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

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

Aged ; Biopsy ; methods ; Bronchoscopy ; methods ; Cryptogenic Organizing Pneumonia ; diagnosis ; Female ; Humans ; Image-Guided Biopsy ; methods ; Lung ; pathology ; Male ; Middle Aged ; Ultrasonography ; methods

No abstract available.
Bronchiolitis Obliterans* ; Bronchiolitis* ; Cryptogenic Organizing Pneumonia* ; Hepatitis C* ; Hepatitis*

A 74-year-old man suffering from cryptogenic organizing pneumonia (OP) visited our department with arthralgia accompanied with partial swellings of proximal interphalangeal and metacarpophalangeal joints with morning stiffness. A diagnose of rheumatoid arthritis (RA) was made. It was thought that OP was associated with RA. We initiated a treatment with salazosulfapyridine and loxoprofen for RA. Although this treatment was effective, it was discontinued due to the development of drug eruption. As an alternative, the patient was treated with prednisolone (PSL) and clarithromycin (CAM). This treatment demonstrated being effective for OP and RA, to a certain extent; however, the RA activity was not completely suppressed. In order to suppress the RA activity further, tacrolimus (TAC) was successfully added with increasing the dosage of CAM that is assumed to raise blood TAC concentrations. The present case shows that treatment with PSL, CAM and TAC may be effective in some cases of RA.
Aged ; Arthralgia ; Arthritis, Rheumatoid ; Clarithromycin ; Cryptogenic Organizing Pneumonia ; Drug Eruptions ; Humans ; Metacarpophalangeal Joint ; Pneumonia ; Prednisolone ; Sulfasalazine ; Tacrolimus

Cryptogenic Organizing Pneumonia ; diagnosis ; therapy ; Hot Temperature ; Humans ; Medicine, Chinese Traditional ; Qi ; Yin-Yang

BACKGROUND: Although organizing pneumonia (OP) responds well to corticosteroid therapy, relapse is common during dose reduction or follow-up. Predictors of relapse in OP patients remain to be established. The aim of the present study was to identify factors related to relapse in OP patients. METHODS: This study was retrospectively performed in a tertiary referral center. Of 66 OP patients who were improved with or without treatment, 20 (30%) experienced relapse. The clinical and radiologic parameters in the relapse patient group (n=20) were compared to that in the non-relapse group (n=46). RESULTS: Multivariate analysis demonstrated that percent predicted forced vital capacity (FVC), PaO2/FiO2, and serum protein level were significant predictors of relapse in OP patients (odds ratio [OR], 0.82; 95% confidence interval [CI], 0.70-0.97; p=0.018; OR, 1.02; 95% CI, 1.00-1.04; p=0.042; and OR, 0.06; 95% CI, 0.01-0.87; p=0.039, respectively). CONCLUSION: This study shows that FVC, PaO2/FiO2 and serum protein level at presentation can significantly predict relapse in OP patients.
Cryptogenic Organizing Pneumonia ; Follow-Up Studies ; Humans ; Methods ; Multivariate Analysis ; Pneumonia* ; Recurrence* ; Retrospective Studies ; Tertiary Care Centers ; Vital Capacity

Cryptogenic organizing pneumonia (COP) is an inflammatory lung disease involving the distal bronchioles, respiratory bronchioles, bronchiolar ducts, and alveolae. The etiology is usually unknown; however, there are several known causes and associated systemic diseases. Corticosteroid therapy is the best treatment option and the prognosis of COP is good, with recovery in up to 80% of patients. We described a patient with in-operable hepatocellular carcinoma (HCC) undergoing chemoembolization with doxorubicin in a drug-eluting bead (DEB). COP developed in the patient after chemoembolization but resolved spontaneously in several months.
Bronchioles ; Carcinoma, Hepatocellular* ; Chemoembolization, Therapeutic ; Cryptogenic Organizing Pneumonia* ; Doxorubicin ; Humans ; Lung Diseases ; Prognosis

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

In connective tissue diseases, autoantibodies cause pulmonary interstitial inflammation and fibrosis, and patients require treatment with an immunosuppressive agent such as a steroid. Dermatomyositis is an incurable, uncommon form of connective tissue disease that occasionally causes diffuse pulmonary inflammation leading to acute severe respiratory failure. In such cases, the prognosis is very poor despite treatment with high-dose steroid. In the present case, a 46-year-old man was admitted to our hospital with dyspnea. He was diagnosed with dermatomyositis combined with cryptogenic organizing pneumonia (COP) with respiratory failure and underwent treatment with steroid and an immunosuppressive agent, but the COP was not improved. However, the respiratory failure did improve after treatment with intravenous immunoglobulin, which therefore can be considered a treatment option in cases where steroids and immunosuppressive agents are ineffective.
Autoantibodies ; Connective Tissue Diseases ; Cryptogenic Organizing Pneumonia* ; Dermatomyositis* ; Dyspnea ; Fibrosis ; Humans ; Immunoglobulins* ; Immunosuppressive Agents ; Inflammation ; Lung Diseases, Interstitial ; Middle Aged ; Pneumonia ; Prognosis ; Respiratory Insufficiency ; Steroids