1/4. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.- - - - - - - - - - ranking = 1keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
2/4. Rapidly progressive bronchiolitis obliterans associated with stevens-johnson syndrome.A middle-aged woman developed fulminant respiratory failure during recovery from stevens-johnson syndrome. Mechanical ventilation was difficult because of unusually high airway pressures, and she died one month later. autopsy revealed not only the usual features of bronchiolitis obliterans, but also obstruction of cartilaginous bronchi. The histopathologic features suggested a relationship between bronchial obstruction and stevens-johnson syndrome.- - - - - - - - - - ranking = 0.55555555555556keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
3/4. Chronic bronchobronchiolitis obliterans associated with stevens-johnson syndrome.We report a case of bronchobronchiolitis obliterans associated with stevens-johnson syndrome diagnosed using bronchography and bronchoscopy in a 25-year-old man with dyspnea. Eighteen months prior to admission, the diagnosis of stevens-johnson syndrome due to anticonvulsant therapy was made at another hospital. On admission, the patient was wheezing and had overinflation of the lungs, with marked obstructive pulmonary dysfunction. ventilation and perfusion scintigrams revealed multiple ventilation and perfusion defects but not mismatch. bronchography revealed central bronchiectasis and obstruction of the peripheral bronchi. Occlusion of the fifth right basal bronchus was observed with fiberoptic bronchoscopy. bronchography and bronchoscopy provide useful information for the diagnosis of this disease.- - - - - - - - - - ranking = 0.55555555555556keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
4/4. bronchiolitis obliterans in children with stevens-johnson syndrome: follow-up with high resolution CT.About one third of children with stevens-johnson syndrome have pulmonary involvement. As a consequence of airway epithelial injury, bronchiolitis obliterans can occur in these patients. Two cases of stevens-johnson syndrome-associated bronchiolitis obliterans in children were diagnosed and followed by high resolution CT without open lung biopsy. Serial changes of high resolution CT features of bronchiolitis obliterans are discussed and the literature is reviewed.- - - - - - - - - - ranking = 0.35831648564571keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |