Lymphocytic interstitial pneumonitis (LIP) is characterized by diffuse bilateral pulmonary infiltrations in both lower lobes. Pleomorphic lymphohistiocytes including mature lymphocytes, variable admixture of plasma cells and other mononuclear cells infiltrate within the pulmonary interstitium, ranging from widened septa to confluent masses. We report a case of LIP associated with Epstein-Barr virus in a patient with SLE and Sj gren's syndrome. A 50-year-old woman was admitted with insidious onset of progressive dyspnea for 20 days. She suffered from arthritis 10 years earlier without specific diagnosis. A radiography of chest has showed bilateral consolidative infiltrations with pleural effusion in both lower lung fields. Open lung biopsy documented lymphocytic interstitial pneumonitis which expressed Epstein-Barr virus genome in situ hybridization study. Following corticosteroid and cyclophosphamide therapy, clinical symptoms and radiologic infiltrations gradually remitted.
Case Report ; Cyclophosphamide/therapeutic use ; Female ; Glucocorticoids, Synthetic/therapeutic use ; Herpesviridae Infections/*complications ; *Herpesvirus 4, Human ; Human ; Immunosuppressive Agents/therapeutic use ; Lung Diseases, Interstitial/*complications/drug therapy ; Lupus Erythematosus, Systemic/*complications ; Middle Age ; Prednisone/therapeutic use ; Sjogren's Syndrome/*complications

OBJECTIVETo explore the clinical and laboratory features and the prognosis of juvenile dermatomyositis (JDM) complicated with interstitial lung disease (ILD).

METHODData of 39 cases of JDM complicated with ILD hospitalized in Beijing Children's Hospital from January 2005 to December 2011 were collected. The clinical features, laboratory data and prognosis of these children were analyzed.

RESULTOf the 39 cases studied, 16 were boys, and 23 girls. The average age of onset was 5.6 years, and 61.5% of the patients' age of onset (24 cases) was under 6 years. Rashes (17 cases, 43.6%), simultaneous eruption of rashes and muscle weakness (14 cases, 35.9%), fever (4 cases, 10.1%), or muscle weakness (3 cases, 7.7%) were common initial symptoms of the disease. Only 51.3% of the patients (20 cases) had the symptoms of respiratory system, but (24 cases) 61.5% were complicated with that of the gastrointestinal system; (27 cases) 69.2% had at the same time electrocardiographic and echocardiographic abnormalities. The chest high resolution computed tomography (HRCT) showed cord or band-like shadows in their lungs of more than half of the cases (25 cases, 64.1%), and other changes included ground glass-like shadow (10 cases, 25.6%), net and lineation-like shadow (9 cases, 23.1%), nodular change (5 cases, 12.8%). The patients complicated with lung essential infiltration accounted for as high as 71.8% (28 cases). These imaging changes were largely seen on both dorsal sides of their lungs. Severe patients also had mediastinal emphysema, pneumothorax, pneumorrhagia or aerodermectasia. Twenty-four patients underwent pulmonary function examination, and 62.5% of the patients' pulmonary function (15 cases) was abnormal. The fatality rate of the cases studied was 10.1%.

CONCLUSIONThe imaging changes of patients suffering from JDM with ILD were often more severe as compared to the clinical symptoms, and were often complicated with damages to other systems and organs. The prognosis of those patients was poorer than others. Patients with JDM especially at a younger age of onset and with various organ damages should be examined with chest HRCT examinations as early as possible.

Child ; Child, Preschool ; Dermatomyositis ; complications ; diagnosis ; drug therapy ; Female ; Glucocorticoids ; administration & dosage ; therapeutic use ; Humans ; Immunosuppressive Agents ; administration & dosage ; therapeutic use ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; etiology ; Male ; Methotrexate ; administration & dosage ; therapeutic use ; Muscle Weakness ; diagnosis ; epidemiology ; etiology ; Prognosis ; Respiratory Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed

Treatment of interstitial lung disease (ILD) in rheumatoid arthritis (RA) has been controversial. Although there have been several anecdotal reports on the efficacies of corticosteroids or cytotoxic agents such as methotrexate, cyclophosphamide, azathioprine, and D-penicillamine for the treatment of ILD associated with RA, no controlled studies have been performed. To date, corticosteroids have been a central agent for the treatment of this disease, but their effects are partial and temporary in most cases. In addition, the adverse effects of these agents are considerable. On the other hand, limited information is available on the cyclosporine use in ILD associated with RA. We describe a 49-yr old female patient with RA and ILD that had initially responded to high dose prednisolone and cyclophosphamide intravenous pulse therapy, and the lung disease was aggravated with the tapering of prednisolone. After 10 months of follow-up loss, the patient was successfully treated with low dose cyclosporine without high dose corticosteroids.
Anti-Inflammatory Agents/therapeutic use ; Antirheumatic Agents/*therapeutic use ; Arthritis, Rheumatoid/complications/*drug therapy/radiography ; Cyclophosphamide/therapeutic use ; Cyclosporine/*therapeutic use ; Disease Progression ; Female ; Glucocorticoids/therapeutic use ; Humans ; Lung Diseases, Interstitial/complications/*drug therapy/radiography ; Middle Aged ; Prednisolone/therapeutic use ; Treatment Outcome

After 4-months of alpha interferon (IFN-alpha), a 64-year old woman with chronic hepatitis C developed a cough and dyspnea and showed diffuse infiltrative opacities on her chest X-ray. Her symptoms persisted after stopping the IFN-alpha therapy. Pulmonary function testing revealed a reduced forced vital capacity. High-resolution computed tomography of the lung showed peripheral and peribronchovascular ground glass attenuation and consolidation associated with reticulation. Bronchoalveolar lavage was performed for further evaluation and showed a lymphocyte level of 8.2%, an uncommon finding in IFN-alpha-induced interstitial pneumonitis. We performed a lung biopsy to diagnose her disease and it suggested interstitial pneumonitis. This was considered to be due to the immunomodulatory effects of INF-alpha. Although rare, any sign of significant pulmonary involvement should be evaluated.
Antiviral Agents/*adverse effects/therapeutic use ; Bronchoalveolar Lavage ; Female ; Hepatitis C, Chronic/complications/*drug therapy ; Humans ; Interferon-alpha/*adverse effects/therapeutic use ; Kidney Failure, Chronic/complications ; Lung Diseases, Interstitial/*chemically induced/pathology/radiography ; Middle Aged ; Respiratory Function Tests ; Tomography, X-Ray Computed

BACKGROUND/AIMS: To evaluate the impact on mortality of anti-tumor necrosis factor (anti-TNF) treatment of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). METHODS: We retrospectively reviewed the medical records of 100 RA-ILD patients who visited our tertiary care medical center between 2004 and 2011, identified those treated with an anti-TNF agent, divided patients into non-survivor and survivor groups and evaluated their clinical characteristics and causes of death. RESULTS: A total of 24 RA-ILD patients received anti-TNF therapy, of whom six died (25%). Mean age at initiation of anti-TNF therapy was significantly higher in the nonsurvivor versus survivor group (76 years [range, 66 to 85] vs. 64 years [range, 50 to 81], respectively; p = 0.043). The mean duration of anti-TNF treatment in the non-survivor group was shorter (7 months [range, 2 to 14] vs. 23 months [range, 2 to 58], respectively; p = 0.030). The duration of anti-TNF therapy in all nonsurviving patients was < 12 months. Pulmonary function test results at ILD diagnosis, and cumulative doses of disease-modifying drugs and steroids, did not differ between groups. Five of the six deaths (83%) were related to lung disease, including two diffuse alveolar hemorrhages, two cases of acute exacerbation of ILD, and one of pneumonia. The sixth patient died of septic shock following septic arthritis of the knee. CONCLUSIONS: Lung complications can occur within months of initial anti-TNF treatment in older RA-ILD patients; therefore, anti-TNF therapy should be used with caution in these patients.
Adult ; Aged ; Aged, 80 and over ; Antirheumatic Agents/adverse effects/*therapeutic use ; Arthritis, Rheumatoid/complications/diagnosis/*drug therapy/immunology/mortality ; Female ; Humans ; Lung Diseases, Interstitial/diagnosis/etiology/*mortality ; Male ; Middle Aged ; Republic of Korea ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Tertiary Care Centers ; Time Factors ; Treatment Outcome ; Tumor Necrosis Factor-alpha/*antagonists & inhibitors