Adult-onset familial pulmonary fibrosis in Japanese brothers.

Two Japanese brothers were diagnosed in their 20s with familial pulmonary fibrosis, the pathological findings of which were consistent with usual interstitial pneumonia (UIP). However, an atypical characteristic was observed in the lungs of these brothers; 2-mm areas of 'honeycomb' were identified throughout the lungs, which is smaller than the generally observed 5-10 mm honeycombing seen in UIP. Fibroblastic foci were demonstrated in the second eldest brother, but not in the eldest, which indicates that the lungs of the eldest brother was in a more advanced stage of fibrosis. Their youngest brother and parents have no clinical evidence of pulmonary fibrosis. All five family members had low values for the diffusion capacity of the lung for carbon monoxide (DLCO), suggesting the presence of an inheritable disease and the existence of different phenotypes. The genomic DNA of the affected brothers was sequenced for the reported surfactant protein C (SP-C) gene mutations in patients with familial pulmonary fibrosis, but none was documented. It is necessary to clarify the presence of novel gene mutations of SP-C or other genes to explain these particular pathological findings and the low DLCO observed in this family.

[Developmental mechanism of the "lung ball"--with reference to the pathological findings of the lung in two resected cases].

The lung ball is a special type of pulmonary aspergillosis (PA) occurring often after chemotherapy for leukemia. Histologically the ball, with air crescent sign on roentgenogram, is compatible with necrotizing lung tissue admixed with Aspergilli. The lung ball differs entirely from the common "fungus ball" in its quality, though they are similar in roentgenological appearances. The present two cases were leukemia which showed pulmonary findings in their therapeutic course, resulting in lung resection. In both cases the lung ball was confirmed histopathologically. Immunostaining of the lung tissue for neutrophil elastase showed elastase in various sites in the bronchial wall, pulmonary blood vessels (artery, vein) and cavitary wall. In our previous studies, much importance was attached to the disturbance of the pulmonary circulation caused by fibrin deposition as a factor in the developmental course of the fungus ball type aspergillosis (semi-invasive type). The circulatory disturbance of the lung was recognized also in the present cases. This two-way destruction of the pulmonary tissue, resulting from both neutrophil elastase activities and pulmonary circulatory disturbances, were regarded as the most important factor for the development of the lung ball. There are few studies on aspergillar lung ball with regard to the above respects.

[A case of mediastinal metastasis of renal small cell carcinoma, performing CHOP therapy for an acute respiratory failure by the air way obstruction under the mechanical ventilation].

A case of mediastinal metastasis of renal small cell carcinoma was reported. A 45-year-old female was admitted to our hospital complaining of worsening dyspnea. The chest radiograph and CT showed stenosis of the bronchi bilaterally due to the mediastinal mass. Prior to pathological diagnosis, CHOP therapy with cyclophosphamide, adriamycin, vincristine and prednisolone was performed under mechanical ventilation for the purpose of resuscitation. The therapy was effective; and the was weaned from the mechanical ventilator, resulting in complete remission. Transbronchial samples from the left main bronchus confirmed that the mediastinal mass was identical pathologically to the renal small cell carcinoma (RSCC) resected 5 years before. As a result, the mediastinal mass was diagnosed as a metastatic focus of RSCC. RSCC is known to have one of the most unfavorable prognoses among malignancies. Despite efforts with chemotherapy, almost all cases that appear in the literature died within 1 year after diagnosis. No standard treatment modality has yet been established. Thus, the present case is rare because an RSCC relapse after a long free period went into remission as a result of CHOP therapy. It is suggested that CHOP therapy may well be an effective tool against RSCC, and could be a valuable choice for a treatment regimen.

Clinical features of recurrent and insidious chronic bird fancier's lung.

BACKGROUND: Chronic bird fancier's lung (BFL) can be subgrouped into two types. One subgroup of patients develops interstitial pulmonary fibrosis after recurrent acute episodes (recurrent BFL), and the other subgroup of patients has no history of acute episodes but has slowly progressive chronic respiratory disease (insidious BFL). OBJECTIVE: To define the clinical characteristics of both types of BFL and to provide clues for diagnosis. METHODS: We performed a retrospective review of the medical records of patients with chronic BFL who were evaluated between October 1992 and March 2001 at the Tokyo Medical and Dental University Hospital in Japan. Patients were evaluated for their clinical characteristics, including history, laboratory, and immunologic findings; imaging; bronchoalveolar lavage; and histologic findings. RESULTS: Thirty-two patients with chronic BFL were included in this study; 15 patients had recurrent BFL and 17 had insidious BFL. The patients with recurrent BFL tended to breed dozens of pigeons in a loft, whereas the patients with insidious BFL were likely to be exposed to smaller birds kept indoors. Specific antibodies against pigeon dropping extracts or budgerigar dropping extracts were positive in 87% of the recurrent BFL cases and 35% of the insidious BFL cases. Antigen-induced lymphocyte proliferation was positive in more than 90% of both groups. The upper lung field was frequently involved in both groups as demonstrated by chest radiographic findings. In all of the patients with insidious BFL, the diagnosis was confirmed by positive laboratory-controlled inhalation test results. CONCLUSIONS: Insidious BFL may be misdiagnosed as idiopathic pulmonary fibrosis if a careful history is not taken and antigen-induced lymphocyte proliferation, careful imaging evaluation, and laboratory-controlled inhalation challenge testing are not conducted. In contrast, the clinical findings of recurrent BFL are consistent with hypersensitivity pneumonitis induced by other antigens.

Clinicopathological features of chronic hypersensitivity pneumonitis.

OBJECTIVE: Only limited information exists concerning the clinical and pathological features of chronic hypersensitivity pneumonitis (HP) in Japan and elsewhere. We present data on clinicopathological features of chronic HP obtained through a Japanese nationwide survey. METHODOLOGY: We studied the clinical and pathological findings in 10 patients with chronic HP who underwent surgical lung biopsy or postmortem examination. RESULTS: There were three types of clinical course: six of the 10 patients had persistent symptoms followed by repeated acute episodes; two showed a subacute onset with persistent symptoms; and two exhibited an insidious onset. Five patients made no attempt to avoid antigen exposure and they all had progressive disease. Pathological findings indicated that lesions were mainly centrilobular with or without epithelioid cell granulomas in specimens obtained during the acute or subacute stage. In contrast, most patients in the chronic stage predominantly showed interstitial fibrosis with a usual interstitial pneumonia pattern. CONCLUSIONS: The pathological findings of chronic HP depend on the stage of the disease at tissue sampling.