1/18. Chronic obstructive pneumonia caused by a vertebral body osteophyte.Osteophytes associated with spondylosis have been implicated as a cause of multiple extraspinal manifestations. Symptoms are more likely to occur with the large osteophytes associated with diffuse idiopathic skeletal hyperostosis. In the thoracic region, osteophytes have been reported infrequently as a cause of extraspinal complications. We report a case in which an anterior thoracic vertebral osteophyte was responsible for chronic obstructive pneumonia due to obstruction of the right main stem bronchus. The patient's condition improved considerably after surgical resection of the compressing thoracic osteophyte.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
2/18. Pneumomediastinum, subcutaneous emphysema, and pulmonary fibrosis in a patient with idiopathic pneumonia syndrome after bone marrow transplantation.An adolescent female underwent bone marrow transplantation for relapsed leukemia and developed acute and chronic graft-versus-host disease and idiopathic pneumonia syndrome. Her lung disease responded to large doses of methylprednisolone but evolved to pulmonary fibrosis and pneumomediastinum and subcutaneous emphysema in the convalescent period. Pulmonary function tests revealed a restrictive pattern. Pneumomediastinum and subcutaneous emphysema are complications not only of obstructive but also of restrictive lung disease and vary with respect to time of onset.- - - - - - - - - - ranking = 5keywords = idiopathic (Clic here for more details about this article) |
3/18. Etanercept (Enbrel) administration for idiopathic pneumonia syndrome after allogeneic hematopoietic stem cell transplantation.Acute pulmonary dysfunction remains a frequent and severe complication of hematopoietic stem cell transplantation (SCT). Almost half of the pulmonary insults that occur in this seating are noninfectious in origin and are referred to as idiopathic pneumonia syndrome (IPS). In this series of 3 patients, etanercept (Enbrel; Immunex, Seattle, WA), a soluble, dimeric tumor necrosis factor alpha-binding protein, was administered to 3 consecutive pediatric allogeneic BMT recipients with IPS. The administration of etanercept, in combination with standard immunosuppressive therapy, was well tolerated and associated with significant improvements in pulmonary dysfunction within the first week of therapy. These data suggest that etanercept may represent a safe, non-cross-reactive, therapeutic option for patients with IPS and that clinical trials studying etanercept for this indication are warranted.- - - - - - - - - - ranking = 5keywords = idiopathic (Clic here for more details about this article) |
4/18. hydroxyurea-induced pneumonitis in a patient with chronic idiopathic myelofibrosis after prolonged drug exposure.We describe a 63-yr-old patient diagnosed with chronic idiopathic myelofibrosis whose disease was treated with hydroxyurea for 2 yr. He developed respiratory symptoms that were extensively investigated including lung biopsy. Clinical, radiological and histological features were compatible with a diagnosis of hydroxyurea-induced pneumonitis. Following drug withdrawal there was remarkable improvement in clinical and radiological findings.- - - - - - - - - - ranking = 5keywords = idiopathic (Clic here for more details about this article) |
5/18. legionella micdadei pneumonia diagnosed by culture isolation and dna-dna hybridization from bronchial lavage fluid.An 80-year-old man was admitted because of dyspnea on effort. We suspected an acute exacerbation of chronic heart failure and idiopathic interstitial pneumonia caused by right-sided pneumonia. A nodular shadow in right upper lobe spread and consolidated into the airspace, and it failed to improve despite administration of meropenem trihydrate, vancomycin hydrochloride and clindamycin. A definitive diagnosis of legionella micdadei pneumonia was made on the basis of this organism being isolated in culture from bronchial lavage fluid and subsequent identification of legionella micdadei using dna-dna hybridization. The airspace consolidation gradually improved following treatment with intravenous erythromycin and minocycline hydrochloride.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
