Idiopathic interstitial pneumonias are currently classified into four categories: usual interstitial pneumonia, nonspecific interstitial pneumonia with fibrosis, acute interstitial pneumonia and desquamative interstitial pneumonia. The fibrotic process in interstitial pneumonias appears to result from a complex interaction between fibroblasts, other lung parenchymal cells and macrophages. The complex relationship between the local release of growth-promoting cytokines by alveolar macrophages and resident fibroblasts represents a necessary step for fibrosis or remodeling after lung injury. Injury to the epithelium and basement membranes is likely necessary for the fibrotic process to occur. Usual interstitial pneumonia, most frequent among interstitial pneumonias and has a poor prognosis, appears on high-resolution CT as patchy subpleural areas of ground-glass attenuation, irregular linear opacity, and honeycombing. Nonspecific interstitial pneumonia with fibrosis, the second most frequent and has a better prognosis than usual interstitial pneumonia, appears as subpleural patchy areas of ground-glass attenuation with associated areas of irregular linear opacity on CT. Acute interstitial pneumonia with high mortality rate presents as extensive bilateral airspace consolidation and patchy or diffuse bilateral areas of ground-glass attenuation. Desquamative interstitial pneumonia with good prognosis presents as patchy subpleural areas of ground-glass attenuation in middle and lower lung zones.
Human ; Lung Diseases, Interstitial/radiography ; Lung Diseases, Interstitial/physiopathology ; Lung Diseases, Interstitial/pathology*

Biopsy ; Diagnosis, Differential ; Humans ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; classification ; diagnostic imaging ; pathology ; Radiography

Chronic active Epstein-Barr virus (CAEBV) infection is characterized by persistent infectious mononucleosis-like symptoms, an unusual pattern of Epstein-Barr virus (EBV) antibodies, detection of the EBV genome in affected tissues or peripheral blood, and chronic illness that cannot be attributed to any other known disease. This is the first reported Korean case of an immunocompetent adult with CAEBV-associated interstitial pneumonitis. A 28-year-old female was admitted with a fever that persisted for 3 weeks. She had multiple lymphadenopathy, hepatosplenomegaly, pancytopenia, and elevated serum aminotransferase levels. Serology for antibodies was positive and chest computed tomography showed diffuse ground glass opacities in both lungs. Histopathology of the lung tissue showed lymphocyte infiltration, and EBV DNA was detected in those lymphocytes using in situ hybridization with an EBV-encoded RNA probe. After 1 month of hospitalization, she improved without specific treatment.
Adult ; Chronic Disease ; Epstein-Barr Virus Infections/complications/*pathology/radiography ; Female ; *Herpesvirus 4, Human ; Humans ; Immunocompetence ; Lung Diseases/etiology/pathology ; Lung Diseases, Interstitial/etiology/*pathology/radiography

Acute interstitial pneumonia (AIP) is a rapidly progressive condition of unknown cause that occurs in a previously healthy individual and produces the histologic findings of diffuse alveolar damage. Since the term AIP was first introduced in 1986, there have been very few case reports of AIP in children. Here we present a case of AIP in a 3-yr-old girl whose other two siblings showed similar radiologic findings. The patient was confirmed to have AIP from autopsy showing histological findings of diffuse alveolar damage and proliferation of fibroblasts. Her 3-yr-old brother was also clinically and radiologically highly suspected as having AIP, and the other asymptomatic 8-yr-old sister was radiologically suspected as having AIP.
Acute Disease ; Child, Preschool ; Family Health ; Fatal Outcome ; Female ; Humans ; Infant ; Lung Diseases, Interstitial/*pathology/*radiography ; Male ; Pulmonary Fibrosis/pathology/radiography ; Siblings ; *Tomography, X-Ray Computed

Pneumoconiosis was diagnosed by open lung biopsy in two dental technicians who had interstitial lung disease. Mineralogical analysis was performed to investigate the origin of the dust that had been inhaled. A marked accumulation of silicon and phosphorus was found in both cases. The hard metals chromium and cobalt were also found. Dental technician's pneumoconiosis is a complex pneumoconiosis in which such dust and hard metals may play a role.
Adult ; *Dental Technicians ; Human ; Lung/pathology ; Lung Diseases, Interstitial/*diagnosis/metabolism ; Male ; Minerals/metabolism ; Occupational Diseases/*diagnosis/metabolism ; Pneumoconiosis/*diagnosis/metabolism ; Radiography, Thoracic ; Tomography, X-Ray Computed

