathological slides from a right upper lobectomy specimen obtained at an outside institution were submitted to our Department of Pathology for review. A pre-operative noncontrast chest CT from the outside institution was submitted along with the path slides. The patient was a 27 y/o man who presented to the outside institution with exercise intolerance and increasing shortness of breath following a bout of COVID in early 2023. The patient also related a history of possible chronic myocarditis. A review of the CT demonstrated 2 distinct right-sided areas of hyperinflated, emphysematous lung with a relatively normal appearing left lung (Figure 1). Although congenital lobar emphysema was considered, the multifocal nature of the findings suggested against this, and a diagnosis of Swyer-James-MacLeod Syndrome was entertained. Histopathological analysis (Figure 2) confirmed this suspicion. When questioned further, the patient related a history of neonatal RSV infection requiring 3 weeks of hospitalization. The constellation of historical, radiological, and pathological findings was consistent with Swyer-James- MacLeod Syndrome.

This syndrome was first described in 1949 in a case report of a six-year-old boy from the UK by pediatrician Paul Swyer and radiologist George James (1). The pulmonologist William MacLeod published a review of nine cases in 1954 (2). Also known as “unilateral hyperlucent lung syndrome”, this is a postinfectious form of bronchiolitis obliterans. Severe infection early in life, while the lungs are still developing, is the proposed mechanism. Although different infections organisms are associated with this, respiratory syncytial virus is most implicated. Swyer-James-MacLeod Syndrome usually affects the lungs asymmetrically. On imaging, the most common pattern is that of a unilateral hyperlucent lung which may or may not be associated with hyperinflation and may or may not be associated with bronchiectasis (3). There is reduction in pulmonary blood flow and formation of septal fibrosis which leads to obstruction of pulmonary capillary beds. Alveolar hyperinflation leads to mechanical resistance to pulmonary blood flow and reduced ventilation leads to pulmonary vasoconstriction. Most patients asymptomatic, and many cases discovered incidentally. Treatment is usually conservative and preventative, focused on controlling pulmonary infections. Inhaled corticosteroids may have a limited role in treatment as well (4).

Samantha Moore, MD, PhD¹ and Clinton Jokerst MD²

Department of Laboratory Medicine and Pathology¹ and Department of Radiology²

Mayo Clinic Arizona, Scottsdale, AZ USA

References

1. Swyer PR, James GC. A case of unilateral pulmonary emphysema. Thorax. 1953 Jun;8(2):133-6. [CrossRef] [PubMed]
2. William Mathieson Macleod. Lancet. 1977 Oct 15;2(8042):833. [PubMed]
3. Lucaya J, Gartner S, García-Peña P, Cobos N, Roca I, Liñan S. Spectrum of manifestations of Swyer-James-MacLeod syndrome. J Comput Assist Tomogr. 1998 Jul-Aug;22(4):592-7. [CrossRef] [PubMed]
4. Mehra S, Basnayake T, Falhammar H, Heraganahally S, Tripathi S. Swyer-James-MacLeod syndrome-a rare diagnosis presented through two adult patients. Respirol Case Rep. 2017 Jun 16;5(5):e00245. [CrossRef] [PubMed]

Cite as: Moore S, Jokerst C. 2023 Medical Image of the Month: Swyer-James-MacLeod Syndrome. Southwest J Pulm Crit Care Sleep. 2023;27(4):45-46. doi: https://doi.org/10.13175/swjpccs042-23 PDF

September 2023 Medical Image of the Month: Aspergillus Presenting as a Pulmonary Nodule in an Immunocompetent Patient

Figure 1. Chest CT showing 11 x10 mm nodule in the anterior segment of the left upper lobe in the background of emphysematous and basal sub segmental atelectatic changes.

Figure 2. Lung biopsy low power (A) showing chronic inflammatory infiltrate in the interstitium along with a collection of fungus (arrow) (H&E: x40). Fungus with an area of necrosis (B) (H&E: x100). Numerous thin, narrow-angle, and branching hyphae with septa morphologically consistent with Aspergillus (C) (H&E: x400). Collection of Aspergillus (D). (Periodic acid–Schiff stain: x400).

A 32-year-old nonsmoking woman presented with complaints of recurrent hemoptysis for 5 months and dyspnea on exertion for 1 month. She denied any history of fever, cough, or COVID infection. She has hypothyroidism controlled on thyroxine 25mcg. During the evaluation, she was found to have an enhancing solitary pulmonary nodule (11 x 10 x 9mm) in the anterior segment of the left upper lobe (Figure 1). The patient was given a course of oral antibiotics (amoxicillin /clavulanic acid) and supportive treatment for hemoptysis. Sputum for Ziehl–Neelsen stain and cartridge based nucleic acid amplification test (CBNAAT) was negative. CT- guided biopsy of the nodule was performed. Histopathology showed fungal organisms which were thin, septate with acute angle branching and focal necrotic areas, morphologically consistent with Aspergillus (Figure 2). Serum-specific IgG against aspergillus antigen was normal. The patient was started on oral itraconazole 200mg BID. Follow-up after 1 month showed both symptomatic and radiological improvement. Repeat chest CT showed a significant decrease in size of the nodule.

