Dermatomyositis ; complications ; Humans ; Lung Diseases, Interstitial ; diagnosis ; etiology ; therapy

Arthritis, Rheumatoid/complications* ; Biomarkers ; Humans ; Lung Diseases, Interstitial/etiology* ; Prognosis

Juvenile dermatomyositis (JDM) is an autoimmune disease manifesting as proximal muscle weakness and skin rash and can involve multiple systems and visceral organs. Myositis-specific autoantibodies (MSAs) are highly associated with various complications and prognosis in JDM. Patients with anti-Mi-2 antibodies tend to have good prognosis and typical clinical symptoms. Patients with anti-MDA5 antibodies often have diffuse interstitial lung disease and skin ulcer, with mild symptoms of myositis. Patients with anti-NXP2 antibodies often have calcinosis, and such antibodies are associated with gastrointestinal bleeding and perforation. Patients with anti-TIF1-γ antibodies have diffuse and refractory skin lesions. Anti-SAE antibodies are rarely detected in children, with few reports of such cases. This article reviews the features of clinical phenotypes in JDM children with these five types of MSAs, so as to provide a basis for the clinical treatment and follow-up management of children with JDM.
Autoantibodies ; Dermatomyositis ; Humans ; Lung Diseases, Interstitial/etiology* ; Myositis ; Prognosis

OBJECTIVEInterstitial lung disease in children represents a heterogeneous group of disorders of both known and unknown causes. This study aimed to understand better the causes of the disease in children and to provide information on the current approach to diagnosis and management of the disease.

METHODThrough the Pediatric Diffuse Parenchymal Lung Disease/Pediatric Interstitial Lung Disease Cooperative Group of China, data of 93 cases of interstitial lung disease of children from 11 hospitals were collected with the same questionnaire in 2009. Respiratory tract secretions were obtained for bacterial culture. Respiratory virus antigen examination, mycoplasma antibody, EB virus, cytomegalovirus, and herpes simplex viruses antibody detection were performed. Cells in the sputum, gastric juice and bronchoalveolar lavage fluid (BALF) were tested for hemosiderin. The CT or high resolution CT (HRCT) of the lung and blood-gas analysis were also performed. Fourteen cases underwent lung biopsy and 25 cases underwent bronchomicrocopy. Data were then pooled and discussed through a series of meetings.

RESULTFifty-three cases were male, 40 were female and their age ranged from 8 months to 14 years. Thirty-nine cases were diagnosed as bronchiolitis obliterans (BO); 39 as idiopathic pulmonary hemosiderosis (IPH); 7 as idiopathic interstitial pneumonia (IIP) of unknown causes, of whom 4 cases had non specific interstitial pneumonia, 1 case as acute interstitial pneumonia and 1 case as lymphocytic interstitial pneumonia, 1 case as idiopathic pulmonary fibrosis; 2 cases as secondary interstitial lung disease, one was secondary to SLE, one to human immunodeficiency virus (HIV) infection; 2 cases had hypersensitive pneumonitis; 2 cases had pulmonary alveolar proteinosis; 1 case had bronchiolitis obliterans organizing pneumonia; 1 case had lipoid pneumonia;1 case of diffuse panbronchiolitis; 1 case of microlithiasis alveolaris pulmonum. Forty two cases had cough, 24 of them also had tachypnea, 8 cases had clubbing. HRCT showed that 56 cases had groundglass-like opacification; 30 cases had mosaic appearance; 1 case had diffuse micronodular opacities, 1 case had diffuse reticulonodular opacities and cysts.

CONCLUSIONInterstitial lung disease in children is a heterogeneous group of disorders. In this study diagnoses of 11 diseases were made, the top third diagnoses were BO, IPH and IIP, respectively.

Adolescent ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Humans ; Infant ; Lung Diseases, Interstitial ; diagnosis ; epidemiology ; etiology ; Male

Adolescent ; Common Variable Immunodeficiency ; complications ; Female ; Humans ; Lung Diseases, Interstitial ; etiology ; pathology ; physiopathology ; Pneumonia ; etiology ; pathology ; physiopathology

