[A case of systemic IgG4-related disease with bilateral pleural effusions].

An 85-year-old man was admitted for non-productive cough and bilateral pleural effusion on a chest X-ray film. Although his pleural effusion was exudative and showed an increased number of lymphocytes and a high level of adenosine deaminase, Mycobacterium tuberculosis infection, even with a microbiological culture, PCR and interferon-gamma release assay, was unverifiable. Cytology of his pleural effusion demonstrated plasmacytoid-like large atypical cells. Immunological tests showed high levels of IgG and IgG4 in both his serum and pleural effusion. A fluorodeoxyglucose-positron emission tomography (FDG-PET) scan showed uptake of FDG in orbital lesions, salivary glands, gastric wall, biliary system and lymph nodes (cervical, supraclavicular, axillary, hilar, mediastinal, along the lesser gastric curvature). Histological examination of an orbital pseudotumor and gastric mucosal biopsy revealed that numerous numbers of mostly IgG4 positive plasma cells, had accumulated. Based on these findings, systemic IgG4-related disease was diagnosed. Corticosteroid therapy was started and the volume of pleural effusion markedly declined. We report a rare case of IgG4-related disease accompanied by pleural effusion with a high level of ADA.

[A case of Epstein-Barr virus-positive diffuse large B-cell lymphoma in an elderly person with a necrotizing pulmonary bulky mass].

A 52-year-old man presented with a pulmonary bulky mass, fever and cough. Chest CT showed a necrotizing bulky mass 10 cm in diameter in the right lung lower lobe. Transbronchial biopsy and CT-guided biopsy of the tumor and endobronchial ultrasound-guided transbronchial needle aspiration of the right hilar lymph node did not yield a definitive diagnosis, but the histological findings showed necrosis. We performed CT-guided biopsy of the part of the lesion where a high uptake of FDG-PET was observed. The histological diagnosis was diffuse large B-cell lymphoma. The immunohistochemical findings were positive for Epstein-Barr virus (EBV). Because the patient was more than 50 years old and had no underlying diseases, he was given a diagnosis of EBV-positive diffuse large B-cell lymphoma of an elderly patient. The pulmonary manifestation of this disease as a bulky tumor is extremely rare. In spite of the presence of a bulky pulmonary tumor, the EBV-positive diffuse large B-cell lymphoma contained necrotizing tissue, and it was difficult to obtain tissue specimens for histological examination.

[A case of hepatopulmonary syndrome with elevated exhaled nitric oxide].

A 76-year-old man was admitted to our hospital because of dyspnea. Three years before admission, dyspnea was recognized and had been given a diagnosis of interstitial pneumonia by a general physician. A year later, he received home oxygen therapy. After admission, we found that he had alcoholic liver cirrhosis and an increased alveolar-arterial oxygen level in his arterial blood gas. Moreover, he had an intrapulmonary vascular shunt, detected by contrast-enhanced echocardiography and perfusion scan with 99mTc-macroaggregated albumin. These results confirmed hepatopulmonary syndrome. Furthermore, exhaled nitric oxide (NO) was elevated in the patient although he had never had bronchial asthma or any other allergic diseases. Animal models of hepatopulmonary syndrome have shown that exhaled NO is associated with dilated vessels. To the best of our knowledge, this paper describes the first case of hepatopulmonary syndrome with elevated exhaled NO in Japan.

Diagnostic usefulness of endobronchial ultrasound-guided transbronchial needle aspiration in a case with malignant pleural mesothelioma.

A 74-year old man was admitted to our hospital with right diffuse pleural thickening and effusion and with subcarinal lymph node swelling. The effusion obtained by thoracentesis showed no malignant cells, although positron emission tomography showed abnormal uptake in the right pleural thickening and subcarinal lymph node. Histopathological examination of the lymph node specimens obtained by endobronchial ultrasound-guided transbronchial needle aspiration showed a sheet of epithelioid-like large atypical cells. The tumor cells were immunohistochemically positive for calretinin and cytokeratin 5/6, and negative for CEA and TTF-1. Therefore, malignant pleural mesothelioma of epithelioid type was diagnosed.

Usefulness of endobronchial ultrasonography for transbronchial lung biopsies of peripheral lung lesions.

BACKGROUND: Peripheral lung lesions are increasing in numbers. Endoscopic diagnosis is essential for the prevention of unnecessary operations. Conventional diagnostic procedures have limitations in availability and results. OBJECTIVES: Endobronchial ultrasonography (EBUS) was investigated as a means to guide transbronchial lung biopsy, to reduce the discomfort during the procedure and to improve diagnostic accuracy. METHODS: In 50 cases, we performed transbronchial lung biopsy combined with EBUS and fluoroscopic guidance. The results were compared to 42 controls assessed by fluoroscopy only. RESULTS: In 38 cases (76%), EBUS could describe the peripheral lesion (33 from inside, including 9 cases with difficulties in fluoroscopic observation, and 5 from an adjacent bronchus, indicating the correct location of the lesion). If successfully placed inside, a change in the patient's position was not required, which helped to reduce patient discomfort. Lung cancer was diagnosed in 24 patients and benign disease in 25 patients; in 1 case diagnosis remained unknown. When the EBUS probe could be introduced inside the lesion, the sensitivity for cancer diagnosis and specificity for cancer exclusion were 100%, respectively (15/15, 18/18). Compared to the controls in whom the biopsy site was determined by fluoroscopy only, the sensitivity tended to be superior by EBUS, although it did not reach statistical significance (p = 0.06). However, specificity and accuracy were statistically significant (both p = 0.02). CONCLUSIONS: When the lesion can be correctly described by EBUS from inside the lesion, EBUS is useful to guide transbronchial lung biopsy, can contribute to a reduction in patient discomfort and improves the accuracy of diagnosis. Additional navigation tools to increase correct positioning of the EBUS probe are desirable.