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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

Findings for Case 3. Chest radiography (A) and chest computed tomography scan (B) revealed the presence of an extensive pneumomediastinum, a pneumothorax of the right lung, and diffuse ground-glass opacities and consolidation in both lungs. Follow-up chest radiography on day 72 of admission revealed an increased level of bilateral infiltrates (C). Histological section (D) obtained from a postmortem biopsy showed extensive interstitial fibrosis with hyperplastic type II pneumocytes and hyaline membrane formation, suggesting the fibrotic phase of diffuse alveolar damage. Bronchiolar destruction and mild bronchiolar obliteration are present. (Hematoxylin and eosin staining, ×400 magnification)
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Figure 2: Findings for Case 3. Chest radiography (A) and chest computed tomography scan (B) revealed the presence of an extensive pneumomediastinum, a pneumothorax of the right lung, and diffuse ground-glass opacities and consolidation in both lungs. Follow-up chest radiography on day 72 of admission revealed an increased level of bilateral infiltrates (C). Histological section (D) obtained from a postmortem biopsy showed extensive interstitial fibrosis with hyperplastic type II pneumocytes and hyaline membrane formation, suggesting the fibrotic phase of diffuse alveolar damage. Bronchiolar destruction and mild bronchiolar obliteration are present. (Hematoxylin and eosin staining, ×400 magnification)

Mentions: A previously healthy 35-month-old girl was referred to our medical center for severe dyspnea. Four weeks prior to admission, she had developed a mild cough, and her oral intake had become poor. Four days prior to admission, she had developed sudden onset dyspnea and subcutaneous emphysema. On admission, she presented with marked tachypnea (respiratory rate, 80/min) and chest retraction, and rales were audible in both lungs on auscultation. Radiography and a CT scan of the chest showed an extensive pneumomediastinum, a pneumothorax in the right hemithorax, and diffuse ground-glass opacities with consolidation in both lungs (Fig. 2A and 2B). Her total leukocyte count was 9,300/µL, with 66% neutrophils and 27% lymphocytes. Blood, sputum, and BAL cultures for bacteria and fungi were negative. Multiplex RT-PCR of nasopharyngeal aspirates revealed the presence of respiratory syncytial virus (RSV). She was admitted to the intensive care unit and treated with steroids, cyclophosphamide, hydroxychloroquine, and broad-spectrum antibiotics. Her response to treatment was poor, and her condition deteriorated rapidly. On the third day of admission, mechanical ventilation was required for impending respiratory failure. Repeated chest radiography revealed the presence of increased opacities in both lungs (Fig. 2C). She then developed refractory hypoxemic and hypercarbic respiratory failure with subsequent multiorgan dysfunction. Despite the administration of antifibrotics and supportive therapy, she died 70 days after admission. A postmortem lung biopsy revealed the features of the fibrotic phase of DAD with occasional hyaline membrane and type II pneumocyte hyperplasia (Fig. 2D).


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)

Findings for Case 3. Chest radiography (A) and chest computed tomography scan (B) revealed the presence of an extensive pneumomediastinum, a pneumothorax of the right lung, and diffuse ground-glass opacities and consolidation in both lungs. Follow-up chest radiography on day 72 of admission revealed an increased level of bilateral infiltrates (C). Histological section (D) obtained from a postmortem biopsy showed extensive interstitial fibrosis with hyperplastic type II pneumocytes and hyaline membrane formation, suggesting the fibrotic phase of diffuse alveolar damage. Bronchiolar destruction and mild bronchiolar obliteration are present. (Hematoxylin and eosin staining, ×400 magnification)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Findings for Case 3. Chest radiography (A) and chest computed tomography scan (B) revealed the presence of an extensive pneumomediastinum, a pneumothorax of the right lung, and diffuse ground-glass opacities and consolidation in both lungs. Follow-up chest radiography on day 72 of admission revealed an increased level of bilateral infiltrates (C). Histological section (D) obtained from a postmortem biopsy showed extensive interstitial fibrosis with hyperplastic type II pneumocytes and hyaline membrane formation, suggesting the fibrotic phase of diffuse alveolar damage. Bronchiolar destruction and mild bronchiolar obliteration are present. (Hematoxylin and eosin staining, ×400 magnification)
Mentions: A previously healthy 35-month-old girl was referred to our medical center for severe dyspnea. Four weeks prior to admission, she had developed a mild cough, and her oral intake had become poor. Four days prior to admission, she had developed sudden onset dyspnea and subcutaneous emphysema. On admission, she presented with marked tachypnea (respiratory rate, 80/min) and chest retraction, and rales were audible in both lungs on auscultation. Radiography and a CT scan of the chest showed an extensive pneumomediastinum, a pneumothorax in the right hemithorax, and diffuse ground-glass opacities with consolidation in both lungs (Fig. 2A and 2B). Her total leukocyte count was 9,300/µL, with 66% neutrophils and 27% lymphocytes. Blood, sputum, and BAL cultures for bacteria and fungi were negative. Multiplex RT-PCR of nasopharyngeal aspirates revealed the presence of respiratory syncytial virus (RSV). She was admitted to the intensive care unit and treated with steroids, cyclophosphamide, hydroxychloroquine, and broad-spectrum antibiotics. Her response to treatment was poor, and her condition deteriorated rapidly. On the third day of admission, mechanical ventilation was required for impending respiratory failure. Repeated chest radiography revealed the presence of increased opacities in both lungs (Fig. 2C). She then developed refractory hypoxemic and hypercarbic respiratory failure with subsequent multiorgan dysfunction. Despite the administration of antifibrotics and supportive therapy, she died 70 days after admission. A postmortem lung biopsy revealed the features of the fibrotic phase of DAD with occasional hyaline membrane and type II pneumocyte hyperplasia (Fig. 2D).

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