Eosinophilic granulomatosis with polyangiitis: A rare case of central airway stenosis with normal eosinophil levels

Li, Long-zhao; Sun, Kai; Wang, Hong-wu

doi:10.1016/j.medcle.2024.03.015

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(A) Bronchoscope showed obvious granulation tissue hyperplasia (red arrow) and central airway stenosis. (B) Central airway biopsy (hematoxylin–eosin staining) showed that eosinophil infiltration in blood vessels and tissues was very obvious (300×, eosinophils are shown by red arrows)." ] ] ] "textoCompleto" => "Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis, which usually manifests as eosinophilia and damage to respiratory system, urinary system and circulatory system. Because EGPA can involve many tissues in the whole body, other special manifestations may appear, which usually leads to mistakes in diagnosis and treatment. This paper reports a special case of EGPA. The patient suffered from acute asphyxia due to the stenosis of central airway area I<a class="elsevierStyleCrossRef" href="#bib0025">1</a> caused by EGPA, and the eosinophil laboratory count was normal. After emergency local interventional therapy, the patient's symptoms were relieved.A 54-year-old male patient came to our respiratory center and complained of severe breath holding, cough and excessive phlegm. His medical history includes recurrent asthma, dyspnea and recurrent low fever within 3 years, long-term use of inhaled β-2 receptor agonist and oral prednisone acetate tablets of 10mg. Laboratory examination 3 years ago: EOS%: 29.6%, EOS: 2.66×109/L.During the physical examination, the auscultation of both lungs showed that the breathing sounds of both lungs were low, and the vital signs showed that the body temperature was 37.5°C. After oxygen inhalation, the oxygen saturation was only 92%, and nothing else was abnormal. Computed tomography showed airway stenosis, lung infiltration and consolidation of both lungs. The patient underwent emergency bronchoscopy, and the results showed severe glottic edema and stenosis. Granulation hyperplasia and stenosis were observed in the central airway (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A) and right main bronchus. In order to relieve the patient's asphyxia, we performed balloon dilation of airway, inflated a 10-30mm micro-technical balloon to a pressure of 2bar for 30s, and performed biopsy on airway granuloma. Although this intervention relieved the asphyxia, the patient's symptoms still existed.<elsevierMultimedia ident="fig0005"></elsevierMultimedia>Further laboratory examination showed that the patient's urine routine and blood routine were normal, and the white blood cell: 9.04×109/L eosinophil: 0.04×109/L. Antibody detection showed that both C-ANCA and PR-3 were negative, and the histological evaluation of granuloma showed that submucosal granuloma was formed, and eosinophils aggregated after amplification (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Combined with the past history of eosinophilia, imaging manifestations, pathological eosinophilic aggregation and objective evidence of airway granulation obstruction, we are more inclined to diagnose EGPA. The patient began to receive a high dose of glucocorticoids of 70mg per day, and cyclophosphamide (0.6g/m2 per pulse) was injected once. After evaluating the patients, we thought it is necessary to carry out local shovel cutting to quickly relieve suffocation, and inject triamcinolone acetonide 20mg near two granulomas in the airway respectively. The patient's wheezing symptoms disappeared, and his cough and expectoration were significantly reduced. Two weeks later, we injected cyclophosphamide again, and gradually reduced the dosage of glucocorticoid to 20mg per day, and then to 10mg per day after 4 weeks. After two months of follow-up, the patient didn’t suffer from wheezing again.EGPA is a rare autoimmune disease, which usually involves the lungs. The main symptoms are asthma and cough, with pathological infiltration of lower respiratory tract, ground glass shadow and thickening of bronchial wall.<a class="elsevierStyleCrossRef" href="#bib0030">2</a> However, this patient showed the central airway stenosis and normal eosinophils caused by granuloma, which is rare. Interestingly, the patient's eosinophils did not increase, and granuloma still progressed. This may be related to the long-term use of glucocorticoids. Many literatures suggest that the false normal of eosinophils will be secondary to the extensive use of glucocorticoids.<a class="elsevierStyleCrossRefs" href="#bib0030">2,3</a>Regarding the treatment of EGPA, in addition to the use of glucocorticoids and cyclophosphamide recommended by the guidelines,<a class="elsevierStyleCrossRef" href="#bib0040">4</a> we have carried out local interventional therapy. This is because of the particularity of granuloma in the central airway, the patient has already appeared asphyxia symptoms. After evaluation, we think that emergency bronchial intervention is necessary. Bronchoscope shovel cutting and hormone injection have achieved good results. We followed up for 2 months and the patient did not develop wheezing symptoms.We report a case of EGPA with normal eosinophils and central airway stenosis. Its special clinical features will mislead doctors’ judgment. Past medical history and pathological results play an important role in diagnosis. Bronchoscopic interventional therapy has a good effect on acute airway stenosis, but the long-term benefit of the disease needs further study.Authors’ contributionsLi Long-zhao: Design and draft articles, and get the approval of the final version.Sun Kai: Organize medical records, consult materials and make key revisions.Wang Hong-wu: Consent to assume responsibility for all facets of the manuscript, offer guidance on manuscript-related matters, and ultimately be responsible for the review and approval of the manuscript.Informed consent statementParticipant was fully informed of the study and gave their informed written consent before participation. This study was conducted in accordance with the Declaration of Helsinki.Data availability statementThe data that support the findings of this study are available from the corresponding author upon reasonable request.Consent for publicationAll authors agree to the publication of this article.Ethical explanationAll the procedures in this study were implemented in accordance with relevant laws and institutional guidelines, and have been approved by the corresponding institutional committees and informed consent of patients. Ethical examination and approval is approved by the Ethics Committee of Dongzhimen Hospital of Beijing University of Chinese Medicine (Project No.: 2023DZMEC-484-01), and the approval time is October 12, 2023.FundingBeijing Tongzhou District Science and Technology Plan Project provided financial support and article publishing expenses, and the project number was KJ2022CX047.Conflict of interestNone." "textoCompletoSecciones" => array:1 [ "secciones" => array:8 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Authors’ contributions" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Informed consent statement" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Data availability statement" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Consent for publication" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Ethical explanation" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Funding" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflict of interest" ] 7 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 722 "Ancho" => 1450 "Tamanyo" => 336369 ] ] "descripcion" => array:1 [ "en" => "EGPA tracheoscopy and pathological findings of a case of central airway stenosis. 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Dubey" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.autrev.2022.103219" "Revista" => array:5 [ "tituloSerie" => "Autoimmun Rev" "fecha" => "2023" "volumen" => "22" "paginaInicial" => "103219" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/36283646" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0035" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "G. Trivioli" 1 => "B. Terrier" 2 => "A. Vaglio" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Rheumatology (Oxford)" "fecha" => "2020" "volumen" => "59" "numero" => "Suppl. 3" "paginaInicial" => "i84" "paginaFinal" => "i94" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0040" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Evidence-based guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "G. Emmi" 1 => "A. Bettiol" 2 => "E. Gelain" 3 => "I.M. Bajema" 4 => "A. Berti" 5 => "S. Burns" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1038/s41584-023-00958-w" "Revista" => array:6 [ "tituloSerie" => "Nat Rev Rheumatol" "fecha" => "2023" "volumen" => "19" "paginaInicial" => "378" "paginaFinal" => "393" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/37161084" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000016300000005/v1_202409110904/S2387020624003711/v1_202409110904/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000016300000005/v1_202409110904/S2387020624003711/v1_202409110904/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020624003711?idApp=UINPBA00004N"]

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Vol. 163. Issue 5.

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