Cases reported "Pulmonary Fibrosis"

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1/29. Cutaneous alternariosis in a patient with idiopathic pulmonary fibrosis.

    A 78-year-old farmer presented with symptomless skin lesions for evaluation. Two years prior, he had developed idiopathic pulmonary fibrosis (IPF) and had been treated thereafter with oral prednisolone 20 mg/day and occasionally with colchicine 1 mg/day. On examination, erythematoviolaceous, slightly infiltrated plaques, measuring approximately 5 x 9 cm, rubbery in consistency, intermingled with pustules, sometimes eroded, with distinctive borders, were noted on the dorsum of both hands and on the extensor surface of both forearms. The lesions had developed over a 20-day period. The skin of these areas was atrophic or eroded with multiple ecchymoses (Fig. 1). The abnormal laboratory findings included an elevated white blood cell count of 17,100/mm3, with 79% neutrophils, 16% lymphocytes, and 5% monocytes, c-reactive protein of 33.15 mg/dL (normal, <0.8 mg/dL), and immunoglobulin g of 598 mg/dL (normal, 701-1545 mg/dL). Other blood and urine tests performed were within normal limits. The diagnosis of IPF was reconfirmed through radiology, high-resolution computed tomography, and spirometry, as well as bronchoscopy and bronchoalveolar lavage fluid analysis. Coexistence of presumptive pulmonary alternariosis was excluded. hematoxylin and eosin stained sections of the excised cutaneous specimen showed focal ulceration of the epidermis adjacent to a mainly intradermal abscess cavity. Within the latter, remnants of a partly destroyed hair follicle were seen amongst degenerating polymorphonuclear leukocytes, as well as many histiocytes and a few Langhans-type multinucleated giant cells. Minute collections of polymorphonuclear leukocytes were seen in the adjacent epidermis. periodic acid-Schiff (PAS) and Gomori's silver methenamine stains showed a multitude of broad branching fungal hyphae and large spores within the aforementioned cavity, both free and within the cytoplasm of giant cells (Fig. 2). immunohistochemistry was performed by means of the alkaline phosphatase anti-alkaline phosphatase (APAAP) method. Sections showed that the infiltrate consisted of an almost equal number of B and T lymphocytes, whereas histiocytes and the few giant cells were labeled with anti-CD68 antibodies. skin smears and biopsy specimens taken twice from all lesions were used for mycologic examination. Wet mounts revealed numerous, brownish, septate hyphae and ovoid skin smears and biopsy specimens taken twice from all lesions were used for mycologic examination. Wet mounts revealed numerous, brownish, septate hyphae and ovoid structures. biopsy material was plated on Sabourand's dextrose agar with cloramphenicol (0.05 mg/mL). After 7 days at 27 degrees C, dark, gray-white colonies with a dark brown underside appeared. Microscopic examination of the colonies revealed hyphae with typical conidia having transverse and longitudinal septa. Based on macroscopic and microscopic examination, the isolates were identified as alternaria alternata (Fig. 3). Treatment with prednisolone was reduced to 10 mg/day and the patient received oral itraconazole (200 mg/day). This resulted in progressive improvement of alternariosis, and the lesions healed completely within 3 months, when treatment was interrupted. Two years later, there is no evidence of recurrence.
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2/29. Giant cell interstitial pneumonia in a 60-year-old female without hard metal exposure.

    Giant cell interstitial pneumonia is a form of pulmonary fibrosis usually caused by exposure to hard metals. We report a case of giant cell interstitial pneumonia in a 60-year-old female office worker who was a non-smoker and did not have any exposure to hard metals.
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3/29. An aspergilloma in an echinococcal cyst cavity.

    Aspergilloma is a saprophytic infection which can colonize preexisting lung cavities. The most common underlying diseases are tuberculosis, sarcoidosis, cavitary lung cancer, etc. Although aspergilloma can also occur in operated hydatid cyst cavities, only a few cases have been reported in literature. A 32-year-old female patient underwent cystectomy for the diagnosis of perforated intraparenchymal giant hydatid cyst located in the right upper lobe, reaching down to the hilum. Capitonnage was not performed and it was observed that a residual cavity remained in the cystectomy area. The patient was discharged; however, during clinical and radiological follow-ups, it was found that the residual cyst cavity had expanded. As it was thought that one of the drainage bronchi in the cyst cavity could have opened, the patient was reoperated. During the operation, it was noted that purulent fluid and necrotic tissues were present in the cystic cavity. It was seen that the posterior upper lobe segment was consolidated and not ventilated. Contents of the cavity were removed and the posterior upper lobe segment was resected. Histopathological examination revealed that the tissue in the cavity was that of an aspergilloma, and that chronic organized pneumonia and diffuse interstitial fibrosis were present in the resected segment. Refraining from surgical obliteration (capitonnage) of cyst cavities in cases of giant hydatid cysts extending to the hilum can lead to opportunistic infections such as aspergilloma.
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4/29. Giant cell interstitial pneumonia.

    Giant cell interstitial pneumonia is a distinctive and uncommon form of interstitial pneumonia. It is distinguished by the prominence of large, actively phagocytic alveolar giant cells of histiocytic origin in the presence of chronic interstitial pneumonia. Multinucleated type 2 granular pneumocytes are also identified. The multinucleated cells lack viral intranuclear inclusions of the type seen in measles pneumonia. Giant cell interstitial pneumonia may be idiopathic or it may occur with occupational exposure to hard metals or cobalt. We report this case to give recognition to an uncommon interstitial pneumonia.
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5/29. Rapidly fatal progression of cobalt lung in a diamond polisher.

