No abstract available.
Classification* ; Idiopathic Interstitial Pneumonias* ; Pathology*

OBJECTIVEIdiopathic interstitial pneumonias (IIPs) have been increasing in children in recent years. The type and prognosis of IIPs in children in China has not been clear. The purpose of this study was to investigate the type and prognosis of IIPs in children.

METHODSeven children diagnosed with IIPs from December 2003 to March 2007 were retrospectively studied. The clinical, radiologic, and pathologic features, type and prognosis of IIPs, were reviewed.

RESULTOf the seven patients with IIPs, three were diagnosed with cryptogenic organizing pneumonia (COP)/bronchiolitis obliterans organizing pneumonia (BOOP), one was diagnosed with usual interstitial pneumonia (UIP) and the remaining three were diagnosed with nonspecific interstitial pneumonia (NSIP). The chief complaints of these patients were chronic cough. Six patients had shortness of breath. Clubbed fingers were found in one patient with UIP and two patients with NSIP. The characteristic computed tomographic findings of COP/BOOP were multiple patchy peripheral consolidation, associated with centrilobular nodules and bronchiole wall thickening in 2 cases and bronchiolectasis in 1 case. In NSIP, diffuse or basal ground-glass opacities predominated over reticular opacities with traction bronchiectasis only in one case. UIP was manifested by patchy infiltrate with predominant honeycombing, traction bronchiectasis and interlobular septal thickening. Two patients with COP/BOOP had plugs of connective tissue within a bronchiole and the adjacent alveolar ducts and alveolar spaces. Lung biopsies showed predominantly interstitial chronic inflammation with infiltrate of lymphocytes and some plasma cells and alveolar septum thickening in three patients with NSIP. One case with NSIP showed a mixed fibrosing and prominent interstitial chronic inflammation. The key histological findings of the UIP was a heterogeneous appearance, with alternating areas of relatively normal lung, interstitial inflammation and fibrosis. All patients were treated with prednisone. The abnormal findings of chest radiography resolved completely after four to twelve months' therapy in three patients with COP/BOOP. The combination of prednisone and MTX or 6-MP was given to the two patients with NSIP. The symptoms and chest radiographic abnormality disappeared except the presence of a few cyst after treatment. One case with NSIP and the case with UIP had not been improved remarkably.

CONCLUSIONCOP/BOOP, NSIP and UIP might occur in children in China. The typing of IIPs was based on clinical-radiologic-pathologic features. Steroid was the first choice for IIPs. The prognosis of IIPs varied among different types.

Child ; Child, Preschool ; Female ; Humans ; Idiopathic Interstitial Pneumonias ; classification ; diagnosis ; pathology ; Infant ; Male ; Prognosis ; Retrospective Studies

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.
Azathioprine ; Bronchiectasis ; Cough ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Dyspnea ; Female ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Immunosuppressive Agents ; Inflammation ; Lung ; Lung Diseases, Interstitial ; Pathology ; Prognosis ; Survival Rate ; Traction

Animals ; Diagnosis, Differential ; Humans ; Idiopathic Interstitial Pneumonias ; pathology ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; classification ; pathology ; Pulmonary Fibrosis ; pathology ; Tomography, X-Ray Computed

Alveolitis, Extrinsic Allergic ; diagnosis ; pathology ; Biopsy ; Chronic Disease ; Cryptogenic Organizing Pneumonia ; diagnosis ; Diagnosis, Differential ; Granuloma, Respiratory Tract ; diagnosis ; Humans ; Idiopathic Interstitial Pneumonias ; diagnosis ; Idiopathic Pulmonary Fibrosis ; diagnosis

Objective: To analyze the clinical characteristics and prognosis of 6 children with idiopathic interstitial pneumonia (IIP). Methods: This retrospective study analyzed the clinical manifestations, examinations, treatment and prognosis of 6 children with IIP who were hospitalized in Children's Hospital of Nanjing Medical University from January 2015 to March 2020. Results: Of the 6 children, 2 were males and 4 were females, aged 4.8 to10.6 years. All children had a subacute onset, and presented with cough, shortness of breath and cyanosis. The lung high-resolution CT (HRCT) showed diffuse patchiness in bilateral lung fields in all the children and reticular pattern in 2 cases. Pulmonary function test found moderate to severe mixed defect in 5 children. Lung biopsy was performed in 4 children. All of the 6 children were treated with systemic glucocorticoids, of whom 2 cases had additional inhaled glucocorticoids. Four children were finally diagnosed as cryptogenic organizing pneumonia (COP), whose lung HRCT return to normal in 1-11 months. Two children were finally diagnosed as nonspecific interstitial pneumonia (NSIP), and had long-term residual fibrosis on lung HRCT. The 6 children were followed up for 1 year to 6 years and 5 months after discontinuation of systemic glucocorticoids, and all had no recurrence. Conclusions: The clinical characteristics of IIP in children are subacute onset presented with cough, shortness of breath, cyanosis and diffuse patchiness in bilateral lungs on HRCT. The common subtypes of IIP in children are COP and NSIP. Systemic glucocorticoid is effective for IIP in children and there is a good prognosis overall.
Child ; Cough/etiology* ; Cryptogenic Organizing Pneumonia ; Cyanosis/pathology* ; Dyspnea/pathology* ; Female ; Glucocorticoids/therapeutic use* ; Humans ; Idiopathic Interstitial Pneumonias/pathology* ; Lung/pathology* ; Male ; Retrospective Studies

