Case Report: Middle Lobe Syndrome: a Rare Presentation in Eosinophilic Granulomatosis with Polyangiitis

Overview

Journal Front Immunol

Specialty Allergy & Immunology

Date 2023 Aug 28

PMID 37638004

Authors

Beatrice Maranini

Ippolito Guzzinati

Gian Luca Casoni

Maria Ballotta

Andrea Lo Monaco

Marcello Govoni

Affiliations

Soon will be listed here.

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.

References

Rahman N, Senanayake E, Mascaro J, Situnayake D, Bishay E, Patel A . Recurrent endobronchial occlusion and aorto-bronchial fistula formation in Behcet's disease. J Cardiothorac Surg. 2023; 18(1):22. PMC: 9838001. DOI: 10.1186/s13019-023-02145-0. View

Latorre M, Baldini C, Seccia V, Pepe P, Novelli F, Celi A . Asthma Control and Airway Inflammation in Patients with Eosinophilic Granulomatosis with Polyangiitis. J Allergy Clin Immunol Pract. 2016; 4(3):512-9. DOI: 10.1016/j.jaip.2015.12.014. View

Lin X, Lin Y, Lai Z, Wei S, Qiu M, Li J . Retrospective comparison of high-resolution computed tomography of eosinophilic granulomatosis with polyangiitis with severe asthma. Ann Transl Med. 2021; 9(12):983. PMC: 8267300. DOI: 10.21037/atm-21-2243. View

GRAHAM E, BURFORD T, MAYER J . Middle lobe syndrome. Postgrad Med. 1948; 4(1):29-34. DOI: 10.1080/00325481.1948.11693655. View

Gupta S, Siddiqui S, Haldar P, Raj J, Entwisle J, Wardlaw A . Qualitative analysis of high-resolution CT scans in severe asthma. Chest. 2009; 136(6):1521-1528. PMC: 2816977. DOI: 10.1378/chest.09-0174. View

Mi W, Zhang C, Wang H, Cao J, Li C, Yang L . Measurement and analysis of the tracheobronchial tree in Chinese population using computed tomography. PLoS One. 2015; 10(4):e0123177. PMC: 4404098. DOI: 10.1371/journal.pone.0123177. View

Sheski F, Mathur P . Long-term results of fiberoptic bronchoscopic balloon dilation in the management of benign tracheobronchial stenosis. Chest. 1998; 114(3):796-800. DOI: 10.1378/chest.114.3.796. View

Jennette J, Falk R, Bacon P, Basu N, Cid M, Ferrario F . 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2012; 65(1):1-11. DOI: 10.1002/art.37715. View

Garcia-Valladares I, Espinoza L . Subglottic stenosis is a form of limited Wegener's granulomatosis. J Rheumatol. 2011; 38(10):2268. DOI: 10.3899/jrheum.110371. View

10.

Ito M, Yasuo M, Yamamoto H, Tsushima K, Tanabe T, Yokoyama T . Central airway stenosis in a patient with autoimmune pancreatitis. Eur Respir J. 2009; 33(3):680-3. DOI: 10.1183/09031936.00051408. View

11.

Yamasaki A, Tomita K, Chikumi H, Tatsukawa T, Shigeoka Y, Nakamoto M . Lung cancer arising in association with middle lobe syndrome. Anticancer Res. 2006; 26(3B):2213-6. View

12.

Stern E, Graham C, Webb W, Gamsu G . Normal trachea during forced expiration: dynamic CT measurements. Radiology. 1993; 187(1):27-31. DOI: 10.1148/radiology.187.1.8451427. View

13.

Sacoto G, Boukhlal S, Specks U, Flores-Suarez L, Cornec D . Lung involvement in ANCA-associated vasculitis. Presse Med. 2020; 49(3):104039. DOI: 10.1016/j.lpm.2020.104039. View

14.

Gudbjartsson T, Gudmundsson G . Middle lobe syndrome: a review of clinicopathological features, diagnosis and treatment. Respiration. 2012; 84(1):80-6. DOI: 10.1159/000336238. View

15.

Livi V, Cancellieri A, Patelli M, Trisolini R . Tracheobronchial involvement in Churg-Strauss syndrome. J Bronchology Interv Pulmonol. 2012; 19(1):81-2. DOI: 10.1097/LBR.0b013e3182422dd1. View

16.

Puchalski J, Musani A . Tracheobronchial stenosis: causes and advances in management. Clin Chest Med. 2013; 34(3):557-67. DOI: 10.1016/j.ccm.2013.05.002. View

17.

Fowler N, Beach J, Krakovitz P, Spalding S . Airway manifestations in childhood granulomatosis with polyangiitis (Wegener's). Arthritis Care Res (Hoboken). 2011; 64(3):434-40. DOI: 10.1002/acr.21565. View

18.

Tian Y, Liu N, Yin H, Duan L . Relationship Between C-Reactive Protein/Serum Albumin Ratio, Neutrophil/Lymphocyte Ratio, and ANCA-Associated Vasculitis Activity: A Retrospective Single Center Cohort Study. Front Med (Lausanne). 2022; 9:855869. PMC: 8971710. DOI: 10.3389/fmed.2022.855869. View

19.

Taylor S, Clayburgh D, Rosenbaum J, Schindler J . Clinical manifestations and treatment of idiopathic and Wegener granulomatosis-associated subglottic stenosis. JAMA Otolaryngol Head Neck Surg. 2013; 139(1):76-81. DOI: 10.1001/jamaoto.2013.1135. View

20.

Polychronopoulos V, Prakash U, Golbin J, Edell E, Specks U . Airway involvement in Wegener's granulomatosis. Rheum Dis Clin North Am. 2007; 33(4):755-75, vi. DOI: 10.1016/j.rdc.2007.09.004. View