There is a large spectrum of pulmonary aspergillosis. From this spectrum, pulmonary nodules are a less common manifestation of chronic pulmonary aspergillosis (CPA), especially in immunocompetent individuals. Aspergillus nodules are defined as small, round, discrete, and focal opacities on chest imaging. It can be further classified on basis of internal cavitation (i.e., non-cavitary nodules and cavitary nodules). Differentiating these nodules from other lung pathology may be difficult on CT findings alone and may demand further investigation like image-guided needle aspiration cytology or biopsy, blood investigations like serum Aspergillus precipitin IgG antibody and/or serum Aspergillus galactomannan. Delay in diagnoses may lead to persistence of pulmonary symptoms, and cavitation of the nodule. This entity has a favorable prognosis if managed accordingly. Although there is data regarding surgical management of aspergillus nodules, but data regarding the benefits of anti-fungal therapy in the same is limited.

Diagnosing aspergillus nodules in an immunocompetent individual is a challenge to all pulmonologists. Literature shows limited case reports and small case series on CPA presenting as non-cavitating SPN on radiology. Usually, in such cases, the diagnosis is made following removal or biopsy of the nodule(s), presuming it to be malignant. Patients diagnosed with Aspergillus nodules can’t be differentiated from lung malignant conditions based on demographics, which are usually similar. In the largest case series of Aspergillus nodules done by Muldoon EG et al. (6), 33 patients were reviewed constituting less than 10 % of the cohort of patients with CPA.  In a study done by Kang et al. (4) 77% of patients with aspergillus nodules were symptomatic and the most common symptom reported was hemoptysis. Similarly in our case hemoptysis was the chief complaint of the patient. Our patient is a woman and non-smoker similar to previous case reports and series.

In the current guidelines, the detection of serum Aspergillus precipitin IgG antibody is a key diagnostic criterion for CPA. Literature is unclear if the presence of Aspergillus IgG antibody could be considered a supportive finding in the making the diagnosis of Aspergillus nodules. Similarly, in our case also serum specific IgG against Aspergillus fumigatus was negative. Azoles are the primary treatment option in all subtypes of CPA including aspergillus nodule. Our patient also showed disease regression during itraconazole treatment. Another option for management is surgical, though it is associated with significant postoperative complications and recurrence of disease at other sites and must be reserved for selected patients.

Dr. Deependra Kumar Rai, Dr. Priya Sharma, Dr. Vatsal Bhushan Gupta

Department of Pulmonary, Critical Care, and Sleep Medicine

AIIMS Patna, Bihar, India

References

1. Kosmidis C, Denning DW. The clinical spectrum of pulmonary aspergillosis. Thorax. 2015 Mar;70(3):270-7. [CrossRef] [PubMed]
2. Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008 Mar;246(3):697-722. [CrossRef] [PubMed]
3. Lee SH, Lee BJ, Jung DY, Kim JH, Sohn DS, Shin JW, Kim JY, Park IW, Choi BW. Clinical manifestations and treatment outcomes of pulmonary aspergilloma. Korean J Intern Med. 2004 Mar;19(1):38-42. [CrossRef] [PubMed]
4. Kang N, Park J, Jhun BW. Clinical Characteristics and Treatment Outcomes of Pathologically Confirmed Aspergillus Nodules. J Clin Med. 2020 Jul 10;9(7):2185. [CrossRef] [PubMed]
5. Yasuda M, Nagashima A, Haro A, Saitoh G. Aspergilloma mimicking a lung cancer. Int J Surg Case Rep. 2013;4(8):690-2. [CrossRef] [PubMed]
6. Muldoon EG, Sharman A, Page I, Bishop P, Denning DW. Aspergillus nodules; another presentation of Chronic Pulmonary Aspergillosis. BMC Pulm Med. 2016 Aug 18;16(1):123. [CrossRef] [PubMed]
7. Denning DW, Cadranel J, Beigelman-Aubry C, et al. Chronic pulmonary aspergillosis: rationale and clinical guidelines for diagnosis and management. Eur Respir J. 2016 Jan;47(1):45-68. [CrossRef] [PubMed]
8. Limper AH, Knox KS, Sarosi GA, et al. An official American Thoracic Society statement: Treatment of fungal infections in adult pulmonary and critical care patients. Am J Respir Crit Care Med. 2011 Jan 1;183(1):96-128. [CrossRef] [PubMed]
9. Godet C, Philippe B, Laurent F, Cadranel J. Chronic pulmonary aspergillosis: an update on diagnosis and treatment. Respiration. 2014;88(2):162-74. [CrossRef] [PubMed]
10. Kousha M, Tadi R, Soubani AO. Pulmonary aspergillosis: a clinical review. Eur Respir Rev. 2011 Sep 1;20(121):156-74. [CrossRef] [PubMed]

Cite as: Rai DK, Sharma P, Gupta VB. September 2023 Medical Image of the Month: Aspergillus Presenting as a Pulmonary Nodule in an Immunocompetent Patient. Southwest J Pulm Crit Care Sleep;27(3):30-32. doi: https://doi.org/10.13175/swjpccs035-23 PDF