Multiple endocrine neoplasia type I and Cushing's syndrome due to an aggressive ACTH producing thymic carcinoid.

Thymic carcinoid in multiple endocrine neoplasia type 1 (MEN 1) is previously reported as a non-ACTH producing tumor. The present case is a 39-year-old man with mortal outcome from thymic carcinoid and Cushing's syndrome with high plasma ACTH. The symptom was first observed at age 29 and was relieved after extended thymectomy, with reduction of ACTH level. The tumor was positive for ACTH, Grimelius silver staining and Chromogranin A. The finding of primary hyperparathyroidism, pituitary adenoma, and a novel germline nonsense mutation (W423X) established the diagnosis of MEN 1. Cushing's syndrome due to ACTH producing thymic carcinoid should be also considered as one phenotype of the MEN 1 spectrum.

[A case of bacteria-associated hemophagocytic syndrome induced by nosocomial pneumonia].

This study reviews the case of a 67-year-old man who initially presented with high fever and dyspnea. He was subsequently admitted for treatment of pneumonia. On admission, severe leucopenia and thrombopenia were observed in the peripheral blood, with significant phagocytosis of hematopoietic cells including erythroblasts and leukocytes by macrophages in the bone marrow. Following the administration of antibiotic therapy, an improvement in pneumonia and cytopenia symptoms was noted. Clinically, we diagnosed this case as bacteria-associated hemophagocytic syndrome (BAHS) with community-acquired pneumonia. In general BAHS is seen following severe hematologic disease or pneumonia, and so the treatment of BAHS is quite difficult. This particular case had no additional hematologic disorder, and all presenting symptoms of BAHS were resolved immediately following the improvement in pneumonia with post-antibiotic therapy.

[Pulmonary actinomycosis diagnosed through transbronchial lung biopsy (TBLB)].

Pulmonary actinomycosis is a rather rare chronic pulmonary infection. Its diagnosis may be difficult without biopsy, because its culture from sputum or bronchial secretion is rarely successful. We report a case of pulmonary actinomycosis diagnosed by means of TBLB. A 57-year-old man was admitted to our hospital because of hemosputum. The chest radiograph at the first visit revealed a nodular lesion in the right middle lobe. The biopsy examination showed sulfur granules of which PAS-positive mycelium was arranged in a radiating pattern. On the basis of these findings, we diagnosed pulmonary actinomycosis. So far, there have been only 4 reported cases of pulmonary actinomycosis diagnosed by TBLB in Japan. TBLB is worth trying in the diagnosis of pulmonary actinomycosis, because it can avoid surgical procedures, if successful.

[A case of sarcoidosis with rheumatic features (Löfgren's syndrome)].

Rheumatoid arthritis was diagnosed in a 30-year-old woman with erythema nodosum and arthritic symptoms since 1994, and she was treated with anti-rheumatic agents. Mediastinal and bilateral hilar lymphadenopathy and abnormal pulmonary shadows were detected in 1996, and she was admitted to our hospital in 1997. We also recognized the elevation of ACE and lysozyme, and found granulomas in a transbronchial lung biopsy and an arthrosis synovia biopsy. From these findings, sarcoidosis was diagnosed. Sarcoidosis demonstrating erythema nodosum, arthritis, and bilateral hilar lymphadenopathy is called Löfgren's syndrome. In Caucasians, Löfgren's syndrome is frequently encountered, but it is rare in Japanese. Our case had coexisting arthrosis symptoms, and satisfied the diagnosis criteria of rheumatic arthritis. Therefore, the differential diagnosis was important. We emphasize that it is necessary to consider Löfgren's syndrome when diagnosing patients with rheumatic features, even in Japan.

[Successful use of immunosuppressive agents for the treatment of progressive rheumatoid interstitial lung disease: a case report].

Interstitial pneumonia (IP) is sometimes a fatal complication of rheumatoid arthritis (RA). We describe a patient with progressive rheumatoid interstitial pneumonia, who responded to intravenous intermittent cyclophosphamide (IV-CY) and cyclosporine (CsA). A 62-year-old man with rheumatoid arthritis was admitted to this hospital because of dyspnea. Examinations on admission revealed that he had active RA with vasculitis and IP Initially, he responded to high-dose corticosteroid therapy. A lung biopsy performed after initial corticosteroid therapy revealed diffuse interstitial pneumonia with marked infiltrations of macrophages into the air spaces. On corticosteroid therapy with prednisolone 30 mg/day, the IP became exacerbated and was refractory to the current high-dose steroid treatment. He responded to intravenous cyclophosphamide, but his IP remained unstable. After CsA treatment was started, a clinical remission was obtained. In this case, CsA was the most effective agent tried. Clinical and pathological considerations led us to speculate that activated alveolar macrophages played a crucial role in the pathogenesis of steroid-resistant IP in this patient, and that the clinical remission induced by CsA may have been due to its inhibitory effect on alveolar macrophages.