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Therapy with sildenafil has been shown to decrease pulmonary vascular resistance and may improve functional status in patients with interstitial pneumonia (IP) and pulmonary hypertension (PH). Patients with IP and PH defined by a mean pulmonary artery pressure (MPAP) of > or = 25mm Hg on right-heart catheterization were followed up in an open-label study of sildenafil. A multilateral evaluation was conducted before, and after 3 months of therapy. We studied 11 patients [8 men and 3 women, mean age 66.5] 6 of whom had IPF (1 with usual interstitial pneumonia {UIP}), 2 with IIP, and 3 with collagen-vascular disease interstitial pneumonia (CVD-IP). The mean modified Medical Research Council (MRC) score was 3.0 +/- 0.89, baseline dyspnea index (BDI) score was 4.5 +/- 1.9, % VC was 58.7 +/- 15.6%, percentage of carbon monoxide diffusing capacity (%DLco) was 20.0 +/- 10.9%, six-minute walk distance (6MWD) was 269.8 +/- 105.5m, shuttle walking test (SWT) was 179.1 +/- 99.7m, St. George Respiratory Questionnaire (SGRQ) was 70.9 +/- 15.6, mean pulmonary artery pressure (MPAP) was 33.8 +/- 7.61mm Hg, and pulmonary vascular resistance index (PVRI) was 658.9 +/- 236.1 dynes x s x cm(-5) x m2. After 3 months of therapy, improvements in BDI (< or = -1), 6MWD (> or = 20%), SWT (> or = 20%), and SGRQ (< or = -7) were observed in 4, 2, 3, and 6 patients, respectively. Improvements in MPAP (< or = - 20%) and PVRI (< or = -20%) were observed in 2 and 3 patients, respectively. No parameter showed statistically significant differences. We conclude that sildenafil may improve dyspnea, exercise tolerance and health-related quality of life (QOL) in some IP patients with PH.

We retrospectively reviewed 9 consecutive cases of primary pulmonary cryptococcosis having no comorbidity. At diagnosis, seven had no subjective symptoms and two had subtle symptom. Chest CT scan showed nodular shadows in 8, while 3 cases had infiltrative shadows. Eight of the nine were diagnosed with histopathology obtained by transbronchial lung biopsy or CT-guided needle aspiration biopsy. We also assessed PHA and Con A lymphocyte stimulation tests to measure cellular immune function in 6, four of whom showed decreased reaction of lymphocytes. We successfully treated seven of the nine with fluconazole alone and used fluconazole as a maintenance regimen in two. No relapse or treatment failure was seen after completion of antifungal treatment. Six cases were seropositive for serum cryptococcus antigen titer at diagnosis and only one showed seroconversion. We concluded that the duration of therapy for primary pulmonary cryptococcosis should not be necessarily determined by serum cryptococcus antigen seroconversion.

We retrospectively reviewed 110 consecutive patients with interstitial lung diseases (ILDs) who underwent surgical lung biopsy over 13 years from 1989-2002. Surgical procedures, medical records, surgical complications and outcome including post-operative exacerbation of ILDs were assessed in order to clarify the safety and efficacy of surgical lung biopsy. Forty-one patients underwent open lung biopsy (OLB), while 69 patients received videothoracoscopic lung biopsy (VTLB). There was no significant difference in patient characteristics, pulmonary function, arterial oxygen pressure and therapy prior to surgery between the two groups. The mean number of biopsy specimens was 2.5. Left side was selected as the site of biopsy in 70 cases. Duration of surgery was not significantly different between 2 groups. Bleeding during operation was less in the VTLB group than in the OLB group, although the mean bleeding amount was only 16.5mL. The duration of thoracic drainage and length of hospital stay were shorter in the VTLB group. The overall rate of surgical complications was 10.9%, with no difference between the two groups. The overall 30-day post-SLB mortality was 6.4% and majority of these had a very poor prognosis pathologically. The diagnostic yield of all 110 cases was 98.2%. SLB is a relatively safe and efficient diagnostic method in the diagnosis of ILDs.

Although quality of life (QOL) has become an important aspect of lung cancer trials, it has rarely been evaluated in general practice. We have been assessing QOL in patients with advanced non-small-cell lung cancer in general practice. In this study, we retrospectively analyzed factors of the baseline QOL score in relation to response to chemotherapy and survival in 43 consecutive advanced non-small-cell lung cancer patients who received chemotherapy. QOL was evaluated with the QLQ-C30 of The European Organization for Research and Treatment of Cancer. In multivariate analysis, the cognitive functioning score was the only significant factor in both response to treatment and survival. In conclusion, the QOL score is an independent factor in both response to chemotherapy and survival even in general practice; and cognitive functioning is the most important factor.

We report a case with Langerhans cell histiocytosis appearing as an extra-pleural tumor. A 20-year-old man was transferred to our hospital because of right chest pain and fever. His chest X-ray showed an extra-pleural mass and chest CT scan showed a mass lesion with right 7th rib fracture. 67Gallium and bone scintigram showed uptake at the same site. We performed a CT-guided puncture biopsy. Pathological findings of the specimen showed diffuse proliferation of histiocytoid cells with some eosinophils. The histiocytes were positive for S-100 protein and CD1a on immunohistochemical stain. Langerhans cell histiocytosis was diagnosed. There was no other involvement of the disease except the rib. The tumor resolved only with smoking cessation and no recurrence was observed during the follow-up period. An association between smoking and progressions of the rib disease was suggested.

To examine the utility of the Pneumonia PORT prediction rule for evaluating the severity of community-acquired pneumonia (CAP) in Japan, 127 consecutive patients with CAP admitted during one-year period were recruited and stratified by the rule. The relationship between CAP severity determined by the rule and drug-resistant Streptococcus pneumoniae was also investigated. The number of the patients classified into each class was 20 (15.7%) in class I, 32 (25.2%) in class II, 32 (25.2%) in class III, 31 (24.4%) in class IV, and 12 (9.4%) in class V, respectively. Risk class-specific mortality rate was 0% in class I and II, 3.1% in class III, 3.2% in class IV, and 41.7% in class V, respectively. Twenty-seven patients (21.3%) were diagnosed as pneumococcal pneumonia. In terms of penicillin-susceptibility of Streptococcus pneumoniae, 19 strains (70.4%) were penicillin-susceptible Streptococcus pneumoniae (PSSP). Five (18.5%) were penicillin-intermediate Streptococcus pneumoniae (PISP), and 3 (11.1%) were penicillin-resistant Streptococcus pneumoniae (PRSP). Highly resistant pneumococci with penicillin MIC > or = 4.0 micrograms/mL was not observed. For severity of drug-resistant pneumococcal pneumonia, 3 patients infected with PISP were classified as class IV, 2 with PISP were as class V, 2 with PRSP were as class IV, and 1 with PRSP was as class V. The patients with drug-resistant pneumococcal pneumonia were classified as high risk classes (class IV to V), however, the mortality rate was low (0%). In conclusion, the Pneumonia PORT prediction rule is effective for evaluating the severity of CAP in Japan, especially in classifying the class V patients who are related to high mortality. The mortality rate of the patients with drug-resistant pneumococcal pneumonia was low.