A 60-year-old Filipino woman diagnosed with dermatomyositis was initially on prednisone and methotrexate. She eventually developed interstitial lung disease (ILD) and so methotrexate was shifted to azathioprine; however, azathioprine was discontinued due to cutaneous tuberculosis. Over eight years, the dermatomyositis was controlled by prednisone alone but the ILD worsened. This case demonstrated that the course of ILD may be independent of dermatomyositis.
dermatomyositis ; interstitial lung disease ; immunomodulator

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease characterized by progressive lung fibrogenesis and histological features of usual interstitial pneumonia. IPF has a poor prognosis and presents a spectrum of disease courses ranging from slow evolving disease to rapid deterioration; thus, a differential diagnosis remains challenging. Several biomarkers have been identified to achieve a differential diagnosis; however, comprehensive reviews are lacking. This review summarizes over 100 biomarkers which can be divided into six categories according to their functions: differentially expressed biomarkers in the IPF compared to healthy controls; biomarkers distinguishing IPF from other types of interstitial lung disease; biomarkers differentiating acute exacerbation of IPF from stable disease; biomarkers predicting disease progression; biomarkers related to disease severity; and biomarkers related to treatment. Specimen used for the diagnosis of IPF included serum, bronchoalveolar lavage fluid, lung tissue, and sputum. IPF-specific biomarkers are of great clinical value for the differential diagnosis of IPF. Currently, the physiological measurements used to evaluate the occurrence of acute exacerbation, disease progression, and disease severity have limitations. Combining physiological measurements with biomarkers may increase the accuracy and sensitivity of diagnosis and disease evaluation of IPF. Most biomarkers described in this review are not routinely used in clinical practice. Future large-scale multicenter studies are required to design and validate suitable biomarker panels that have diagnostic utility for IPF.
Humans ; Idiopathic Pulmonary Fibrosis/diagnosis* ; Biomarkers ; Lung Diseases, Interstitial ; Lung ; Bronchoalveolar Lavage Fluid ; Disease Progression ; Prognosis

OBJECTIVEAcute interstitial pneumonia (AIP) is a rare lung interstitial disease in children. This study was conducted to understand the clinical features of the AIP in children.

METHODThe data of the three cases with AIP admitted to our hospital from March 2008 to November 2009 were reviewed. Of the 3 cases, 2 were male, one was female. Their age ranged from 1 year and 4 months to 10 years. The clinical manifestation, pulmonary function test and the high resolution computed tomography (HRCT) and pathology of the lung were studied retrospectively. Tissue specimens of the lung were obtained by video-assisted thoracoscopic biopsy. Viral etiologic examinations for the respiratory syncytial virus, adenovirus, influenza virus, parainfluenza viruses, EB virus, cytomegalovirus, enterovirus and herpes simplex virus were performed. The IgM antibody to Mycoplasma pneumoniae in the serum was also detected.

RESULTAll the 3 cases rapidly developed respiratory failure of unknown origin, none of these cases had failure of any other organs. All three cases had cough and dyspnea. No case had the rales and digital clubbing. The examinations for viruses, bacteria and Mycoplasma pneumoniae infection were all negative. No evidence for the diagnosis of connective tissue disease was obtained. The HRCT of the chest showed diffuse alveolar consolidation, air bronchogram and ground glass appearance in the bilateral lungs, and the traction-associated bronchiectasis in areas. All the three cases had the histological proof of diffuse alveolar damage by the biopsy. All the three cases were treated with CPAP and corticosteroid. Two cases were treated with corticosteroid in early stage of the disease, the condition of these cases were improved obviously. The third case was treated with high-dose steroid pulse therapy days, the condition of this case was improved slightly in a month. One year follow-up showed that case 1 and case 2 had no hypoxemia and the HRCT of the chest showed obvious improvement. The pulmonary function of case 2 had restrictive deficiency.

CONCLUSIONAIP has a rapidly progressive clinical course leading to respiratory failure. The HRCT of the chest showed alveolar consolidation and ground glass-like change. The pathology of the lung includes diffuse alveolar damage. The prognosis of the AIP in children may be improved by the treatment with respiratory assistance and corticosteroids.

