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A 22-year-old Japanese man was found to have bilateral hilar lymphadenopathy (BHL), and was diagnosed with sarcoidosis in 1995. He was followed without treatment until 2002, when a bone fracture due to osseous sarcoidosis was found in his left thumb. Despite systemic treatment with corticosteroid and methotrexate, a new bone lesion developed in his right foot and his right middle finger was fractured. The patient also suffered multiple organ involvements including brain and muscle lesions. This is the first report of a sarcoidosis patient who presented with BHL, and developed bone fractures after a long stable period of more than 5 years.

Four patients with sarcoidosis presented as hypothalamic-hypophyseal syndrome including diabetes insipidus (DI) were followed up for more than 8 years from the onset of clinical manifestation. The mean age was 26 years, male : female ratio was 3 : 1 and the mean disease duration of 10 years. All patients had hypogonadism, hyperprolactinemia. Pituitary enlargement with thickening of the pituitary stalk were detected by magnetic resonance imaging (MRI) with gadolinium enhancement and attenuation in the intensity of the posterior lobe of the pituitary was detected without enhancement. Corticosteroid therapy resulted in the initial improvement of symptoms and gradual decrease in the tumor size but failed to cure polyuria due to DI. The use of desmopressin was necessary for a long period. None of these patients died from DI or central neurosarcoidosis.

We report a case of genital sarcoidosis that presented characteristic features on MRI. A 25-year-old man sarcoidosis patient with ocular and lung lesions presented a painful mass in the left scrotum together with systemic symptoms of fever, appetite loss, headache, and stomach-ache during the tapering of steroids. The patient was hypercalcemic, and this was thought to be the cause of his systemic symptoms. MRI showed multiple nodules of bilateral testes and enlargement of bilateral epididymis; the patient was diagnosed with testicular and epididymal lesions of sarcoidosis. An increased steroid dosage improved his hypercalcemia and genital lesions.

We report the case of a 58-year-old woman who was considered to have died of cyclophosphamide (CPA)-induced late-onset lung disease. She underwent a right-sided mastectomy due to breast cancer at the age of 50 followed by daily administration of 50 mg CPA and 20 mg tamoxifen for 2 years. A refractory cough and dyspnea began at the age of 56. Chest radiographs revealed diffuse infiltrates and pleural thickening. Her vital capacity was markedly reduced. Corticosteroid therapy was ineffective. The disease rapidly progressed with occasional episodes of pneumothoraces to her death. Postmortem examination revealed pulmonary fibrosis with marked elastosis.

Objective: We attempted to evaluate the feasibility (therapeutic efficacy, tolerance, and clinical courses after treatment) of pirfenidone, an anti-fibrotic drug for patients with chronic pulmonary fibroses such as idiopathic pulmonary fibrosis (IPF)., Methods: Open-label one-year treatment for compassionate-use., Patients or Materials: Oral pirfenidone (40 mg/kg body weight) was administered to 8 patients with advanced IPF and 2 with interstitial pneumonia associated with diffuse systemic sclerosis. The plasma concentration of the drug was serially followed. Radiographic scores, pulmonary functions, and arterial blood oxygen pressure were compared at three time points: at one-year before treatment, at the time of entry, and at one-year after entry., Results: While pirfenidone did not show a definite therapeutic effect on overall survival (2 years after entry). During one-year treatment, there was no significant deterioration in terms of chest radiographic scores and arterial oxygen pressure, and the drug was well tolerated with minimal adverse effects within the ranges observed in this study, plasma pirfenidone concentrations did not seem to relate to the appearance of adverse effects or differences in therapeutic effects., Conclusion: The feasibility of Pirfenidone as a therapeutic drug was confirmed for patients with advanced pulmonary fibrosis.