[A case of sarcoidosis acutely aggravated with high fever and diffuse interstitial pulmonary infiltrates].

We present a case of sarcoidosis acutely aggravated with high fever and diffuse interstitial pulmonary infiltrates in a female patient at the age of 64. Sarcoidosis was diagnosed in another hospital as a result of iritis, chest radiography findings, and a negative reaction in a tuberculin skin test. She was admitted to our hospital because of dyspnea and a high temperature of 39 degrees C in February 1994. A marked hypoxemia (PaO2 46.5 torr) was found in arterial blood gas analysis. Chest radiography revealed a bilateral diffuse reticulo-nodular shadows, and chest CT showed ground glass opacity predominant posteriorly. Analysis of bronchoalveolar lavage fluid revealed an increase in lymphocytes and an increased ratio of CD4 to CD8 T lymphocyte. Transbronchial lung biopsy revealed lymphocytic alveolitis and proliferation of epithelioid cell granulomas in the alveolar septa and intraalveolar spaces. The patient was treated for deterioration of sarcoidosis with 40 mg of prednisolone and her respiratory status and the radiographic findings improved rapidly. With dose tapering of prednisolone, dyspnea and deterioration of the radiographic findings occurred, but with addition of a weekly low dose of methotrexate, dose reduction of prednisolone was achieved.

Pulmonary tuberculosis with unusual cystic change in an immunocompromised host.

We present a rare case of upper zone cystic change of the lung with disseminated tuberculosis of a non-smoking 30-year-old immunocompromised male. He suffered from repeated pneumothorax. The basic pathological feature of video-assisted thoracoscopic lung biopsy revealed granulomatous involvement in the respiratory bronchioles with poorly developed epithelioid cells and disruption of elastic fibers. Electron microscopy demonstrated a decrease in elastic fibers and disruption of the epithelial basement membrane of the respiratory bronchiole and no Langerhans cells in the lesion. Autopsy of the lung revealed centroacinar distribution of multiple cystic lesions in the bilateral upper lobe. Almost all cystic walls showed loss of elastic fibers and cysts frequently involved the respiratory and terminal bronchioles, alveolar ducts and, occasionally, alveoli. Some larger cystic lesions revealed communication to the bronchi. The cystic changes in this case of pulmonary tuberculosis may be caused by a check-valve mechanism due to granulomatous involvement of the bronchioles and also by excavation of caseous necrotic material by draining bronchi.

[A case of Wegener's granulomatosis which showed early spontaneous remission].

An 80-year-old woman presented at our hospital on October 1995 with fever, hemoptysis and a cavitary shadow on chest X-ray. Blood examination revealed an accelerated erythrocyte sedimentation ratio and elevated CRP. Pulmonary cryptococcosis was suspected, but serological tests and bronchoscopic examination for cryptococcus were both negative. There was also no evidence of the tuberculosis or malignancy. She was treated with the antibiotic cefpirome sulfate intravenously for thirteen days. Her chest X-ray and abnormal blood test findings became almost completely normal following the i.v. antibiotic treatment. In February 1996 (2 months after her first admission), she had severe right cheek pain, and Coldwell Luc's operation was performed after right maxillary sinusitis was diagnosed. A high fever (39 degrees C) continued after surgery, and multiple cavitary shadows were seen on chest X-ray. Blood examination revealed an accelerated ESR, elevated CRP and slightly elevated c-ANCA. She was treated with i.v. infusion of antibiotics and antifungal drug's, but did not improve. Wegener's granulomatosis was diagnosed after transcutaneous lung biopsy and histopathological examination of the maxillary sinus. Dramatic improvement was seen following treatment with oral cyclophosphamide and prednisolone. Whether her first remission was due to antibiotic treatment or spontaneous is an interesting question.

Vascular involvement in cutaneous sarcoidosis.

Sarcoidosis is a systemic granulomatous disease with systemic vascular involvement, that is, granulomatous angiitis and microangiopathy. To determine if there is vascular involvement in cutaneous sarcoidosis, we examined 42 skin specimens taken from 32 patients with cutaneous lesions of sarcoidosis. Cutaneous sarcoidosis was prevalent in older females with high serum angiotensin-converting enzyme (ACE) levels. Most skin lesions appeared during the following-up of sarcoidosis. Granulomatous angiitis was present in 12 specimens of sarcoid skin lesions (30.8%). Eight of the 12 specimens showed venous involvement in the dermis. There was no correlation between the incidence of granulomatous angiitis and the gross pattern of cutaneous sarcoidosis. Immunohistochemically, thrombomodulin was negative in the vascular endothelium close to the granuloma or a periphlebitis lesion. Electron microscopy revealed endothelial swelling, luminal narrowing, and basal lamina layering of the basement membrane in the capillaries and venules in the dermis. These findings demonstrated that granulomatous angiitis and microangiopathy coexist in cutaneous sarcoidosis.

