Nephrotic Syndrome and a Retroperitoneal Mass: A Case Report of a Patient with Recurrent Invasive Thymoma.

A 68-year-old man was admitted to our hospital to undergo an examination for nephrotic syndrome while concurrently complicated with recurrent thymoma in the parietal pleura and retroperitoneum. He had been diagnosed with invasive thymoma and had undergone thymo-thymectomy seven years previously. Based on the renal biopsy findings, his nephrotic syndrome was ascribed to minimal change disease. He was treated with corticosteroid monotherapy, which resulted in complete remission six months later, despite the fact that the recurrent thymoma remained. The role of thymoma in the pathogenesis of paraneoplastic glomerulopathy and the therapeutic concerns that emerged in this case are also discussed.

Clinical features of pulmonary aspergillosis associated with interstitial pneumonia.

OBJECTIVE: We retrospectively investigated the clinical features of pulmonary aspergillosis associated with interstitial pneumonia. METHODS: We reviewed the medical records of all patients treated for interstitial pneumonia with or without pulmonary aspergillosis at our institution between April 2006 and August 2012 and evaluated the clinical features as well as risk and prognostic factors for pulmonary aspergillosis associated with interstitial pneumonia. RESULTS: Among 539 patients with interstitial pneumonia, 15 who suffered from pulmonary aspergillosis were identified. The median age was 69.2±7.0 years, and fourteen patients were men. The subtypes of pulmonary aspergillosis were chronic pulmonary aspergillosis (n=14) and invasive pulmonary aspergillosis (n=1). The forms of interstitial pneumonia included idiopathic pulmonary fibrosis (n=9), rheumatoid arthritis-related interstitial pneumonia (n=4) and pleuroparenchymal fibroelastosis (n=2). The underlying conditions were emphysema (n=9) and a history of oral corticosteroid and/or immunosuppressive use (n=4). Home oxygen therapy (HOT) was administered in 11 patients. Following the diagnosis of pulmonary aspergillosis, all patients were treated with antifungal drugs. Ten patients (66.6%) died. A comparison of the interstitial pneumonia patients with and without pulmonary aspergillosis showed that the presence of emphysema, use of HOT and death were significantly associated with pulmonary aspergillosis. CONCLUSION: Pulmonary aspergillosis is one of the major complications of interstitial pneumonia and its prognosis is poor. Therefore, providing careful monitoring and proper treatment is extremely important.

Clinical significance of interleukin 33 (IL-33) in patients with eosinophilic pneumonia.

BACKGROUND: Interleukin 33 (IL-33) works as a functional mediator in allergic disease by enhancing the activity of eosinophils and inducing expression of T helper 2 (Th2)-associated cytokines. However, the role of IL-33 in pulmonary eosinophilia has not been elucidated. We investigated the levels of IL-33 in eosinophilic pneumonia (EP) together with associated cytokines, and discussed the clinical significance of IL-33 in EP. METHODS: Sera and bronchoalveolar lavage fluid (BALF) were obtained from 16 patients with EP, including acute eosinophilic pneumonia (AEP) and chronic eosinophilic pneumonia (CEP). Twelve patients with acute respiratory distress syndrome (ARDS) were also included for comparison. The concentration of IL-33 and Th2 cytokines (IL-4, IL-5, IL-13) were measured by enzyme-linked immunosorbent assay (ELISA). RESULTS: The concentration of serum IL-33 was significantly higher in patients with AEP than in CEP. In CEP, only patients with atopic factors showed mild increase of serum IL-33. The concentration of BALF IL-33 was also significantly elevated in AEP, however, it remained quite low in CEP. Among Th2 cytokines, IL-5 was significantly increased in both serum and BALF in AEP, and the level of IL-5 was positively correlated with that of IL-33. ARDS showed no increase of serum and BALF IL-33. CONCLUSIONS: The remarkable increase of BALF IL-33 in AEP indicated the local production of IL-33 in lungs. IL-33 is considered to be a local key molecule for triggering pulmonary eosinophilia, together with IL-5. BALF IL-33 appears to be a useful marker for discriminating AEP from CEP and ARDS.

A rare case of asymptomatic diffuse pulmonary ossification detected during a routine health examination.

A 26-year-old man presented at our hospital in 2008 to undergo detailed investigations as part of a routine health examination. Chest computed tomography (CT) showed linear and reticular opacities with, in part, diffuse calcification in the lung fields bilaterally. A surgical lung biopsy was performed and the histological findings were compatible with a diagnosis of diffuse pulmonary ossification (DPO) of the dendriform type. DPO usually occurs as a secondary disease. As the histological changes in interstitial fibrosis were minimal rather than diffuse and not significant enough to be regarded as interstitial pneumonia, we considered this to be an idiopathic case. However, the findings appear to suggest that inflammation and fibrosis were associated with ossification.

[Interstitial pneumonia with anti-PL-7 antibody difficult to distinguish from rheumatoid lung].

