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1/13. Acute interstitial pneumonitis. Case series and review of the literature.

    Acute interstitial pneumonitis (AIP) is an acute, idiopathic interstitial lung disease characterized by rapidly progressive diffuse pulmonary infiltrates and hypoxemia requiring hospitalization. The case-fatality ratio is high. Previous reports suggested that survivors of the acute event have a favorable outcome. We undertook this study to examine the natural history of survivors. We had observed several patients who experienced recurrent episodes of AIP and chronic progressive interstitial lung disease. We sought to determine longitudinal survival in these patients and to compare our experience with that in the medical literature. overall, we identified 13 biopsy-proven cases of AIP. The mean patient age was 54 years in our review, which is identical to previous reports. Twelve patients were hospitalized and all 12 required mechanical ventilation. overall hospital survival was 67%. All patients demonstrated abnormalities in gas exchange at presentation. Radiographs typically demonstrated bilateral patchy densities that progressed to a diffuse alveolar filling pattern in nearly all cases. All biopsy specimens showed organizing diffuse alveolar damage. Longitudinal data were available for 7 patients. Two died of AIP recurrences. A third died of complications of heart failure shortly after hospital discharge. One patient progressed to end-stage lung disease and required lung transplantation. Two patients experienced persistent pulmonary symptoms, accompanied in 1 by progressive lung fibrosis. One patient had nearly complete recovery of lung function 2 years following AIP. (Follow-up information was unavailable for 2 survivors.) In our literature review, 5 of 7 patients reported experienced some recovery of lung function. One case of progressive interstitial lung disease requiring lung transplantation was reported. The reported mortality was much higher than in our experience (74% versus 33%). The mean time from symptom onset to death was 26 days, compared with 34 days in our experience. The use of corticosteroids did not appear to influence survival, although this has not been tested in a rigorous manner. The better survival in our series may be related in part to a survivor selection bias. In contrast to previous reports, we found that survivors of AIP may experience recurrences and chronic, progressive interstitial lung disease. We did not identify any clinical or pathologic features that predict mortality in these patients. Likewise, there were no features that predicted the longitudinal course in survivors. Further study to identify causal factors is required in the hope of preventing morbidity and mortality related to this disease.
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2/13. Idiopathic progressive pulmonary fibrosis.

    Five patients with progressive fibrotic lung disease are described. The dominant symptom was slowly increasing dyspnoea, and cough and sputum were not prominent. Marked weight loss was also a feature. There was severe restrictive impairment of ventilation with normal arterial gas tensions. The changes were confined to the upper parts of the lung in some but others had more generalized disease. The duration has varied so far from two to 17 years. The lung changes are considered to be due to dense progressive fibrosis. Necropsy in two confirmed this. Histologically there was monotonous fibrosis with lymphoid collections and secondary bronchiectasis, a picture similar to that found in association with ankylosing spondylitis. None of these patients had joint disease. Tuberculosis was excluded as a cause by exhaustive bacteriological tests and the failure of chemotherapy to stop deterioration. All other recognized types of infective and non-infective progressive lung fibrosis were also excluded, and this is not considered to be a variant of cryptogenic fibrosing alveolitis. Though these patients have many features in common they do not necessarily have the same pathogenesis. They are presented as an encouragement to further study.
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ranking = 0.78548305021414
keywords = alveolitis
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3/13. A case of cough, lymphocytic bronchoalveolitis and coeliac disease with improvement following a gluten free diet.

    Chronic cough is a common reason for presentation to a respiratory clinic. In up to 20% of cases the cause remains unclear after investigations. We report one such case where there was bronchoscopic evidence of lymphocytic airway inflammation in association with newly diagnosed coeliac disease. All features improved markedly on a gluten free diet, suggesting a causal relationship between coeliac disease, cough, and lymphocytic bronchoalveolitis.
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ranking = 3.9274152510707
keywords = alveolitis
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4/13. Pneumonitis induced by rifampicin.

    An 81-year-old man was admitted to hospital with pulmonary mycobacterium tuberculosis infection and was treated with rifampicin (RFP), isoniazid (INH), and ethambutol (EB). On day 9 he developed fever and dyspnoea. Chest radiographs showed new infiltration shadows in the right lung. Bronchoalveolar lavage (BAL) was performed and increased numbers of lymphocytes were recovered. Drug induced pneumonitis was suspected so the antituberculous regimen was discontinued and methylprednisolone was administered. The symptoms and infiltration shadows improved. INH and EB were reintroduced without any recurrence of the abnormal shadows. T cell subsets in the BAL fluid and a positive lymphocyte stimulation test for RFP suggest that RFP induced pneumonitis may be related to a complex immunological response.
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keywords = pneumonitis
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5/13. Chronic hypersensitivity pneumonitis caused by aspergillus complicated with pulmonary aspergilloma.

    A 57-year-old man consulted our hospital with a history of the gradual onset of dyspnea and a productive cough. Chest computed tomographic (CT) scans showed a nodular shadow in a cavity lesion, and reticulonodular, cystic, and ground-grass opacities in the bilateral lung fields with honeycombing. He was diagnosed as having pulmonary aspergilloma and idiopathic pulmonary fibrosis (IPF). As an outpatient, he suffered from dyspnea upon physical exertion with exacerbation of the high-resolution CT (HRCT) opacities. An inhalation provocation test for aspergillosis fumigatus was positive and chronic hypersensitivity pneumonitis (CHP) caused by aspergillus was finally diagnosed. Insidious CHP is sometimes misdiagnosed as IPF. The diagnosis of insidious CHP should be made on the basis of a detailed history, specific HRCT findings, and lymphocyte-dominant bronchoalveolar lavage fluid cell findings.
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keywords = pneumonitis
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6/13. Rheumatoid arthritis associated interstitial lung disease.

    Rheumatoid arthritis (RA) is a systemic disease manifest as a symmetric polyarthritis usually in the setting of elevated autoantibodies (rheumatoid factor). This disease affects 1-2% of the world's population, most frequently in the 25-55 year old age group and has a female predominance (2.5:1). Nearly 50% of patients with RA demonstrate some type of extra-articular manifestation of the disease such as pleuritis, pleuropericarditis, vasculitis, pneumonitis, pulmonary fibrosis, scleritis or nodulosis. Pulmonary involvement in RA is common and can be due to the disease itself as well as to the therapies used to treat it. In fact, lung disease is the second most common cause of death, following infection, for patients with RA and has been reported to effect between 1-40% of patients. RA associated interstitial lung disease (ILD) is often subtle in onset, slowly progressive and of unclear etiology and response to treatment. This article aims to clarify the current clinical, radiographic and pathologic status of RA-ILD.
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ranking = 0.2
keywords = pneumonitis
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7/13. A woman with cough, fever, and micronodular infiltrates on chest radiograph.

    Hot tub lung is a form of hypersensitivity pneumonitis (HP) reported to be associated with exposure to large aerosolized inocula of mycobacterium avium complex (MAC). Although the pathogenesis of the disease is still poorly understood, the pathology of MAC-related HP can be similar to that of sarcoidosis, with well-formed granulomas. We describe a patient with HP featuring well-formed granulomas on transbronchial biopsy specimens, a finding believed to be unique to MAC-related HP, also referred to as "hot tub lung".
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ranking = 0.2
keywords = pneumonitis
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8/13. Familial idiopathic pulmonary hemosiderosis.

    Two brothers, aged 3 and 6 years, respectively, had their pulmonary conditions diagnosed as idiopathic pulmonary hemosiderosis (IPH). Both boys had severe iron-deficiency anemia, chronic cough, hemoptysis, and exertional dyspnea, and one had recurrent epistaxis. The results of light microscopic lung histopathologic studies in both patients showed numerous hemosiderinladen macrophages and chronic interstitial pneumonitis. No specific patterns of immunofluorescence of the alveolar capillary basement membranes were found. The results of electron microscopic examinations showed intact alveolar and capillary basement membranes and no evidence of electron-dense deposits. The lack of clinical or biochemical evidence for renal disease as well as the absence of serum antinuclear and antibasement membrane antibodies excluded associated autoimmune disorders. Evaluation for milk-protein allergy was negative and neither child demonstrated a clinical response to a milk-free diet. Sequential pulmonary function studies performed over four years showed episodes of acute obstructive airway disease that correlated with pulmonary hemorrhage and mild persistent restrictive lung disease. The results of this family study suggested that some cases of IPH may have a genetic basis.
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ranking = 0.2
keywords = pneumonitis
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9/13. Congenital tracheoesophageal fistula without esophageal atresia.

    Congenital tracheoesophageal fistula (TEF) without esophageal atresia, commonly referred to as H-type fistula, comprises only 4.2% of all TEF's. Four infants with this entity were surgically treated at Santa rosa Children's Hospital during the period of 1974 to 1977, and their diagnosis, surgical management, and hospital course are described in detail. H-type TEF is characterized by a clinical triad consisting of paroxysms of coughing precipitated by feeding, gaseous distention of the gastrointestinal tract, and pneumonitis. Demonstration of H-type TEF can be difficult and may be accomplished only by repeated examinations. The esophagogram using cinefluoroscopy and image intensification is the primary diagnostic technique utilized. If this is nondiagnostic, then tracheobronchoscopy should be performed in conjunction with ancillary techniques which improve endoscopic yield. Surgical correction can be accomplished through a cervical approach in 80% of cases. Preoperatival treatment of H-type TEF is dependent upon a high index of suspicion, an aggressive diagnostic approach, and prompt surgical correction. Our series of patients demonstrates that early diagnosis is feasible.
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ranking = 0.2
keywords = pneumonitis
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10/13. sulfasalazine pneumonitis.

    A man with alleged Crohn's disease of the terminal ileum was started on sulfasalazine. Five weeks later he developed progressive cough, shortness of breath, and fever associated with peripheral eosinophilia and bilateral pulmonary infiltrates. After withdrawal of sulfasalazine all abnormalities returned to normal. This case supports the conviction that there is an entity of sulfasalazine-induced lung disease.
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ranking = 0.8
keywords = pneumonitis
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