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

Sjogren's syndrome (SS) is an autoimmune disorder in which lymphocytes infiltrate the exocrine glands, resulting in the development of sicca symptoms. Lymphocytes may also invade various other organs and cause diverse symptoms. Interstitial pneumonia has been observed frequently in SS patients. Typically, the pneumonia responds well to systemic steroids, and fatal cases are rare. We experienced a case of lymphocytic pneumonia accompanied by SS and treated with cyclophosphamide pulse therapy, and we present details of the case herein.
Adult ; Humans ; Lung/*pathology ; Lung Diseases, Interstitial/drug therapy/*pathology ; Lymphocytes/*pathology ; Male ; Plasma Cells/pathology ; Sjogren's Syndrome/*pathology

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

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

Gefitinib is regarded as a relatively safe agent for the treatment of an advanced non-small cell lung cancer (NSCLC). Pulmonary toxicity such as interstitial lung disease associated with gefitinib is uncommon with an estimated all time incidence around 1% worldwide. Moreover, a case of gefitinib associated with pulmonary cystic changes has not been reported yet. In this report we present a case of progressive multiple air cystic changes in both lungs in a patient with NSCLC and intrapulmonary metastases who underwent a gefitinib therapy.
Antineoplastic Agents/*adverse effects ; Brain Neoplasms/secondary ; Carcinoma, Non-Small-Cell Lung/*drug therapy/secondary ; Cysts/*chemically induced ; Female ; Humans ; Lung/pathology ; Lung Diseases/*chemically induced ; Lung Diseases, Interstitial ; Lung Neoplasms/*drug therapy ; Middle Aged ; Quinazolines/*adverse effects

The purpose of this study was to investigate the long-term clinical course of non-specific interstitial pneumonia (NSIP) and to determine which factors are associated with a response to steroid therapy and relapse. Thirty-five patients with pathologically proven NSIP were included. Clinical, radiological, and laboratory data were reviewed retrospectively. The male-to-female ratio was 7:28 (median age, 52 yr). Thirty (86%) patients responded to steroid therapy, and the median follow-up was 55.2 months (range, 15.9-102.0 months). Five patients (14%) showed sustained disease progression and three died despite treatment. In the five with sustained disease progression, NSIP was associated with various systemic conditions, and the seropositivity of fluorescent antinuclear antibody was significantly associated with a poor response to steroids (P = 0.028). The rate of relapse was 25%, but all relapsed patients improved after re-treatment. The initial dose of steroids was significantly low in the relapse group (P = 0.020). In conclusion, progression is associated with various systemic conditions in patients who show progression. A low dose of initial steroids is significantly associated with relapse.
Adult ; Aged ; Antibodies, Antinuclear/blood ; Female ; Follow-Up Studies ; Humans ; Idiopathic Interstitial Pneumonias/drug therapy/pathology ; Lung Diseases, Interstitial/*drug therapy/mortality/pathology ; Male ; Middle Aged ; Prognosis ; Recurrence ; Retrospective Studies ; Steroids/*therapeutic use

Most of the interstitial lung diseases are rare, chronic, progressive and fatal disorders, especially in familial form. The etiology of the majority of interstitial lung disease is still unknown. Host susceptibility, genetic and environmental factors may influence clinical expression of each disease. With familial interstitial lung diseases, mutations of surfactant protein B and surfactant protein C or other additional genetic mechanisms (e.g. mutation of the gene for ATP-binding cassette transporter A3) could be associated. We found a 21 month-old girl with respiratory symptoms, abnormal radiographic findings and abnormal open lung biopsy findings compatible with nonspecific interstitial pneumonitis that is similar to those of her older sister died from this disease. We performed genetic studies of the patient and her parents, but we could not find any mutation in our case. High-dose intravenous methylprednisolone and oral hydroxychloroquine were administered and she is still alive without progression during 21 months of follow-up.
Child, Preschool ; Female ; Humans ; Hydroxychloroquine/administration and dosage ; Infant ; Korea ; Lung Diseases, Interstitial/drug therapy/*genetics/pathology/physiopathology ; Methylprednisolone/administration and dosage ; Siblings ; Tomography, X-Ray Computed

From 2006 to 2011, an outbreak of a particular type of childhood interstitial lung disease occurred in Korea. The condition was intractable and progressed to severe respiratory failure, with a high mortality rate. Moreover, in several familial cases, the disease affected young women and children simultaneously. Epidemiologic, animal, and post-interventional studies identified the cause as inhalation of humidifier disinfectants. Here, we report a 4-year-old girl who suffered from severe progressive respiratory failure. She could survive by 100 days of extracorporeal membrane oxygenation support and finally, underwent heart-lung transplantation. This is the first successful pediatric heart-lung transplantation carried out in Korea.
Child, Preschool ; Disinfectants/toxicity ; Extracorporeal Membrane Oxygenation ; Female ; Humans ; *Humidifiers ; Lung/drug effects/pathology ; Lung Diseases, Interstitial/*chemically induced/pathology/*therapy ; *Lung Transplantation ; Republic of Korea ; Respiratory Rate ; Retrospective Studies ; Thorax/diagnostic imaging ; Tomography, X-Ray Computed

OBJECTIVETo analyze the clinical features of interstitial pneumonitis (IP) associated with interferon therapy for chronic hepatitis C.

METHODSWe report the first case of IP in China resulting from pegylated interferon alpha-2a in combination with ribavirin for treatment of hepatitis C viral infection. A statistical analysis of the related literatures documenting such IP cases was performed using SPSS 11.5 software.

RESULTSOf the 22 patients reported to develop IP after interferon therapy alone or in combination with ribavirin, 83%, 72% and 56% of the patients had the symptoms of dyspnoea, dry cough and fever, respectively. Twenty of these cases presented with restrictive pulmonary functional impairment and/or hypoxemia, and diffuse infiltration on chest radiography and/or CT. Complications were documented in 71% of the cases within 12 weeks of the treatment. The majority (85%) of the patients had favorable prognoses with an average recovery time of 7.5 weeks. Compared with the patients with mild and moderate pulmonary function impairment, 8 patients with severe pulmonary functional impairment had early onset of IP during the interferon therapy (6.6 vs 14.1 weeks, P<0.05), and a higher rate of corticosteroid treatment (75% vs 54%, P>0.05).

CONCLUSIONIP is a rare pulmonary complication associated with IFN therapy, and patients with chronic hepatitis C should be followed up closely in the first 12 weeks of interferon therapy. Prompt discontinuation of medication is mandatory in the presence of IP, and corticosteroid therapy may not be essential for patients with mild or moderate pulmonary functional impairment under close monitoring. The severity of pulmonary damage is associated with the time of complication occurrence, and corticosteroids are required when obvious pulmonary toxicity occurs in early stage of the treatment (within 6 weeks) to reduce the pulmonary damage.

Adult ; Aged ; Female ; Hepatitis C, Chronic ; drug therapy ; Humans ; Interferon-alpha ; adverse effects ; therapeutic use ; Lung ; diagnostic imaging ; drug effects ; pathology ; physiopathology ; Lung Diseases, Interstitial ; chemically induced ; diagnostic imaging ; pathology ; physiopathology ; Male ; Middle Aged ; Time Factors ; Tomography, X-Ray Computed

Acetates ; therapeutic use ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hyperplasia ; Infant ; Lung ; diagnostic imaging ; pathology ; physiopathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; physiopathology ; Methylprednisolone ; therapeutic use ; Neuroendocrine Cells ; pathology ; Quinolines ; therapeutic use ; Tomography, X-Ray Computed