The purpose of this study was to investigate the long-term clinical course of non-specific interstitial pneumonia (NSIP) and to determine which factors are associated with a response to steroid therapy and relapse. Thirty-five patients with pathologically proven NSIP were included. Clinical, radiological, and laboratory data were reviewed retrospectively. The male-to-female ratio was 7:28 (median age, 52 yr). Thirty (86%) patients responded to steroid therapy, and the median follow-up was 55.2 months (range, 15.9-102.0 months). Five patients (14%) showed sustained disease progression and three died despite treatment. In the five with sustained disease progression, NSIP was associated with various systemic conditions, and the seropositivity of fluorescent antinuclear antibody was significantly associated with a poor response to steroids (P = 0.028). The rate of relapse was 25%, but all relapsed patients improved after re-treatment. The initial dose of steroids was significantly low in the relapse group (P = 0.020). In conclusion, progression is associated with various systemic conditions in patients who show progression. A low dose of initial steroids is significantly associated with relapse.
Adult ; Aged ; Antibodies, Antinuclear/blood ; Female ; Follow-Up Studies ; Humans ; Idiopathic Interstitial Pneumonias/drug therapy/pathology ; Lung Diseases, Interstitial/*drug therapy/mortality/pathology ; Male ; Middle Aged ; Prognosis ; Recurrence ; Retrospective Studies ; Steroids/*therapeutic use

Acquired Immunodeficiency Syndrome ; diagnosis ; pathology ; Alzheimer Disease ; diagnosis ; pathology ; Autopsy ; Diagnosis, Differential ; Hepatitis, Viral, Human ; diagnosis ; pathology ; Humans ; Idiopathic Interstitial Pneumonias ; diagnosis ; pathology ; Neoplasms ; diagnosis ; pathology ; Pathology, Molecular ; methods ; Staining and Labeling

Interstitial lung disease is a generic term for a heterogeneous group of lung disease that primarily affect the interstitium although the disease is not clearly restricted to the interstitium. The majority of interstitial lung diseases represent inflammatory insults to the microscopic anatomic space bounded by the basement membrane of epithelial and endothelial cells, which may occur as slowly developing process and ultimately end up as end-stage honeycomb fibrosis. The currently prevalent classification of interstitial pneumonia with practical utility and easy reproducibility pertaining only to idopathic interstitial pneumonia encompasses several different entities some of which may represent different aspects of the same condition. Honeycomb fibrosis is usually caused by a variety of pulmonary disease including chronic interstitial lung disease. It is important to recognize that usual inter-stitial pneumonia and honeycomb fibrosis are not synonymous. In the era of chemotherapy for malignant tumor, aggressive immunosuppression for autoimmune diseases and transplant recipients and acquired immunodeficiency syndrome, lung disease in the immunocompromised host has been common. Diagnostic lung biopsy becomes increasingly needed because proper treatment of interstitial lung disease relies on correct morphologic diagnosis. This review summarizes the pathologic spectrum of idiopathic interstitial pneumonias together with other inflammatory process with known or suggestive etiologies simulating interstitial pneumonias.
Acquired Immunodeficiency Syndrome ; Autoimmune Diseases ; Basement Membrane ; Biopsy ; Classification ; Diagnosis ; Drug Therapy ; Endothelial Cells ; Fibrosis ; Idiopathic Interstitial Pneumonias ; Immunocompromised Host ; Immunosuppression ; Lung ; Lung Diseases ; Lung Diseases, Interstitial* ; Pathology* ; Pneumonia ; Transplantation

Although autoantibodies are routinely screened in patients with idiopathic interstitial pneumonia, there are no reliable data on their clinical usefulness. The aim of this study was to investigate the prognostic value of autoantibodies for predicting the development of new connective tissue disease in these patients and also mortality. We conducted retrospective analysis of the baseline, and follow-up data for 688 patients with idiopathic interstitial pneumonia (526 with idiopathic pulmonary fibrosis, 85 with nonspecific interstitial pneumonia, and 77 with cryptogenic organizing pneumonia) at one single tertiary referral center. The median follow-up period was 33.6 months. Antinuclear antibody was positive in 34.5% of all subjects, rheumatoid factor in 13.2%, and other specific autoantibodies were positive between 0.7%-6.8% of the cases. No significant difference in patient survival was found between the autoantibody-positive and -negative groups. However, the presence of autoantibodies, especially antinuclear antibody with a titer higher than 1:320, was a significant predictor for the future development of new connective tissue diseases (relative risk, 6.4), although the incidence was low (3.8% of all subjects during follow-up). In conclusion, autoantibodies are significant predictors for new connective tissue disease development, although they have no prognostic value.
Aged ; Antibodies, Antinuclear/blood ; Autoantibodies/*blood ; Cohort Studies ; Connective Tissue Diseases/pathology ; Female ; Follow-Up Studies ; Humans ; Idiopathic Interstitial Pneumonias/*blood/diagnosis ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Rheumatoid Factor/blood ; Risk Factors ; Tertiary Care Centers ; Tomography, X-Ray Computed