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

Interstitial lung disease (ILD) is a rare condition characterized by extensive inflammation and fibrosis mainly involving the pulmonary interstitium or alveoli. Usually, patients with ILD clinically present with chronic cough and exertional dyspnea. ILD is classified into subtypes based on clinical characteristics, detailed history obtained from patients, and radiological, and/or histopathological features. The most common type of idiopathic interstitial pneumonia is idiopathic pulmonary fibrosis (IPF). IPF is a chronic progressive fibrosing ILD and is associated with poor prognosis. An exclusive diagnosis of IPF requires no known condition causing ILD and typical radiological and/or histopathological features of lung fibrosis. Fibrosis observed in this condition is attributable to repetitive epithelial injury with consequent abnormal wound healing in genetically susceptible and elderly individuals. Currently, pirfenidone and nintedanib are useful disease-modifying agents available to treat IPF. In this article, we review the concept, diagnosis, clinical course, and treatment of ILD.
Aged ; Cough ; Diagnosis ; Dyspnea ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Inflammation ; Lung ; Lung Diseases, Interstitial ; Prognosis ; Wound Healing

Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the co-occurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjögren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.
Arthritis, Rheumatoid ; Asian Continental Ancestry Group ; Autoantibodies ; Connective Tissue Diseases ; Connective Tissue ; Diagnosis ; Disease Management ; Guidelines as Topic ; Humans ; Idiopathic Interstitial Pneumonias ; Incidence ; Lung ; Lung Diseases, Interstitial ; Myositis ; Prognosis ; Scleroderma, Systemic ; Thorax

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

Idiopathic interstitial pneumonia (IIP) is a histologically identifiable pulmonary disease without a known cause that usually infiltrates the lung interstitium. IIP is largely classified into idiopathic pulmonary fibrosis, idiopathic non-specific interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease (ILD), cryptogenic organizing pneumonia, desquamative interstitial pneumonia, and acute interstitial pneumonia. Each of these diseases has a different prognosis and requires specific treatment, and a multidisciplinary approach that combines chest high-resolution computed tomography (HRCT), histological findings, and clinical findings is necessary for their diagnosis. Diagnosis of IIP is made based on clinical presentation, chest HRCT findings, results of pulmonary function tests, and histological findings. For histological diagnosis, video-assisted thoracoscopic biopsy and transbronchial lung biopsy are used. In order to identify ILD associated with connective tissue disease, autoimmune antibody tests may also be necessary. Many biomarkers associated with disease prognosis have been recently discovered, and future research on their clinical significance is necessary. The diagnosis of ILD is difficult because patterns of ILD are both complicated and variable. Therefore, as with other diseases, accurate history taking and meticulous physical examination are crucial.
Biomarkers ; Biopsy ; Classification ; Connective Tissue Diseases ; Cryptogenic Organizing Pneumonia ; Diagnosis ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Lung ; Lung Diseases ; Lung Diseases, Interstitial ; Physical Examination ; Prognosis ; Respiratory Function Tests ; Thorax

BACKGROUND: Lung transplantation is a life-saving procedure in patients with end-stage lung disease, and is increasingly performed in Korea. METHODS: We retrospectively evaluated the outcomes of patients who received a lung transplant at Asan Medical Center between January 2008 and December 2016. Thirteen of 54 patients experienced multiorgan transplantation; the remaining 41 who received only lung grafts were included. RESULTS: The mean age of the lung transplant recipients was 44.6 years; 27 were men and 14 were women. The most frequent reasons were idiopathic interstitial pneumonia (21 of 41 patients, 51.2%), interstitial lung disease (9 of 41, 22.0%), and bronchiolitis obliterans after bone marrow transplantation (7 of 41, 17.1%). The median waiting time was 47 days, and many patients received preoperative intensive care (27 of 41, 65.9%), ventilator support (26 of 41, 63.4%), or extracorporeal life support (19 of 41, 46.3%). All 41 patients received bilateral lung grafts. Ten deaths occurred (24.3%), including 5 cases of early mortality (12.2%) and 5 cases of late mortality (12.2%). The 1-, 3-, and 5-year survival rates were 78.9%, 74.2%, and 69.3%, respectively. CONCLUSION: Despite a high percentage of patients who required preoperative intensive care, the transplantation outcomes were acceptable.
Bone Marrow Transplantation ; Bronchiolitis Obliterans ; Chungcheongnam-do ; Critical Care ; Female ; Humans ; Idiopathic Interstitial Pneumonias ; Korea ; Lung Diseases ; Lung Diseases, Interstitial ; Lung Transplantation ; Lung ; Male ; Mortality ; Retrospective Studies ; Survival Rate ; Transplant Recipients ; Transplants ; Ventilators, Mechanical

