Adult ; Diagnosis, Differential ; Humans ; Lung ; pathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; pathology ; Male ; Middle Aged

Child ; Diagnosis, Differential ; Humans ; Lung ; pathology ; Lung Diseases, Interstitial ; diagnosis ; therapy ; Male ; Treatment Outcome

Diagnosis, Differential ; Humans ; Incidence ; Lung ; pathology ; Lung Diseases, Interstitial ; epidemiology ; pathology ; therapy ; Prognosis ; Pulmonary Fibrosis ; epidemiology ; pathology ; therapy

Chronic pneumonitis of infancy (CPI) is a very rare interstitial lung disease. Its pathological features differ from other types of interstitial pneumonia that occur in adults and children. The mortality rate of CPI is high, even with treatment. We report a case of a 3 month old girl diagnosed with CPI after an open lung biopsy who improved after proper treatment.
Biopsy ; Female ; Humans ; Infant ; Lung Diseases, Interstitial/*diagnosis/drug therapy/pathology ; Pneumonia/*diagnosis/drug therapy/pathology ; Tomography, X-Ray Computed

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

Bronchopulmonary Dysplasia ; diagnostic imaging ; pathology ; therapy ; Female ; Glycogen Storage Disease ; diagnosis ; pathology ; therapy ; Humans ; Infant ; Infant, Newborn ; Lung ; diagnostic imaging ; growth & development ; pathology ; Lung Diseases, Interstitial ; classification ; diagnosis ; pathology ; therapy ; Male ; Neurosecretory Systems ; pathology ; Pediatrics ; Tomography, X-Ray Computed

Diagnostic Errors ; Humans ; Lung Diseases, Fungal ; diagnosis ; drug therapy ; pathology ; Male ; Middle Aged ; Mucormycosis ; diagnosis ; drug therapy ; pathology ; Radiography, Thoracic ; Tomography, X-Ray Computed

BACKGROUNDPulmonary alveolar proteinosis (PAP) is a rare lung disease, the most common type of which is autoimmune PAP. The gold standard therapy for PAP is whole lung lavage (WLL). Few studies have reported the optimal technique with which to evaluate the response to WLL. In this study, we aimed to identify parameters with which to assess the need for repeat WLL during a long-term 8-year follow-up.

METHODSWe conducted a retrospective analysis of 120 patients with autoimmune PAP with 80 of whom underwent WLL. Physiologic, serologic, and radiologic features of the patients were analyzed during an 8-year follow-up after the first WLL treatment.

RESULTSOf the 40 patients without any intervention, 39 patients either achieved remission or remained stable and only one died of pulmonary infection. Of the 56 patients who underwent WLL for 1 time, 55 remained free from a second WLL and 1 patient died of cancer. Twenty-four required additional treatments after their first WLL. The baseline PaO 2 (P = 0.000), PA-aO 2 (P = 0.000), shunt fraction rate (P = 0.001), percent of predicted normal diffusing capacity of the lung for carbon monoxide (DLCO%Pred) (P = 0.016), 6-min walk test (P = 0.013), carcinoembryonic antigen (CEA) (P = 0.007), and neuron-specific enolase (NSE) (P = 0.003) showed significant differences among the three groups. The need for a second WLL was significantly associated with PaO 2 (P = 0.000), CEA (P = 0.050) , the 6-minute walk test (P = 0.026), and DLCO%Pred (P = 0.041). The DLCO%Pred on admission with a cut-off value of 42.1% (P = 0.001) may help to distinguish whether patients with PAP require a second WLL.

CONCLUSIONSWLL is the optimal treatment method for PAP and provides remarkable improvements for affected patients. The DLCO%Pred on admission with a cut-off value of 42.1% may distinguish whether patients with PAP require a second WLL.

Adult ; Autoimmune Diseases ; diagnosis ; therapy ; Bronchoalveolar Lavage ; methods ; Female ; Follow-Up Studies ; Humans ; Lung ; pathology ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis ; diagnosis ; therapy ; Retrospective Studies ; Treatment Outcome

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

Here we report a case of 41-year-old man with a soft tissue density mass at right upper lung and palpable abscesses at right upper backside and right wrist. ¹⁸F-fluorodeoxyglucose positron emission tomography/computed tomography demonstrated a 7.8 × 5.0 cm mass with soft-tissue density in the upper lobe of the right lung with high metabolic activity. The infiltrative mass extended to adjacent chest wall soft tissue. Final diagnosis of pulmonary actinomycosis with multiple abscesses was made. The patient responded well to antibiotics treatment.
Abscess ; Actinomycosis/*diagnosis/drug therapy/microbiology ; Adult ; Anti-Bacterial Agents/therapeutic use ; Diagnosis, Differential ; Fluorodeoxyglucose F18/chemistry ; Humans ; Lung Diseases/*diagnosis/drug therapy/microbiology ; Lung Neoplasms/pathology ; Male ; *Positron-Emission Tomography ; Tomography, X-Ray Computed