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Objective: Our objective was to evaluate the effectiveness of combination therapy consisting of low-dose corticosteroids with weekly methotrexate in patients with cardiac sarcoidosis in whom long-term therapy is required. Combination therapy was selected because long-term standard corticosteroid therapy tends to result in various adverse effects and the steroid-sparing effects of methotrexate have been reported., Methods: This study was a small open-label study comparing long-term functional changes between patients who received combination therapy (5-15 mg/day of prednisolone and 6 mg/week of methotrexate) and patients who received corticosteroids alone. The comparative analysis was based on the following therapeutic indexes: ejection fraction (EF), left ventricular end-diastolic diameter (LVDd) on echocardiography, serum N-terminal fragment pro-brain natriuretic peptide (NT-proBNP) and cardiothoracic ratio (CTR) on plain chest radiographs., Patients: Seventeen patients with cardiac sarcoidosis were examined in the sarcoidosis clinic. Cardiac sarcoidosis was diagnosed based on the Japanese diagnostic guidelines published in 2006., Results: The EF was significantly stabilized in the combination therapy group but not in the corticosteroids alone group at three years after the first treatment. The CTR and NT-proBNP levels were significantly stabilized in the combination therapy group compared with those observed in the corticosteroids alone group at both three and five years after the first treatment. The LVDd values tended to be stable in the combination therapy group compared with those observed in the corticosteroids alone group. The combination therapy was associated with few adverse effects., Conclusion: Weekly methotrexate therapy with daily small doses of corticosteroids stabilized the EF, CTR and NT-proBNP levels in the serum without eliciting adverse effects longitudinally.

Object: This study was undertaken to investigate the frequency of pulmonary hypertension (PH), and clinical parameters associated with PH in patients with idiopathic interstitial pneumonias (IIPs) and collagen vascular disease-associated interstitial pneumonias (CVD-IPs)., Patients and Methods: Retrospective analyses were performed in 163 consecutively enrolled patients (78 IIPs and 85 CVD-IPs) who were being evaluated for PH by Doppler echocardiography. PH was defined as an estimated systolic pulmonary artery pressure (sPAP) of > or = 40 mmHg. The frequency of PH was evaluated and clinical parameters were compared between patients with PH and those without PH., Results: Among patients successfully evaluated for sPAP by echocardiography, 20 of 70 (28%) patients with IIPs and 17 of 80 (21%) patients with CVD-IPs had PH. Among patients with IIP and CVD-IP, those with PH were more frequently treated with supplemental oxygen. Patients with IIP who had PH tended to be older and had decreased % diffusion capacity of carbon monoxide (%DLCO). Additionally, in patients with IIP, sPAP was negatively correlated with %DLCO. However, this association was not found in patients with CVD-IP. Patients with CVD-IP who had PH were predominantly male., Conclusion: PH was frequently observed in patients with both IIPs (28%) and CVD-IPs (21%) in the chronic phase of the disease. PH should be evaluated in patients with IPs who need supplemental oxygen, IIP patients with decreased diffusing capacity, and in patients with CVD-IP.

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.

We report a case of a patient with anti PL-12 antibody accompanied by interstitial pneumonia and severe pulmonary hypertension. At first presentation, hyperkeratotic skin lesions were found, although the diagnosis of CVD was not conclusive. Lung histology showed diffuse fibrosing interstitial pneumonia predominantly in the subpleural regions. During the seven-year follow-up period, severe pulmonary hypertension developed, although the progression of lung fibrosis was relatively limited. Anti-PL12 antibody was detected, and therefore the patient was diagnosed as having antisynthetase syndrome. Lung histology and pulmonary arteriogram suggested that vascular involvement of the disease contributed to the development of severe pulmonary hypertension.

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.

Objective: To adapt the Nottingham Health Profile (NHP) for Japanese and to describe the results of the assessment of its psychometric properties., Methods: Assessments included test-retest reliability over approximately a 2-week interval, internal consistency and construct validity in 133 patients with COPD., Results: The distribution of scores indicated that most of the NHP sections exhibited a floor effect, although this is greatly reduced with the NHP-Distress scale. The test-retest reliability was above 0.8 for all sections when patients reporting any change in their health status rating were excluded. Cronbach's alpha coefficients reflected the number of items contained in each section. The internal consistency of the emotional reactions section at one timepoint and the physical mobility section at both timepoints were lower than expected to be higher. All sections except the pain section could be used to distinguish patients who reported their health status to be good or fair from those who rated it to be poor or very poor., Conclusion: The adaptation of the NHP for Japanese was successful. Most sections showed reasonable test-retest reliability, indicating that they produced acceptable levels of random measurement error. The internal consistency of the sections was confirmed, although the alpha values of the emotional reactions and physical mobility sections were lower than might be expected for scales of their length. Different sections of the Japanese NHP were shown to have known group validity.

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.

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.