Nationwide epidemiological survey of chronic hypersensitivity pneumonitis in Japan.

BACKGROUND: In 1999, a Japanese epidemiological survey of chronic hypersensitivity pneumonitis (HP) showed that summer-type HP was the most prevalent variant of the disease. The number of reported cases of chronic HP has recently been increasing, and the clinical features of the disease seem to have changed. We conducted another nationwide epidemiological survey of chronic HP in Japan to determine better estimates of the frequency and clinical features of the disease. METHODS: A questionnaire was sent to qualified hospitals throughout Japan, and data on cases of chronic HP diagnosed between 2000 and 2009 were collected. RESULTS: In total, 222 cases of chronic HP from 22 hospitals were studied. Disease subtypes included bird-related HP (n=134), summer-type HP (n=33), home-related HP (n=25), farmer's lung (n=4), isocyanate-induced HP (n=3), and other types (n=23). The median proportion of lymphocytes in bronchoalveolar lavage fluid was high (24.5%). The primary findings of computed tomography of the chest were ground-glass attenuation and interlobular septal thickening. Centrilobular fibrosis was the major pathological finding on examination of surgical lung biopsy specimens from 93 patients. The median survival time was 83 months. CONCLUSIONS: The proportion of bird-related HP was higher than that in the previous epidemiological survey, and the proportions of isocyanate-induced HP and farmer's lung were lower. A crucial step in diagnosing chronic HP is to thoroughly explore the possibility of antigen exposure.

[A case of Churg-Strauss syndrome with subarachnoid hemorrhage and left phrenic nerve paralysis].

A 60-year-old woman was given a diagnosis of Churg-Strauss syndrome (CSS) in 2000 because of peripheral blood eosinophilia, eosinophilic pneumonia, asthma, polyarticular pain, and limb numbness. She was treated with prednisolone (PSL), and the above symptoms improved but then relapsed on tapering of PSL. In September 2009, after 7 days of tapering of PSL to 5mg/day, the patient developed a subarachnoid hemorrhage and was admitted. MRA and cerebral angiography revealed no aneurysm; the source of bleeding could not be determined, but her symptoms indicated a benign course. A chest X-ray 27 days after admission showed left diaphragmatic elevation, and left phrenic nerve paralysis was diagnosed by a phrenic nerve stimulation test. Peripheral blood eosinophilia had progressed gradually during the admission period, and although it is rare for subarachnoid hemorrhage and phrenic nerve paralysis to be associated with CSS, we regarded these as vasculitis symptoms related to CSS.

[A case of melioidosis occurring after a long-term stay in Vietnam that developed pulmonary cavitation and relapsed with multiple pulmonary nodules].

A 69-year-old man complained of fever in September 2009, after returning from Vietnam where he has been working for 20 years. He had diabetes mellitus and was on diabetic oral medication. He was examined at a nearby hospital, and found out to have pneumonia with cavity formation in the right upper lobe which was found out to be not due to tuberculosis. Although the patient once recovered with antibacterial medicine, after a few months, in January 2010, he was admitted to our hospital because of recurrent fever. Computed tomography revealed multiple pulmonary nodules which were thought to be pulmonary emboli, as well as subcutaneous abscess, spleen abscess, and kidney abscess. Blood test showed that he also had DIC. As Burkholderia pseudomallei was cultured from the subcutaneous abscess and blood, was diagnosed as melioidosis. The patient was treated with meropenem for 8 weeks, and then a maintenance oral antibacterial medicine was continued for the next 6 months. The patient fully recovered after those treatments and has not relapsed since then. This is the ninth case report of melioidosis in Japan which is an imported infectious disease.

[Clinical features of reversed halo sign in cryptogenic organizing pneumonia].

Reversed halo sign (RHS) is often seen in computed tomography (CT) scans of cryptogenic organizing pneumonia (COP). To investigate its clinical features, we retrospectively reviewed 30 cases of COP in 13 men and 17 women, whose age range 28 to 73, with a mean of 58.4 years. All diagnoses were made with transbronchial lung biopsy (TBLB), but it took an average of 24.8 days from the first visit until the diagnosis of COP. RHS was seen in 7 cases (23%) and multiple RHSs were seen in 3 cases. We treated 5 cases (71%) with steroids. Their CT images showed parenchymal abnormalities which started as nodular lesions, then enlarged, and then the central lesion changed into ground-glass opacities, until finally, RHS was formed. The presence of RHS does not necessarily indicate a marked difference in the clinical course of COP. In conclusion, in the present series RHS was a phase of the clinical course of COP, and was useful to diagnose COP.

[Pulmonary arterio-venous malformation protruding from the pleural surface in a case of Rendu-Osler-Weber disease].

A 57-year-old woman complaining of dyspnea on exertion and left back pain was admitted to our hospital. Chest X-ray films revealed a nodular lesion in the lower left lung field. Enhanced chest computed tomography (CT) scans demonstrated a nodular lesion communicating with a dilated pulmonary artery and vein in the subpleural region of the left S9. The presence of cutaneous telangiectases, repeated epistaxis and a family history of arteriovenous malformation (AVM) indicated a diagnosis of pulmonary AVM during a course of Rendu-Osler-Weber disease. Lobectomy of the left lower lung was performed. The AVM appeared to be located in the subpleural region of the left S9 on chest CT images. However, it was actually located in the left S9 and was protruding from the pleural surface. The wall of the AVM appeared very flimsy and was likely to rupture easily.