Ultrastructural features of alveolitis in sarcoidosis.

Seventy-nine specimens of lung parenchyma from 61 patients with sarcoidosis were examined ultrastructurally with a focus on alveolitis, and they were compared with specimens of hypersensitivity pneumonitis (HP) and the percentage of lymphocytes in bronchoalveolar lavage fluid (BALF). Lymphocytes and monocytes were frequently observed in the capillary lumina, and these cells and macrophages were found in the interstitium of the alveolar walls in the specimens with alveolitis of sarcoidosis and HP. Increases in the percentage of lymphocytes in BALF correlated with the degree of alveolitis. Swelling and bleb formation of the endothelium of alveolar capillaries and changes in the capillary basement membrane were commonly found and were significantly increased in the specimens with alveolitis. The frequency of bleb formation was significantly higher in alveolitis of sarcoidosis than in that of HP. However, the changes in alveolar epithelium were not significant in sarcoidosis. Microvascular changes in alveolitis are not specific for sarcoidosis, but they are observed in other interstitial lung diseases. These alterations may play an important role in the development of pulmonary sarcoidosis.

[Clinical evaluation of cefpodoxime proxetil, a new oral cephem, in the treatment of respiratory tract infections with the special reference to community-acquired Streptococcus Pneumoniae infections].

The clinical usefulness of cefpodoxime proxetil (CPDX-PR) was investigated in the treatment of pneumonia and chronic airway infections occurring in patients first visiting our outpatient clinic or those being treated at the outpatient clinic. CPDX-PR was orally administered twice a day after meals at a dose of 100-200 mg for acute respiratory tract infections and at a dose of 200 mg for chronic respiratory tract infections. Excellent, good, fair, and poor responses were observed in 20, 33, 10, and 3 of 66 patients (4 with acute bronchitis, 27 with pneumonia, and 35 with acute exacerbation of chronic airway infection), respectively, demonstrating an 80.3% efficacy rate (53/66). Causative organisms, including Streptococcus pneumoniae, were all eradicated from the patients whose causative organisms were examined over time, although 2 of the patients were superinfected with Pseudomonas aeruginosa. There were no serious adverse reactions or abnormal changes in laboratory test results. It was concluded that CPDX-PR could be used as a first-choice drug for the treatment of respiratory tract infections at an outpatient clinic, and that this drug should acquire greater importance in particular consideration of recent increases in infections with S. pneumoniae.

Pulmonary vascular involvement in sarcoidosis: granulomatous angiitis and microangiopathy in transbronchial lung biopsies.

To evaluate the occurrence of granulomatous angiitis and microangiopathy in the lung with sarcoidosis, transbronchial lung biopsy specimens were examined from 174 cases with sarcoidosis. Granulomatous angiitis was seen in 72 cases, which corresponded to 53% of the cases with granulomata. Granulomatous angiitis showed venous involvement (65%), both venous and arterial involvement (24%) or arterial involvement only (11%). There was no significant difference in occurrence of granulomatous angiitis between upper and lower lobes. The cases with granulomatous angiitis in the lung had a higher frequency of ophthalmic symptoms and elevated serum angiotensin converting enzyme level. Basal lamina layering in the microvasculature was more often observed in the bronchial mucosa than in the alveolar walls and is not exclusively related to granulomata. Endothelial proliferation and basal lamina alterations in granulomatous angiitis may be closely associated with granulomas. The present study revealed coexistence of granulomatous angiitis and microangiopathy in the lung with sarcoidosis and suggests that both may participate in the development of pulmonary sarcoidosis.

A case of pneumocytoma (so-called sclerosing hemangioma) with lymph node metastasis.

A case of "sclerosing hemangioma" (pneumocytoma) of the lung with lymph node metastasis is reported. A 22-year-old Japanese man was found to have a well-defined round lesion in the right lung (S7), which increased in size slightly during a 2-year follow-up period. He underwent right lower lobectomy with a preoperative diagnosis of a benign lung tumor. The pulmonary tumor revealed histological features characteristic of "sclerosing hemangioma" of the lung, in addition to which there were many large polygonal foamy cells, forming tubular or papillary structures. These cells were found by electron microscopy to contain numerous cytoplasmic lamellar bodies and showed a positive reaction with anti-surfactant apoprotein antibody immunohistochemically. Therefore, they were considered to be cells differentiating toward type II pneumocytes. Review of 21 typical "sclerosing hemangiomas" disclosed a few or some such foamy cells in 10 cases. A single hilar lymph node was the site of microscopic metastases, which consisted of "large clear foamy cells" and smaller polygonal or round cells with slightly eosinophilic cytoplasm, both of which were components of the pulmonary "sclerosing hemangioma." This case supports the theory that "sclerosing hemangioma" is a neoplasm of type II pneumocyte lineage. Although it is said to be benign, rare cases apparently show metastatic potential.