OBJECTIVE: To evaluate the usefulness of an automated system for quantification and discrimination of usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP). MATERIALS AND METHODS: An automated system to quantify six regional high-resolution CT (HRCT) patterns: normal, NL; ground-glass opacity, GGO; reticular opacity, RO; honeycombing, HC; emphysema, EMPH; and consolidation, CONS, was developed using texture and shape features. Fifty-four patients with pathologically proven UIP (n = 26) and pathologically proven NSIP (n = 28) were included as part of this study. Inter-observer agreement in measuring the extent of each HRCT pattern between the system and two thoracic radiologists were assessed in 26 randomly selected subsets using an interclass correlation coefficient (ICC). A linear regression analysis was used to assess the contribution of each disease pattern to the pulmonary function test parameters. The discriminating capacity of the system between UIP and NSIP was evaluated using a binomial logistic regression. RESULTS: The overall ICC showed acceptable agreement among the system and the two radiologists (r = 0.895 for the abnormal lung volume fraction, 0.706 for the fibrosis fraction, 0.895 for NL, 0.625 for GGO, 0.626 for RO, 0.893 for HC, 0.800 for EMPH, and 0.430 for CONS). The volumes of NL, GGO, RO, and EMPH contribute to forced expiratory volume during one second (FEV1) (r = 0.72, beta values, 0.84, 0.34, 0.34 and 0.24, respectively) and forced vital capacity (FVC) (r = 0.76, beta values, 0.82, 0.28, 0.21 and 0.34, respectively). For diffusing capacity (DLco), the volumes of NL and HC were independent contributors in opposite directions (r = 0.65, beta values, 0.64, -0.21, respectively). The automated system can help discriminate between UIP and NSIP with an accuracy of 82%. CONCLUSION: The automated quantification system of regional HRCT patterns can be useful in the assessment of disease severity and may provide reliable agreement with the radiologists' results. In addition, this system may be useful in differentiating between UIP and NSIP.
Female ; Humans ; Idiopathic Pulmonary Fibrosis/pathology/radiography ; Logistic Models ; Lung Diseases, Interstitial/pathology/*radiography ; Male ; Middle Aged ; Pattern Recognition, Automated/*methods ; Respiratory Function Tests ; Severity of Illness Index ; *Tomography, X-Ray Computed

OBJECTIVETo investigate the association of ATP-binding cassette transporter A3 (ABCA3) gene mutations with severe neonatal respiratory distress syndrome (NRDS) and lung disease in children.

METHODThirty-eight children hospitalized with respiratory disorders in Children's Hospital of Chongqing Medical University from January 2010 to December 2011 were screened. Two mutations (E292V, G1221S) in the ABCA3 gene were identified. Interstitial lung disease (ILD) was present in 10 cases, NRDS was found in 23 and congenital pulmonary dysplasia in 5 cases. There were 24 males and 14 females, with an age range of 1 hour to 15 years. Genomic DNA was prepared from blood samples and sequences were analyzed by polymerase chain reaction (PCR). Clinical feature, imaging characteristics and the results of gene detection were retrospectively analyzed.

RESULTFour cases with ABCA3 gene mutations were found; 2 patients (case 2 and case 4) had the heterozygous mutation of ABCA3 E292V. One was a 3-hour-old girl and another was a 52-day-old boy, 2 patients (case 1 and case 4) had the heterozygous mutation of ABCA3 G1221S. One was a 78-day-old boy and another was a girl, 15 years and one month old. The family history was negative for respiratory disease. Three patients (case 1, 2, 4 ) had NRDS and 2 (case 1, 2) of them were premature. One patient (case 3) had normal growth and development. She was diagnosed clinically as interstitial lung disease (ILD) after admission. The clinical outcomes of 4 cases were various. Case 1 had recurrent wheezing and inhaled corticosteroid was needed. Case 2 died because she failed to wean from mechanical ventilator. Case 3 was discharged with improvement but lost to follow-up. Case 4 grows normally.

CONCLUSIONGenetic variants within ABCA3 may be the genetic causes or background of a contributor to some unexplained refractory NRDS, and chronic lung disease developed in latter childhood. Identification of ABCA3 genetic variants in NRDS infants is important to offer genetic counseling, as well as early prognosis estimation and intervention in pediatric chronic lung disease.