Churg-Strauss syndrome versus chronic eosinophilic pneumonia on high-resolution computed tomographic findings.

OBJECTIVES: The aim of this study was to compare the high-resolution computed tomographic findings between Churg-Strauss syndrome (CSS) and chronic eosinophilic pneumonia (CEP). METHODS: We retrospectively reviewed the clinical records of 16 patients with CSS and 34 patients with CEP. RESULTS: Twelve (35%) of the 34 patients with CEP had a history of asthma. Although the subpleural distribution of ground-glass opacities (GGOs) and consolidation was common both in CSS and CEP, the midzone distribution was more frequent in CSS (44%) than in CEP (12%). Centrilobular nodules within GGOs were significantly more frequent in CSS (56%) than in CEP (18%). In contrast, traction bronchiectasis associated with volume loss was demonstrated more frequently in CEP (74%) than in CSS (25%). CONCLUSIONS: On high-resolution computed tomography, the presence of the midzone distribution and nodules within GGOs without traction bronchiectasis suggests CSS rather than CEP.

[A case of extranodal NK/T-cell lymphoma, nasal type, with skin ulceration and multiple nodules in the lung].

We report a rare case of extranodal NK/T-cell lymphoma, nasal type, with skin ulceration and multiple nodules in the lung, and will compare this case with others in the literature. A 54-year-old man was troubled with flare and swelling of his right arm for one month. He was referred to our hospital because of subcutaneous nodules with ulceration on the right arm, fever, and general fatigue. Chest radiograph revealed multiple nodular shadows in both lungs. The patient was admitted for further evaluation. Chest computed tomography revealed multiple nodules in both lungs with ground-glass opacities around some of them. Some nodules seemed to be clustered in the right lower lobe. Biopsy specimens of subcutaneous nodules and transbronchial biopsy specimens of pulmonary tissue demonstrated only extensive necrosis. A VATS lung biopsy from the right S9 was then taken. Pathological and immunohistological findings suggested a diagnosis of extranodal NK/T-cell lymphoma, nasal type (WHO classification), stage IVB. The patient was transferred to the department of hematology in another hospital for systemic chemotherapy, but died 2 months later.

[A case of hypersensitivity pneumonitis resulting from inhalation of Aspergillus niger in a greenhouse worker who raised roses].

A 57-year-old woman was referred because of exertional dyspnea, fever, and cough in June 2006. She had been employed to culture roses in greenhouses since 1991 and had developed a cough during the summer from 2003. Chest CT scan revealed diffuse centrilobular micronodules. Transbronchial lung biopsy specimens demonstrated alveolitis with lymphocytes and non-necrotizing epithelioid cell granulomas. After admission, both the patient's symptoms and laboratory data improved without medication. However, upon her return to work in the greenhouse, cough and exertional dyspnea reappeared. Aspergillus niger was detected in the greenhouse. Her serum was assayed for precipitating antibodies against various antigens, and precipitating antibodies against Aspergillus fumigatus, Aspergillus flavus, Aspergillus glaucus, and Aspergillus niger were demonstrated. In a double immunodiffusion test, cross-reactivity between Aspergillus niger and other Aspergillus species was indicated. Consequently, she was diagnosed as having hypersensitivity pneumonitis resulting from the inhalation of Aspergillus niger.

[A case of sarcoidosis with pleural involvement but with no mediastinal or hilar lymph node enlargement].

A 66-year-old woman was referred to our hospital because of right chest pain on inspiration. Chest X-ray film revealed diffuse opacities, predominantly in the lower lung field, and right pleural thickening. Serum ACE and lysozyme levels were elevated. Chest CT revealed diffuse ground-glass opacity, centrilobular granular nodules, bronchovascular bundle irregular thickening and right pleural irregular thickening over the right inferior lobe, but bilateral hilar lymph node enlargement was not present. Bronchoalveolar lavage (BAL) fluid demonstrated increased numbers of total cells and CD4 T-helper lymphocytes. The transbronchial lung biopsy specimen revealed non-caseating epithelioid cell granulomas. From these findings, we established a diagnosis of type III sarcoidosis with pleural involvement. The patient has been observed without treatment, and 10 months later the lung involvement and pleural thickening have disappeared.

[An autopsy case of invasive thymoma extending to the right atrium with superior vena cava syndrome as the initial manifestation].

We report a case of invasive thymoma presenting with superior vena caval obstruction and intracardiac extension. A 74-year-old man was admitted in July 2002 with swelling of the face and right upper extremity. Computed tomography of the chest revealed a small anterior mediastinal mass, which infiltrated the lumen of the superior vena cava extending into the right atrium. Invasive thymoma was strongly suspected, but he refused any medical treatment. His health declined steadily, with repeated right-sided heart failure. He died due to cardiac tamponade 50 months after his first visit. On autopsy, the tumor was diagnosed as a thymoma classified as type B3 according to the WHO histological classification. Formation of a tumor thrombus in the superior vena cava and the right atrium is a rare mode of extension of thymoma. In this respect, our case may be valuable for improving the understanding of the natural course of invasive thymoma.