OBJECTIVE: To investigate the clinical features, radiologic scores and clinically relevant risk factors prognosis of secondary interstitial lung disease (ILD) in patients with systemic lupus erythematosus (SLE). METHODS: In this study, 60 SLE patients in Department of Rheumatology of the First Affiliated Hospital of Baotou Medical College and Taizhou First People's Hospital from January 2015 to March 2019 were retrospectively analyzed. All of those 60 patients with SLE underwent lung high resolution computed tomography (HRCT) examination. We used a 1 ∶1 case-control study. There was a matching of age and gender between the two groups. Thirty patients with SLE related ILD (SLE-ILD) were in the case group, and 30 patients with SLE without ILE (SLE non-ILD) were in the control group. The clinical features, pulmonary function test, radiologic characteristic of SLE patients were collected and were used to analyze SLE-ILD. RESULTS: In this study, we reached the following conclusions: First, there were statistically significant differences in chest tightness/shortness of breath, Raynaud's phenomenon, and Velcro rale between SLE-ILD and SLE non-ILD patients (both P < 0.05); Second, hemoglobin (Hb) and albumin (ALB) in the patients of SLE-ILD had a significant decrease compared with the patients of SLE non-ILD. Blood urea nitrogen (BUN), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) increased in SLE-ILD patients compared with SLE non-ILD patients, the difference had statistical significance (P < 0.05); Third, for SLE-ILD patients, the most common type was non-specific interstitial pneumonia (NSIP), followed by usual interstitial pneumonia and lymphocytic interstitial pneumonia; Fourth, there was no significant difference in clinical-radiology-physiology scores between the different ILD types (P>0.05), similarly, the lung HRCT score and lung function between different ILD types had no significant difference (P>0.05); Fifth, multivariate Logistic regression analysis showed that decreased albumin and chest tightness/shortness of breath might be the risk factor for SLE-ILD. CONCLUSION There are statistically significant differences between the SLE-ILD group and SLE non-ILD group in terms of chest tightness/shortness of breath, Velcro rale and Raynaud's phenomenon. Decreased albumin and chest tightness/shortness of breath in SLE patients should be alerted to the occurrence of ILD. NSIP is the most common manifestation of SLE-ILD.
Case-Control Studies ; Humans ; Lung/diagnostic imaging* ; Lung Diseases, Interstitial/etiology* ; Lupus Erythematosus, Systemic/complications* ; Retrospective Studies

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

Adult ; Biopsy ; Electric Power Supplies ; Humans ; Lung ; pathology ; Lung Diseases, Interstitial ; etiology ; Male ; Metallurgy ; Nickel ; adverse effects ; Occupational Diseases ; etiology ; Tomography, X-Ray Computed

Occupational and environmental exposure can directly cause specific lung diseases, and can also induce autoimmune diseases that can lead to various types of interstitial lung diseases. In recent years, it was discovered that certain occupational and environmental exposure was related to the increased risk of Idiopathic pulmonary fibrosis (IPF) disease and progression, including metal and mineral dust, wood dust, organic dust, asbestos dust, silica dust, cigarette smoke and air pollution. IPF is a chronic progressive fibrotic lung disease of unknown etiology, with a characteristic imaging and histologic pattern called usual interstitial pneumonia. This article is a review based on the correlation and mechanism of occupational and environmental exposure in the pathogenesis and disease progression of IPF to improve the understanding of the disease and promote the formulation of treatment plans.
Humans ; Idiopathic Pulmonary Fibrosis/etiology* ; Dust ; Environmental Exposure/adverse effects* ; Occupational Exposure/adverse effects* ; Lung Diseases, Interstitial

BACKGROUNDGiant cell interstitial pneumonia (GIP) was a rare form of pneumoconiosis, associated with exposure to hard metals, which had been reported mostly as isolated case reports. We described eight cases of GIP diagnosed in our hospital during the past seven years, with particular reference to new findings.

METHODSEight patients with GIP confirmed by biopsy in the Nanjing Drum Tower Hospital affiliated to Medical School of Nanjing University from 2005 to 2011 were retrospectively analyzed. For each patient, the occupy histories and medical records were thoroughly reviewed and clinic data were extracted. Two radiologists, without knowledge of any of the clinical and functional findings, independently reviewed the HRCT scans of all patients. Follow-up data were collected.

RESULTSAmong the eight patients, seven had a history of exposure to hard metal dusts, one denied an exposure history. The most common manifestations were cough and dyspnea. One patient initiated with pneumothorax and another pleural effusion, both of which were uncommon to GIP. The main pathologic appearances were the presence of macrophages and multinucleated giant cells in the alveolar space. The clinical symptoms and radiographic abnormalities were obviously improved after cessation of exposure and receiving corticosteroid treatments, recurrences were observed in two patients when they resumed work. In spite of exposure cessation and corticosteroid treatment, one patient developed pulmonary fibrosis at seven years follow-up.

CONCLUSIONSAwareness of the patients' occupational history often provided clues to the diagnosis of GIP. Histopathologic examinations were necessary to establish the right diagnosis. Exposure cessation was of benefit to most patients; however, pulmonary fibrosis was possible in spite of exposure cessation and corticosteroid treatment. Better ways should be found out to improve the outcome and quality of life.

Adult ; Alloys ; toxicity ; Cobalt ; toxicity ; Female ; Humans ; Lung ; pathology ; Lung Diseases, Interstitial ; diagnosis ; etiology ; Male ; Middle Aged ; Pulmonary Fibrosis ; diagnosis ; etiology ; Retrospective Studies ; Tungsten ; toxicity ; Young Adult