ATP-Binding Cassette Transporters ; genetics ; Adolescent ; Child ; Child, Preschool ; DNA Mutational Analysis ; Exons ; Female ; Heterozygote ; Humans ; Infant ; Infant, Newborn ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnosis ; genetics ; Male ; Mutation ; genetics ; Radiography ; Respiratory Distress Syndrome, Newborn ; diagnosis ; genetics

Acute interstitial pneumonia is a fulminant disease of unknown etiology that usually occurs in a previously healthy person and produces the histologic findings of the organizing phase of diffuse alveolar damage. We experienced an autopsy case of acute interstitial pneumonia of unknown etiology. The patient was a 48 year old man who had been healthy and had not been exposed to organic dusts or other toxic materials. The chief complaints represented were dyspnea and a dry cough for several weeks before hospitalization, and the chest radiographs showed bilateral interstitial infiltrates. Patchy consolidation of air space was also identified and ground-glass attenuation similar to those described in ARDS was detected on high-resolution computed tomography. Steroid pulse therapy, mechanical ventilation, and antibiotics for superimposed bacterial infection were performed, but the symptoms did not improve and the patient died of generalized respiratory insufficiency and severe hypoxemia 2 1/2 months after hospitalization. At autopsy the macroscopic and microscopic findings were confined mainly to the lungs. On the whole, both lungs were firm in consistency and the external surface showed a cobblestone appearance. The cut surface showed almost complete replacement of the normal lung parenchyma with gray to yellow fibrous tissue with a little residual functional area remaining. The pathology of both open lung biopsy and autopsy tissue showed marked hyperplasia of type II pneumocytes, hyaline membrane formation, thickening of the alveolar wall due to extensive fibroblast proliferation, and relatively abundant young collagen deposition in the interstitium. An immunohistochemical stain for cytokeratin revealed epithelial hyperplasia and showed that the alveolar spaces were markedly shrunken by fibrous tissue.
Anoxia ; Anti-Bacterial Agents ; Autopsy* ; Bacterial Infections ; Biopsy ; Collagen ; Cough ; Dust ; Dyspnea ; Fibroblasts ; Hospitalization ; Humans ; Hyalin ; Hyperplasia ; Keratins ; Lung ; Lung Diseases, Interstitial* ; Membranes ; Middle Aged ; Pathology ; Pneumocytes ; Pulmonary Fibrosis ; Radiography, Thoracic ; Respiration, Artificial ; Respiratory Insufficiency

This report presents the case of a patient demonstrating multicentric Castleman's disease (MCD) with a lung lesion that was successfully treated with an anti-interleukin-6 receptor antibody, tocilizumab in combination with corticosteroid and tacrolimus. A 43-yr-old female with abnormal shadows on a chest X-ray was referred to the hospital for further examination. She was diagnosed as having MCD based on the characteristic pathology of inguinal lymph node, lung lesions, laboratory data, and undifferentiated arthritis. Corticosteroid and rituximab therapy did not fully ameliorate the symptoms; thus, the therapeutic regimen was changed to include tocilizumab, oral corticosteroid and tacrolimus. This regimen resulted in clinical remission and the dose of tocilizumab and corticosteroid could be tapered. Tocilizumab in combination with corticosteroid and tacrolimus may therefore be a beneficial treatment regimen for lung lesions associated with MCD.
Adrenal Cortex Hormones/therapeutic use ; Adult ; Antibodies, Monoclonal/*therapeutic use ; Drug Therapy, Combination ; Female ; Giant Lymph Node Hyperplasia/*diagnosis/drug therapy/radiography ; Humans ; Immunosuppressive Agents/therapeutic use ; Lung Diseases, Interstitial/*drug therapy/pathology ; Lymph Nodes/pathology ; Receptors, Interleukin-6/antagonists & inhibitors ; Tacrolimus/therapeutic use ; Tomography, X-Ray Computed

A 69-year-old male was diagnosed in February 2004 with stage IV extranodal marginal zone B cell lymphoma involving the mediastinal nodes, lung parenchyma and bone marrow with high LDH. Shortness of breath developed following the 5th course of Rituximab-CHOP chemotherapy (cyclophosphamide, Vincristine, Doxorubicin, Prednisolone). Bronchoscopy guided transbronchial lung biopsy revealed interstitial thickening and type II pneumocyte activation, compatible with interstitial pneumonitis. After treatment with prednisolone a complete resolution of the dyspnea was observed. The patient was well on routine follow-up at the outpatient clinic, with no progression of lymphoma or interstitial pneumonitis.
Aged ; Antibodies, Monoclonal/*adverse effects/*therapeutic use ; Antineoplastic Combined Chemotherapy Protocols/*adverse effects/*therapeutic use ; Biopsy ; Cyclophosphamide/adverse effects/therapeutic use ; Doxorubicin/adverse effects/therapeutic use ; Humans ; Lung Diseases, Interstitial/*chemically induced/*pathology/radiography/surgery ; Lymphoma, B-Cell, Marginal Zone/*drug therapy ; Male ; Prednisone/adverse effects/therapeutic use ; Tomography, X-Ray Computed ; Vincristine/adverse effects/therapeutic use