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Case Reports
. 2023 Aug 10:14:1222431.
doi: 10.3389/fimmu.2023.1222431. eCollection 2023.

Case Report: Middle lobe syndrome: a rare presentation in eosinophilic granulomatosis with polyangiitis

Beatrice Maranini  1 , Ippolito Guzzinati  2 , Gian Luca Casoni  2 , Maria Ballotta  3 , Andrea Lo Monaco  1 , Marcello Govoni  1
Affiliations
Case Reports

Case Report: Middle lobe syndrome: a rare presentation in eosinophilic granulomatosis with polyangiitis

Beatrice Maranini et al. Front Immunol. .

Abstract

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by necrotizing inflammation of small- and medium-sized blood vessels and the presence of circulating ANCA. Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic ANCA-associated vasculitis, characterized by peripheral eosinophilia, neuropathy, palpable purpuras or petechiae, renal and cardiac involvement, sinusitis, asthma, and transient pulmonary infiltrates. Middle lobe syndrome (MLS) is defined as recurrent or chronic atelectasis of the right middle lobe of the lung, and it is a potential complication of asthma.

Case presentation: Herein, we describe a case of MLS in a 51-year-old woman, never-smoker, affected by EGPA, presenting exclusively with leukocytosis and elevated concentrations of acute-phase proteins, without any respiratory symptom, cough, or hemoptysis. Chest computed tomography (CT) imaging documented complete atelectasis of the middle lobe, together with complete obstruction of lobar bronchial branch origin. Fiberoptic bronchoscopy (FOB) revealed complete stenosis of the middle lobar bronchus origin, thus confirming the diagnosis of MLS, along with distal left main bronchus stenosis. Bronchoalveolar lavage (BAL) did not detect any infection. Bronchial biopsies included plasma cells, neutrophil infiltrates, only isolated eosinophils, and no granulomas, providing the hypothesis of vasculitic acute involvement less likely. First-line agents directed towards optimizing pulmonary function (mucolytics, bronchodilators, and antibiotic course) were therefore employed. However, the patient did not respond to conservative treatment; hence, endoscopic management of airway obstruction was performed, with chest CT documenting resolution of middle lobe atelectasis.

Conclusion: To the best of our knowledge, this is the first detailed description of MLS in EGPA completely resolved through FOB. Identification of MLS in EGPA appears essential as prognosis, longitudinal management, and treatment options may differ from other pulmonary involvement in AAV patients.

Keywords: Churg–Strauss; EGPA; asthma; eosinophilic granulomatosis with polyangiitis; middle lobe syndrome.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
High-resolution chest CT showing a wedge-shaped density extending from the hilum anteriorly and inferiorly towards the chest wall, confirming the presence of middle lobe syndrome [red arrow in (A), axial view, and (B), sagittal view]. Coronal view (C) shows polypoid-like neoformation of the anterior wall of the left common bronchus (red arrowhead), confirmed in axial view (D, red arrowhead). High-resolution chest CT performed 15 days after the bronchodilation procedure documented regression of the middle lobe atelectasis (E, axial view).
Figure 2
Figure 2
On bronchoscopy, the right main bronchus was partially obstructed by fibrotic stenosis (A). (B) shows bronchoscopic images after dilatation and 1 month later showing complete healing of bronchial tear (C).
Figure 3
Figure 3
Microscopic examination revealed: (A) low magnification and (B) high magnification of hematoxylin and eosin staining, showing plasma cell infiltrate in the absence of eosinophils and vasculitis. Since main differential diagnosis includes lymphoma, kappa and lambda light chain immunostains were needed. Immunoglobulin free light chain (IgLC) levels in lung tissue were determined by immunohistochemistry, and no abnormalities were observed both at (C) low and (D) high magnification.
Figure 4
Figure 4
Main points to consider in AAV patients at risk of MLS development. BS, bronchial stenosis; CT, computed tomography; FOB, fiberoptic bronchoscopy; SGS, subglottic stenosis; TB, tracheobronchial. The image was created with BioRender.
See this image and copyright information in PMC

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