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Two series of familial cases with unclassified interstitial pneumonia with fibrosis.

Lee E, Seo JH, Kim HY, Yu J, Song JW, Park YS, Jang SJ, Do KH, Kwon J, Park SW, Park JH, Hong SJ - Allergy Asthma Immunol Res (2012)

Bottom Line: Their clinical and radiological findings were similar to those of acute interstitial pneumonia, but there were some differences in the pathological findings.Some of the individual cases showed rapidly progressive respiratory failure of unknown cause, with comparable clinical courses and similar radiological and pathological features, including lung fibrosis.Each family member was affected almost simultaneously in the spring, different kinds of viruses were detected in two patients, and all members were negative for bacterial infection, environmental and occupational agents, drugs, and radiation.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Asan Medical Center Children's Hospital, Seoul, Korea.

ABSTRACT
Several children presenting with mild symptoms of respiratory tract infection were diagnosed with unclassified interstitial pneumonia with fibrosis. Their clinical and radiological findings were similar to those of acute interstitial pneumonia, but there were some differences in the pathological findings. Unclassified interstitial pneumonia with fibrosis is characterized by histological findings of centrilobular distribution of alveolar damage and bronchiolar destruction with bronchiolar obliteration. This report describes two different series of familial cases of unclassified interstitial pneumonia with fibrosis, which developed almost simultaneously in the spring. Some of the individual cases showed rapidly progressive respiratory failure of unknown cause, with comparable clinical courses and similar radiological and pathological features, including lung fibrosis. Each family member was affected almost simultaneously in the spring, different kinds of viruses were detected in two patients, and all members were negative for bacterial infection, environmental and occupational agents, drugs, and radiation. These findings implicate a viral infection and/or processes related to a viral infection, such as an exaggerated or altered immune response, or an unknown inhaled environmental agent in the pathogenesis of unclassified interstitial pneumonia with fibrosis.

No MeSH data available.


Related in: MedlinePlus

Chest radiography and biopsy specimens for Case 1. Chest radiography (A) on day 2 of the exacerbation of cough and dyspnea. Chest computed tomography (CT) scan (B) on admission revealed the presence of ground-glass attenuation with diffuse centrinodular opacity in both lungs. Pre-discharge chest radiography (C) and chest CT (D) showed a reduction in the extent of ground-glass opacities in both lungs. Biopsy specimens (E) obtained on day 14 of the exacerbation of symptoms. Alveolar pneumocyte hyperplasia (arrow) and interstitial fibroblastic proliferation are features consistent with the organizing phase of diffuse alveolar damage. Bronchioles are destructed and obliterated by fibroblastic proliferation (asterisk). (Hematoxylin and eosin staining, ×200 magnification)
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Figure 1: Chest radiography and biopsy specimens for Case 1. Chest radiography (A) on day 2 of the exacerbation of cough and dyspnea. Chest computed tomography (CT) scan (B) on admission revealed the presence of ground-glass attenuation with diffuse centrinodular opacity in both lungs. Pre-discharge chest radiography (C) and chest CT (D) showed a reduction in the extent of ground-glass opacities in both lungs. Biopsy specimens (E) obtained on day 14 of the exacerbation of symptoms. Alveolar pneumocyte hyperplasia (arrow) and interstitial fibroblastic proliferation are features consistent with the organizing phase of diffuse alveolar damage. Bronchioles are destructed and obliterated by fibroblastic proliferation (asterisk). (Hematoxylin and eosin staining, ×200 magnification)

Mentions: A previously healthy 2-year-old boy presented with a 1-month history of cough and dyspnea. He had had a mild cough for 1 week before a sudden deterioration in his condition. The dyspnea increased despite treatment, and he was transferred to the Department of Pediatrics at the Asan Medical Center for further management. On arrival, he was apyrexial and tachypneic (respiratory rate, 66/min). Mild subcostal retraction was observed, and coarse breath sounds without crackles were noted on auscultation. Arterial blood gas analysis (ABGA) showed mild hypoxemia. His white blood cell (WBC) count was 14,300/µL, with 55.8% neutrophils and 36.3% lymphocytes. Radiography and computed tomography (CT) of the chest revealed the presence of fine peribronchial ground-glass opacities in both lungs (Fig. 1A and 1B). Blood, bronchoalveolar lavage (BAL) fluid, and sputum cultures were negative for bacteria, viruses, and fungi. A lung biopsy performed on day 3 of admission showed the organizing phase of DAD distributed mainly in the centrilobular area, with destruction and obliteration of bronchioles by fibroblasts (Fig. 1E). The patient was administered intravenous corticosteroids (2 mg/kg/day), followed by oral prednisolone (which was gradually tapered), hydroxychloroquine, and oral cyclophosphamide. His condition gradually improved, although exercise intolerance persisted. At the 1-year follow-up, a repeat CT scan of the chest revealed a decrease in the extent of ground-glass opacities in the affected areas of both lungs (Fig. 1C and 1D).