Humans ; Idiopathic Interstitial Pneumonias ; classification ; diagnosis

Here, we present a case of desquamative interstitial pneumonia (DIP) that was initially misdiagnosed as chronic eosinophilic pneumonia due to an increased number of eosinophils in the bronchoalveolar lavage fluid (BALF). A 56-year-old male smoker presented with a productive cough that had been present for 1 month. High-resolution computed tomography (HRCT) revealed multifocal patchy ground-glass and reticular opacities in the subpleural area. BALF analysis revealed an elevated level of eosinophils (37%). Thus, the patient was initially diagnosed with chronic eosinophilic pneumonia and was administered prednisolone (0.5 mg/kg/day). However, his symptoms and the diffuse infiltrative shadows on HRCT did not improve after 2 months of treatment, and a video-assisted thoracoscopic lung biopsy led to the diagnosis of DIP. Prednisolone (1 mg/kg/day) was administered again, and the patient's symptoms improved. At 1 year after the end of treatment, the patient remained symptom-free.
Biopsy ; Bronchoalveolar Lavage Fluid* ; Bronchoalveolar Lavage* ; Cough ; Diagnosis ; Eosinophils* ; Humans ; Idiopathic Interstitial Pneumonias ; Lung ; Lung Diseases, Interstitial* ; Male ; Middle Aged ; Prednisolone ; Pulmonary Eosinophilia

Cryptogenic organizing pneumonia (COP) is an idiopathic interstitial pneumonia characterized by a subacute course and favorable prognosis with corticosteroids. However, some patients show resistance to steroids. Macrolides have been used with success in those patients showing resistance to steroids. A few reports showed treatment failure with macrolides in patients with COP who were resistant to steroids. In this report, we described two cases of COP who showed different responses to clarithromycin. One recovered completely, but the other gradually showed lung fibrosis with clarithromycin.
Adrenal Cortex Hormones ; Clarithromycin* ; Cryptogenic Organizing Pneumonia* ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Lung ; Macrolides ; Prognosis ; Steroids ; Treatment Failure

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare, recently classified entity that consists of pleural and subjacent parenchymal fibrosis predominantly in the upper lungs. In an official American Thoracic Society/European Respiratory Society statement in 2013, this disease is introduced as a group of rare idiopathic interstitial pneumonias. We describe a case of a 76-year-old woman with cough and recurrent pneumothorax. She was admitted to our hospital with severe cough at first. High resolution computed tomography (HRCT) disclosed multifocal subpleural consolidations with reticular opacities in both lungs, primarily in the upper lobes, suggesting interstitial pneumonia. Rheumatoid lung was diagnosed initially through an elevated rheumatoid factor, HRCT and surgical biopsy at the right lower lobe. However, one month later, pneumothorax recurred. Surgical biopsy was performed at the right upper lobe at this time. The specimens revealed typical subpleural fibroelastosis. We report this as a first case of idiopathic PPFE in Korea after reviewing the symptoms, imaging and pathologic findings.
Aged ; Biopsy ; Cough ; Female ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Korea ; Lung ; Lung Diseases, Interstitial ; Pneumothorax* ; Rheumatoid Factor