Acute Disease ; Child ; Child, Preschool ; Female ; Humans ; Lung Diseases, Interstitial ; diagnosis ; pathology ; therapy ; Male ; Respiratory Insufficiency

Pulmonary interstitial glycogenosis (PIG) is a very rare interstitial lung disease in infants. It is poorly understood, but its pathological features are distinct; they include uniform alveolar septal thickening, caused by a group of oval to spindle-shaped cells containing abundant glycogen, without apparent inflammation or fibrosis. PIG is usually associated with a good prognosis. However, in the present report, we describe the case of a 5-month-old male infant who died due to PIG; he was severely underweight and not administered proper treatment or care. The pathology of PIG was determined following a medico-legal autopsy.
Autopsy ; Fibrosis ; Glycogen ; Glycogen Storage Disease ; Humans ; Infant ; Inflammation ; Lung Diseases, Interstitial ; Male ; Prognosis ; Thinness

Antibodies, Monoclonal, Humanized/therapeutic use* ; Autoantibodies ; Dermatomyositis ; Disease Progression ; Humans ; Lung Diseases, Interstitial/drug therapy*

Pemetrexed is approved as a first-line treatment for advanced non-squamous non-small cell lung cancer (NSCLC) with cisplatin and as a single agent for second-line treatment or for patients who show no disease progression after four cycles of platinum-based doublet induction chemotherapy as maintenance therapy. Pemetrexed has a modest toxicity profile and has not traditionally been regarded as a cause of interstitial pneumonitis. Here, we report on a rare case of pemetrexed-induced pneumonitis in a patient with NSCLC.
Adenocarcinoma ; Carcinoma, Non-Small-Cell Lung ; Cisplatin ; Disease Progression ; Glutamates ; Guanine ; Humans ; Induction Chemotherapy ; Lung Diseases, Interstitial ; Pneumonia ; Pemetrexed

PURPOSE: In this nationwide 5-year longitudinal population-based study, we aimed at investigating the incidence of lung cancer among patients with interstitial lung disease. MATERIALS AND METHODS: Data was collected from the Korean National Health Insurance Research Database from 49,773,195 Korean residents in 2009. Thirteen thousand six hundred and sixty-six patients with interstitial lung disease diagnosed January-December 2009. The end of follow-up was June 30, 2014. Up to four matching chronic obstructive pulmonary disease controls were selected to compare the lung cancer high-risk group based on age, sex, diagnosis date (within 30 days), and hospital size. The number of patients with newly developed lung cancer was determined. RESULTS: The incidences of lung cancer were 126.98, 156.62, and 370.38 cases per 10,000 person-years (2,732, 809, and 967 cases of cancer, respectively) in the chronic obstructive pulmonary disease, interstitial lung disease, and chronic obstructive pulmonary disease with interstitial lung disease groups, respectively. Of the 879 patients with idiopathic pulmonary fibrosis, 112 developed lung cancer (incidence, 381.00 cases per 10,000 person-years). CONCLUSION: Incidence of lung cancer among patients with interstitial lung disease was high. Interstitial lung diseases have a high potential for developing into lung cancer, even when concurrent with chronic obstructive pulmonary disease.
Diagnosis ; Follow-Up Studies ; Health Facility Size ; Humans ; Idiopathic Pulmonary Fibrosis ; Incidence ; Longitudinal Studies ; Lung Diseases, Interstitial* ; Lung Neoplasms* ; Lung* ; National Health Programs ; Pulmonary Disease, Chronic Obstructive

We report two cases of rituximab (RTX)-induced interstitial pneumonia in two lymphoma patients receiving RTX treatment. Interstitial pneumonia was successfully managed in these two cases after a one-week-long intervention with corresponding treatments without affecting further treatment of the primary disease. RTX-induced interstitial pneumonia is characterized by a latent onset with an unclear pathological mechanism and absence of typical symptoms. High-resolution CT scan can provide valuable evidence for early diagnosis of RTX-induced interstitial pneumonia, which might be attributed partially to an increased susceptibility to P. jirovecii and fungal infection due to prolonged RTX treatment.
Antibodies, Monoclonal, Murine-Derived ; adverse effects ; Disease Susceptibility ; Humans ; Lung Diseases, Interstitial ; chemically induced ; Rituximab ; Tomography, X-Ray Computed

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

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