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Bilateral hilar opacities

  • João A. Cunha Neves
    Affiliations
    Gastroenterology Department, Serviço de Gastrenterologia, Centro Hospitalar Universitário do Algarve – Unidade de Portimão, Estrada do Poço Seco, 8500-338 Portimão, Portugal
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  • Maria Margarida Rosado
    Correspondence
    Corresponding author.
    Affiliations
    Internal Medicine Department, Serviço de Medicina Interna, Centro Hospitalar Universitário do Algarve – Unidade de Portimão, Estrada do Poço Seco, 8500-338 Portimão, Portugal
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  • Marcelo Gordinho
    Affiliations
    Intensive Care Medicine Department, Serviço de Medicina Intensiva, Centro Hospitalar Universitário do Algarve – Unidade de Portimão, Estrada do Poço Seco, 8500-338 Portimão, Portugal
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Published:September 23, 2020DOI:https://doi.org/10.1016/j.ejim.2020.09.010

      Keywords

      1. Case description

      A 20-year-old male was admitted to the emergency department with a 1-week history of fever, right pleuritic chest pain and aggravating sporadic hemoptoic cough.
      The patient mentioned oral aphthous ulcers more than 3 times per year and recurrent genital aphthous ulcers since puberty. He had a past medical history of multiple episodes of conjunctivitis, acne and skin lesions compatible with erythema nodosum. He denied pathergy reaction.
      Physical examination was unremarkable.
      Laboratory tests revealed anaemia [Haemoglobin: 10.8 g/dL (13.5-18.0 g/dL)], leukocytosis [Leukocytes: 20.8×103/µL (4.0-10.0×103/µL)] with neutrophilia [14.8×103/µL (2.0-8.0×103/µL)] and elevation of C-reactive protein [176.7 mg/L (0-5.0 mg/L)].
      Chest X-Ray (CXR) (Fig. 1A) showed bilateral well-defined rounded hilar opacities of large dimensions. A chest Computed Tomography (CT) (Fig. 1B) was performed. What is the diagnosis?
      Fig. 1
      Fig. 1Panel A. CXR showing rounded bilateral hilar opacities. Panel B. Chest CT showing multiple pulmonary artery aneurysms.

      2. Discussion

      The chest CT revealed multiple pulmonary artery aneurysms (PAAs), the largest located in the right middle lobe and superior portion of the left inferior lobe, both with a diameter of approximately 6 centimetres and variable degrees of mural thrombosis (Fig. 1B). Clinical history, CXR and CT lead to the diagnosis of multiple PAAs secondary to Behçet's syndrome.
      Behçet's syndrome is a multisystemic condition of unknown etiology, characterized by recurrent oral and genital ulcers and ocular involvement [
      • Yazici H
      • Seyahi E
      • Hatemi G
      • Yazici Y.
      Behçet syndrome: a contemporary view.
      ].
      Pulmonary involvement has a reported prevalence of 1-7.7%. PAAs are the most common finding and are mainly located in the right lower lobar artery, even though they can be located anywhere in the pulmonary arterial tree [

      Erkan F, Gül A, Tasali E. Pulmonary manifestations of Behçet's disease 2001:572–8.

      ].
      PAAs usually manifest with hemoptysis, which can be massive and life threatening. Fever, pleuritic chest pain, dyspnea and/or cough, may also be present. Scarcely, they can also be asymptomatic [
      • Yazici H
      • Seyahi E
      • Hatemi G
      • Yazici Y.
      Behçet syndrome: a contemporary view.
      ].
      Uni or bilateral hilar opacities are the most frequent finding on CXR, but the definitive diagnosis is achieved through chest CT [

      Erkan F, Gül A, Tasali E. Pulmonary manifestations of Behçet's disease 2001:572–8.

      ].
      The presence of PAAs in the main or lobar branches of a young man is almost unique to Behçet's syndrome.
      According to EULAR recommendations, the medical management of PAAs consists of high dose glucocorticoids and cyclophosphamide. Surgical indications are not well established and surgery should not be undertaken except in life-threatening situations [
      • Hatemi G
      • Christensen R
      • Bang D
      • Bodaghi B
      • Celik AF
      • Fortune F
      • et al.
      2018 Update of the EULAR recommendations for the management of Behçet's syndrome.
      ].
      Our patient underwent medical treatment with resolution of symptoms.

      CRediT authorship contribution statement

      João A. Cunha Neves: Writing - original draft. Maria Margarida Rosado: Writing - original draft. Marcelo Gordinho: Writing - original draft.

      Declaration of Competing Interest

      The authors report no conflicts of interest or funding from any organization.

      References

        • Yazici H
        • Seyahi E
        • Hatemi G
        • Yazici Y.
        Behçet syndrome: a contemporary view.
        Nat Rev Rheumatol. 2018; 14: 107-119
      1. Erkan F, Gül A, Tasali E. Pulmonary manifestations of Behçet's disease 2001:572–8.

        • Hatemi G
        • Christensen R
        • Bang D
        • Bodaghi B
        • Celik AF
        • Fortune F
        • et al.
        2018 Update of the EULAR recommendations for the management of Behçet's syndrome.
        Ann Rheum Dis. 2018; 77: 808-818