Mimicking hypersensitivity pneumonitis as an uncommon initial presentation of chronic granulomatous disease in children
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LETTER TO THE EDITOR
Open Access
Mimicking hypersensitivity pneumonitis as an uncommon initial presentation of chronic granulomatous disease in children Hui Liu1, Jinrong Liu1, Huimin Li1, Yun Peng2 and Shunying Zhao1*
Abstract Dry cough, dyspenea and diffuse centrilobular nodules in both lungs of radiologic findings similar to hypersensitivity pneumonitis (HP) are rare initial presentation in chronic granulomatous disease (CGD). CGD is remarkable for increased susceptibility to bacterial and fungal infections as well as high sensitivity to inciting antigens such as Aspergillus species due to dysregulated inflammation. We identified three children who had an initial presentation mimicking HP and were subsequently diagnosed as CGD. All patients developed invasive pulmonary A. fumigatus infection (IPAI) following systemic glucocorticoid therapy. Two of the three patients were found to have mutations in NCF1 gene and one patient in NCF2 gene. As HP is uncommon in children, we should consider the possibility of CGD in children with HP, even in mimicking HP patients with suggestive inhalation history and negative fungal cultures. A prompt diagnosis of CGD is essential to enable initiation of prophylactic antibacterial and antifungal therapies. Keywords: Chronic granulomatous disease, Hypersensitivity pneumonitis, A. fumigatus, Glucocorticoid, Children
Letter to the editor Chronic granulomatous disease (CGD) is characterized by recurrent and severe bacterial and fungal infections as well as excessive inflammation, which are most prominent in gastrointestinal and genitourinary tracts, such as granulomata mimicking Crohn’s disease [1]. An exuberant respiratory inflammation induced by the exposure to inciting antigens and clinically manifested as hypersensitivity pneumonitis (HP) or allergic bronchopulmonary aspergillosis (ABPA) has also been described in CGD [2–7]. However, HP as an initial presentation of CGD is uncommon and has never been reported. Here we review three children who had an initial presentation mimicking HP, developed invasive pulmonary A. fumigatus infection (IPAI) following systemic glucocorticoid therapy and were subsequently diagnosed as CGD.
* Correspondence: [email protected] 1 Department of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Nanlishi Road 56, Xicheng District, Beijing, China Full list of author information is available at the end of the article
Case presentation Case 1
A 4-year-old boy was admitted to the hospital on September 8, 2011 after 3 weeks of dry cough, progressive dyspnea and fever. He lived in a fruit stall with many rotten fruits inside. He had a history of pneumonia at 3 months old. He also had a history of severe eczema and seasonal rhinitis at one year old. On admission, his oxygen saturation at rest was 92%, and decreased to 86% after walking. Bilateral basilar rales were noted on auscultation. Chest high-resolution CT (HRCT) scan showed diffuse nodular opacities and slight ground-glass (
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