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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a brainstem predominant lymphocytic inflammatory disease, which often relapses without oral immunosuppressants. This report describes a 37-year-old male case of CLIPPERS without relapse for 1 year after early steroid treatment. He was admitted to our hospital because of sensory disturbance in the left side of his body and ataxic gait. Gadolinium-enhanced T 1 -weighted MRI revealed multiple punctate and curvilinear enhancements in the pons and right middle cerebellar peduncle. We started treatment with high-dose intravenous methylprednisolone (IVMP) therapy on the 20th day of the illness. His neurological symptoms dramatically improved. Follow-up MRI showed that the enhancing lesions disappeared. We diagnosed him with CLIPPERS based on the clinical course, radiological findings, and steroid response. He did not take any oral immunosuppressant after discharge. However, there was no clinical and radiological relapse for 1 year after the IVMP therapy. Although this case requires careful follow-up because of recurrence risk, early steroid treatment was possibly related to 1-year remission.

A 73-year-old male was admitted in January 1999 with hyperimmunoglobulinemia with a serum IgG level of 6530 mg/dl, bilateral eyelid tumors, bilateral submandibular swelling, and swelling of the superficial lymph nodes. A left submandibular gland biopsy showed severe chronic sialoadenitis with fibrosis. A left cervical lymph node biopsy showed invasion by many mature lymphocytes and plasma cells, but no lymphoma cells. The patient was diagnosed as having so-called Mikulicz disease associated with a disease similar to multicentric Castleman disease (MCD) characterized by multicentric lymphadenopathy and polyclonal hyperimmunoglobulinemia. Steroid therapy (prednisolone, 20 mg/day) was effective in reducing the symptoms, the bilateral eyelid tumors and the swelling of the bilateral submandibular glands and superficial lymph nodes. The lack of any increase of serum IL-6 suggested that this case had a hitherto unknown etiology, other than MCD.

A 27-year-old man initially had low back pain and ankle arthralgia. He was admitted because fever, cough, and polyarthralgia developed and continued for three months. Chest X-ray and CT revealed bilateral hilar and mediastinal lymphadenopathy with pulmonary lesions. Furthermore, elevated serum-ACE level and noncaseating epitheloid cell granuloma obtained by TBLB confirmed the diagnosis of sarcoidosis. After hospitalization, erythema nodosum appeared and ocular involvement was demonstrated. As a result, this case fulfilled the criteria of Löfgren's syndrome (arthritis, erythema nodosum, and BHL). Löfgren's syndrome is not uncommon in European countries, but is extremely rare in Japan. So far, only six cases with Löfgren's syndrome were reported in Japan, and all were female cases. This is the first male case in Japan. Löfgren's syndrome is usually a self-limiting disease. We used steroids for this case and remission has been maintained after the beginning of the treatment for the past one year.

The lungs, eyes and skin are affected in most cases of sarcoidosis. We encountered a rare case of sarcoidosis which presented extensive lymphadenopathy, various skin lesions, lesions in the thoracic cavity with bilateral pleural effusion, eosinophilia (43% in the periphery blood).

A 53-year-old man in whom reticulonodular shadows had been noted since 1999 in chest radiographs was admitted to our hospital in January 2003 with a complaint of persistent cough. His thoracic computed tomography (CT) showed diffuse ground-glass opacities in both lung fields and bilateral hilar and mediastinal lymphadenopathy. Histopathological findings from video-assisted thoracoscopic surgical biopsy included diffuse, monotonous interstitial pneumonia with accumulation of macrophages in the alveolar spaces and many lymphoid follicles, which was compatible with desquamative interstitial pneumonia (DIP). There was follicular hyperplasia in the enlarged lymph node without a suggestion of granulomous disease. He responded well to corticosteroid administration, resulting in an improvement of his clinical condition associated with a decrease in the ground-glass opacities. The bilateral hilar and mediastinal lymphadenopathy was slightly reduced. Since we could find no literature reporting DIP with bilateral hilar and mediastinal lymphadenopathy on thoracic CT, we consider the diagnostic problems and therapeutic response of this case to warrant a brief report.

A 46-year-old man had had an occasional dry cough in the early morning since about the age of 20, but had received no treatment. He had been taking an antirheumatic drug for 2 years for rheumatoid arthritis. The patient complained of fever and dry coughing that began in the middle of November 1995, and he was treated for acute bronchitis. His condition did not improve, and he was admitted to the hospital in early December. Wheezing and rhonchi were heard in both lung fields. His white blood cell count was 19,000/mm3, and the eosinophil percent age was 48%. A chest CT scan revealed macular lesions with an increased density in both lung fields, and markedly swollen mediastinal and hilar lymph nodes. Analysis of alveolar lavage fluid revealed an increased number of cells (total) and eosinophilia (37%), and examination of a transbronchial lung biopsy specimen indicated infiltration with eosinophils and lymphocytes. Our diagnosis was eosinophilic pneumonia. The patient's condition improved soon after the start of pulse therapy with steroids. Bilateral swelling of mediastinal and hilar lymph nodes is rare in patients who have pulmonary in filtration with eosinophilia (the PIE syndrome).