    Interstitial lung disease was diagnosed in a 52-yr-old male diamond polisher, who worked with polishing disks containing cobalt. After a further 7 months of probably high occupational exposure without any specific treatment, he had to quit work because of dyspnea. Despite treatment with systemic corticosteroids and continuous oxygen administration, he died 3 months later in respiratory distress. Postmortem examination of the lung tissue showed a typical giant-cell interstitial fibrosis, with active inflammatory cell infiltration superimposed on an established centrilobular fibrosis. The lung tissue contained 2.1 micrograms cobalt/g wet weight (more than 100-fold the normal concentration); cobalt particles, mainly localized in macrophages, were identified by transmission electron microscopy and energy-dispersive X-ray analysis. We speculate that the rapid deterioration and fatal outcome resulted from the continued exposure to cobalt, leading to a high pulmonary concentration of cobalt, and from the oxygen treatment because cobalt promotes the formation of hydroxyl free radicals.
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6/29. "Blue bodies" in a case of cryptogenic fibrosing alveolitis (desquamative type) an ultra-structural study.

    A patient with cryptogenic fibrosing alveolitis, with both mural and desquamative features, had two lung biopsies at the times of coronary artery surgery. These lung specimens were studied, using light and electron microscopy, with immunofluorescence techniques and electron microanalysis. In addition to the typical changes of cryptogenic fibrosing alveolitis previously reported, we found "blue-staining bodies" within alveolar macrophages and giant cells. These bodies were 15--25 micrometer in diameter with an iron rich outer rim and core of connective tissue mucin--possibly chondroitin sulphate or dermatan sulphate. It seems unlikely that these "blue bodies" were due to fibreglass dust to which the patients had had a trivial exposure, but their exact nature and significance remains unclear.
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7/29. The respiratory effects of cobalt.

    We studied seven subjects with certain manifestations of cobalt-induced lung disease. All worked with cobalt and were involved in either the production or use of hard metal. The mode of presentation varied from an acute hypersensitivity pneumonitis that cleared completely when exposure ceased to progressive severe interstitial fibrosis of the lungs. In one subject reexposure was followed by a recurrence of the symptoms. All subjects showed restrictive ventilatory impairment and a reduction of their diffusing capacity. The radiologic appearances varied greatly. While two subjects had clear roentgenograms with small lung volumes, others had a micronodular pattern or small blotchy nodular infiltrates, and one had diffuse reticulonodulation as is seen in cryptogenic fibrosing alveolitis. The pathologic appearances varied between desquamative interstitial pneumonia and overt mural fibrosis of the alveoli. Six of the seven patients had multinucleated giant cells in their biopsy specimens or bronchoalveolar lavage fluid.
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8/29. Giant-cell interstitial pneumonia and hard-metal pneumoconiosis. A clinicopathologic study of four cases and review of the literature.

    We report four cases of giant-cell interstitial pneumonia that occurred in association with exposure to hard metals. All patients presented with chronic interstitial lung disease and had open-lung biopsies that revealed marked interstitial fibrosis, cellular interstitial infiltrates, and prominent intraalveolar macrophages as well as giant cells displaying cellular cannibalism. We also review the literature to determine the sensitivity and specificity of giant-cell interstitial pneumonia for hard-metal pneumoconiosis. Although hard-metal pneumoconiosis may take the form of usual interstitial pneumonia, desquamative interstitial pneumonia, and giant-cell interstitial pneumonia, the finding of giant-cell interstitial pneumonia is almost pathognomonic of hard-metal disease and should provoke an investigation of occupational exposure.
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9/29. A case of giant cell interstitial pneumonia.

    This report is of a case of giant cell interstitial pneumonia (GIP) diagnosed by transbronchial lung biopsy (TBLB) and treated effectively with corticosteroid. The patient was a 61-year-old man with cough, dyspnea and findings of diffuse reticular opacities on chest X-ray. In the microscopic section of a biopsied specimen, varied forms of giant cells were observed, some with a cannibalistic appearance. Numerous giant cells and some lymphocytes were also observed in the bronchoalveolar lavage fluid. The possibility of measles pneumonia was ruled out by a negative serum antibody test and the absence of inclusion bodies in the giant cells. The patient had neither a past history of exposure to heavy metals nor to organic dusts. A distinct improvement was obtained with 30 mg of prednisolone administered daily for two weeks and then tapered off gradually.
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10/29. Giant-cell pneumonia caused by measles and methotrexate in childhood leukaemia in remission.

    Four children who had acurate lymphoblastic leukaemia in remission and developed pneumonia were studied. Investigations including electron microscopy and immunofluorescence of lung biopsy material disclosed measles, although there was no clinical evidence of the disease. Despite an identical presentation, two types of illness developed: two children died of giant-cell pneumonia, while the other two developed pneumonia indistinguishable from that associated with methotrexate treatment, recovering when treated with steroids and gammaglobulin. measles infection is easily overlooked in the absence of rash. The diagnosis may be suggested by clinical and radiological features and confirmed by specific immunofluorescence staining of lung biopsy tissue.
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