6/18. bronchiolitis obliterans organizing pneumonia: the clinical and radiological features of seven cases and a review of the literature.The clinical and radiological features of seven cases of bronchiolitis obliterans organizing pneumonia (BOOP) are presented and the literature is reviewed. Six of these cases were idiopathic, one patient had rheumatoid arthritis. Detailed respiratory function tests were available in four cases and showed a restrictive defect with impaired gas transfer. The majority of chest radiographs showed consolidation with no zonal preponderance. One case with unilobar consolidation was mistaken for malignancy. Another case with interstitial shadowing was thought to have cryptogenic fibrosing alveolitis. In those cases with consolidation, it was mainly peripheral. Six of the cases showed good response to oral steroid therapy. Open lung biopsy was the principal means by which tissue was obtained for diagnosis. BOOP should be included in the differential diagnosis of multifocal consolidation. The condition is usually responsive to steroid therapy, so it is important to confirm the diagnosis histologically.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
7/18. Disseminated nocardia farcinica infection in a uraemia patient with idiopathic thrombocytopenia purpura receiving steroid therapy.nocardia farcinica has been reported as an increasingly frequent cause of localized and disseminated infections in immunocompromised patients in recent years, but N. farcinica bacteraemia remains a rare finding. Here, the case is described of a 68-year-old man with end-stage renal disease and idiopathic thrombocytopenia purpura treated with steroid therapy who developed disseminated infection (bacteraemia, multilobar pneumonia and brain abscesses) due to N. farcinica. The isolate was confirmed by partial sequencing analysis of the 16S rRNA gene. The patient recovered after prolonged trimethoprim-sulfamethoxazole therapy with no recurrence in over 1 year.- - - - - - - - - - ranking = 5keywords = idiopathic (Clic here for more details about this article) |
8/18. Etanercept therapy for late-onset idiopathic pneumonia syndrome after nonmyeloablative allogeneic hematopoietic stem cell transplantation.Idiopathic pneumonia syndrome (IPS) is a noninfectious pulmonary complication of allogeneic hematopoietic stem cell transplantation (AHSCT), which usually develops within the first 100 days after transplantation. Donor T-cell-derived tumor necrosis factor-alpha (TNF-alpha) may play a crucial role in the pathogenesis of IPS, and inhibition of TNF-alpha has been used as a therapeutic option. We report two patients who had late-onset IPS about day 150 after nonmyeloablative AHSCT (NMA-AHSCT). They responded well to etanercept in combination with standard immunosuppressive drugs. Both patients had relapses and responded to retreatment with etanercept-based therapy. One patient was alive at 30 months after the initial diagnosis on long-term maintenance therapy with etanercept. The second patient was lost to follow-up at our institution but died 13 months after the onset of IPS. Our two cases showed that IPS could develop late after NMA-AHSCT and inhibition of TNF-alpha activity can be therapeutically effective.- - - - - - - - - - ranking = 4keywords = idiopathic (Clic here for more details about this article) |
9/18. Inflammatory and autoimmune complications of common variable immune deficiency.Common variable immune deficiency (CVID) is associated with autoimmune and inflammatory complications in addition to recurrent infections. The most common conditions are idiopathic thrombocytopenia purpura, autoimmune hemolytic anemia, sarcoid-like granulomatous disease and gastrointestinal inflammation. IVIG administration reduces the frequency of infections, but does not always prevent autoimmunity or inflammation. TNF antagonists and anti-CD20 immunomodulators have shown some efficacy in CVID in a few patients; further controlled studies are needed to determine the best management of these conditions in the setting of immunodeficiency.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
10/18. bcr-abl-positive T-cell acute lymphoblastic leukemia associated with parvovirus B19 infection.The authors report an unusual presentation of a philadelphia chromosome-positive acute lymphoblastic leukemia with two unusual features: a bcr-abl fusion mRNA coding for p210 protein and a T-cell immunophenotype. The patient was a 16-year-old boy who presented with septic shock and pancytopenia, likely precipitated by an acute parvovirus B19 infection. Management consisted of supportive therapy, followed by chemotherapy for T-cell acute lymphoblastic leukemia and stem cell transplantation. He died 8 months after transplant due to idiopathic pneumonia syndrome, but without evidence of relapsed disease.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
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