Two series of familial cases with unclassified interstitial pneumonia with fibrosis.

Lee E, Seo JH, Kim HY, Yu J, Song JW, Park YS, Jang SJ, Do KH, Kwon J, Park SW, Park JH, Hong SJ - Allergy Asthma Immunol Res (2012)

Chest radiography and biopsy specimens for Case 1. Chest radiography (A) on day 2 of the exacerbation of cough and dyspnea. Chest computed tomography (CT) scan (B) on admission revealed the presence of ground-glass attenuation with diffuse centrinodular opacity in both lungs. Pre-discharge chest radiography (C) and chest CT (D) showed a reduction in the extent of ground-glass opacities in both lungs. Biopsy specimens (E) obtained on day 14 of the exacerbation of symptoms. Alveolar pneumocyte hyperplasia (arrow) and interstitial fibroblastic proliferation are features consistent with the organizing phase of diffuse alveolar damage. Bronchioles are destructed and obliterated by fibroblastic proliferation (asterisk). (Hematoxylin and eosin staining, ×200 magnification)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3378931&req=5

Figure 1: Chest radiography and biopsy specimens for Case 1. Chest radiography (A) on day 2 of the exacerbation of cough and dyspnea. Chest computed tomography (CT) scan (B) on admission revealed the presence of ground-glass attenuation with diffuse centrinodular opacity in both lungs. Pre-discharge chest radiography (C) and chest CT (D) showed a reduction in the extent of ground-glass opacities in both lungs. Biopsy specimens (E) obtained on day 14 of the exacerbation of symptoms. Alveolar pneumocyte hyperplasia (arrow) and interstitial fibroblastic proliferation are features consistent with the organizing phase of diffuse alveolar damage. Bronchioles are destructed and obliterated by fibroblastic proliferation (asterisk). (Hematoxylin and eosin staining, ×200 magnification)
Mentions: A previously healthy 2-year-old boy presented with a 1-month history of cough and dyspnea. He had had a mild cough for 1 week before a sudden deterioration in his condition. The dyspnea increased despite treatment, and he was transferred to the Department of Pediatrics at the Asan Medical Center for further management. On arrival, he was apyrexial and tachypneic (respiratory rate, 66/min). Mild subcostal retraction was observed, and coarse breath sounds without crackles were noted on auscultation. Arterial blood gas analysis (ABGA) showed mild hypoxemia. His white blood cell (WBC) count was 14,300/µL, with 55.8% neutrophils and 36.3% lymphocytes. Radiography and computed tomography (CT) of the chest revealed the presence of fine peribronchial ground-glass opacities in both lungs (Fig. 1A and 1B). Blood, bronchoalveolar lavage (BAL) fluid, and sputum cultures were negative for bacteria, viruses, and fungi. A lung biopsy performed on day 3 of admission showed the organizing phase of DAD distributed mainly in the centrilobular area, with destruction and obliteration of bronchioles by fibroblasts (Fig. 1E). The patient was administered intravenous corticosteroids (2 mg/kg/day), followed by oral prednisolone (which was gradually tapered), hydroxychloroquine, and oral cyclophosphamide. His condition gradually improved, although exercise intolerance persisted. At the 1-year follow-up, a repeat CT scan of the chest revealed a decrease in the extent of ground-glass opacities in the affected areas of both lungs (Fig. 1C and 1D).

Bottom Line: Their clinical and radiological findings were similar to those of acute interstitial pneumonia, but there were some differences in the pathological findings.Some of the individual cases showed rapidly progressive respiratory failure of unknown cause, with comparable clinical courses and similar radiological and pathological features, including lung fibrosis.Each family member was affected almost simultaneously in the spring, different kinds of viruses were detected in two patients, and all members were negative for bacterial infection, environmental and occupational agents, drugs, and radiation.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Asan Medical Center Children's Hospital, Seoul, Korea.

ABSTRACT
Several children presenting with mild symptoms of respiratory tract infection were diagnosed with unclassified interstitial pneumonia with fibrosis. Their clinical and radiological findings were similar to those of acute interstitial pneumonia, but there were some differences in the pathological findings. Unclassified interstitial pneumonia with fibrosis is characterized by histological findings of centrilobular distribution of alveolar damage and bronchiolar destruction with bronchiolar obliteration. This report describes two different series of familial cases of unclassified interstitial pneumonia with fibrosis, which developed almost simultaneously in the spring. Some of the individual cases showed rapidly progressive respiratory failure of unknown cause, with comparable clinical courses and similar radiological and pathological features, including lung fibrosis. Each family member was affected almost simultaneously in the spring, different kinds of viruses were detected in two patients, and all members were negative for bacterial infection, environmental and occupational agents, drugs, and radiation. These findings implicate a viral infection and/or processes related to a viral infection, such as an exaggerated or altered immune response, or an unknown inhaled environmental agent in the pathogenesis of unclassified interstitial pneumonia with fibrosis.

No MeSH data available.


Related in: MedlinePlus