A 63-year-old woman had abnormal shadows on a chest radiograph taken on a medical examination during treatment for rheumatoid arthritis. The chest radiograph showed linear and reticular shadows and ground glass opacities in the bibasilar lung fields. Though we considered rheumatoid arthritis due to joint disorder and a positive reaction for serum anti-CCP antibody, there were no bone lesions or articular narrowing on radiographs of the fingers. We diagnosed antisynthetase syndrome after we obtained a positive reaction for serum anti-PL-7 antibody. Antisynthetase syndrome demonstrates findings similar to those of rheumatoid arthritis, and this disease should be taken into consideration if chest computed tomography demonstrates consolidation with predominant patchy and irregular bronchovascular bundle thickening with a subpleural distribution.

Primary cardiac angiosarcoma of the right auricle with difficult-to-treat bilateral pleural effusion.

A 70-year-old woman was admitted to our hospital with pleuritis and pericarditis. Cytological examination of pleural and pericardial effusion, and pleural biopsy specimens under thoracoscopy revealed no specific pathological findings. The pleural effusion was drained continuously; however, she died of circulatory insufficiency at day 45 from admission. At autopsy, a fragile hemorrhagic mass arising from the right auricle had invaded bilateral pleura and the pericardium directly without distant metastasis. Immunohistochemical staining showed that the tumor cells expressed endothelial markers such as CD31 and CD34 antibodies, and factor VIII-related protein. These findings supported the diagnosis of a poorly differentiated angiosarcoma.

Clinical features of lymphangioleiomyomatosis complicated by renal angiomyolipomas.

OBJECTIVE: Renal angiomyolipomas (R-AMLs) are major complications of lymphangioleiomyomatosis (LAM). The objective of this study was to better understand the influence of R-AMLs in patients with LAM on the prognosis and other clinical factors related to respiration, and to investigate the management of R-AMLs in patients with LAM. PATIENTS AND METHODS: We retrospectively investigated the clinical features of 7 patients with LAM [4 were TSC (Tuberous sclerosis complex)-LAM and 3 were S (sporadic)-LAM] complicated by R-AMLs admitted to our hospital from 1997 to 2008. RESULTS: All patients were females and the mean age at diagnosis of LAM was 40.7 years (31.7 years for TSC-LAM and 52.7 years for S-LAM). Although 5 patients had symptoms related to R-AMLs, only 1 patient experienced symptoms related to R-AMLs at the time of diagnosis. Five patients had bilateral and 2 patients had unilateral R-AMLs. R-AMLs ruptured in 4 cases (3 patients were TSC-LAM) including 2 patients in whom they ruptured bilaterally, and who underwent bilateral nephrectomy. In 1 case, unilateral R-AMLs grew larger and appeared on the other side during the follow-up period. CONCLUSION: Although only rare cases of LAM show symptoms related to R-AMLs initially, R-AMLs are a notable complication. To avoid nephrectomy, R-AMLs should be diagnosed when they are small and should be followed up carefully by periodic echograms or CT scans.

Long-term efficacy of inhaled N-acetylcysteine in patients with idiopathic pulmonary fibrosis.

BACKGROUND: Inhalation of N-acetylcysteine (NAC) has been carried out in our department since 1994 for treating interstitial pneumonia such as idiopathic pulmonary fibrosis (IPF). In this study, the clinical efficacy and safety of long-term NAC inhalation monotherapy for IPF was investigated. METHODS: NAC inhalation was carried out in 23 of 34 cases diagnosed as IPF by surgical lung biopsy in our department between 1994 and 2008. The treatment was continued for one year or longer in 14 cases. In these 14 cases and in 11 cases without treatment, the clinical courses, prognosis, lung function (%FVC, %DLco, and %TLC), and changes in serum markers for interstitial pneumonia (KL-6 and SP-D) were examined. RESULTS: There were no significant differences in survival curves between the two groups. Acute exacerbation was observed in 4 of 14 cases (28.6%) receiving NAC inhalation. Compared with the results just before the beginning of NAC inhalation, Δ%FVC and Δ%DLco in the treated cases were -4.7% and -2.9% one year later and -4.0% and -5.8% two years later, respectively. In cases without treatment, Δ%FVC and Δ%DLco were -3.5% and +5.3% one year later and +0.2% and +1.0% two years later, respectively. CONCLUSION: Since this study is an open case-control study in a single institute and the number of cases is not large, its use in evaluating the efficacy of NAC inhalation monotherapy is limited. In addition, the role of NAC inhalation in combination with a steroid, an immunosuppressive agent, and a new anti-fibrosis drug should also be investigated.

[Case of minocycline-induced acute eosinophilic pneumonia accompanied by marked neutrophilia in the peripheral blood].

A 37-year-old woman presenting with high fever, dry cough and progressive dyspnea was admitted to our hospital. She took 100 mg of minocycline hydrochloride orally because of a common cold one day prior to her admission. A chest CT scan showed diffuse ground-glass opacities with interlobular septal thickening and thickening of bronchovascular bundles. An analysis of bronchoalveolar lavage fluid showed an increase in both the total cell counts and the number of eosinophils. The result of a lymphocyte stimulation test performed on peripheral blood lymphocytes was positive for minocycline. This patient had a history of pneumonia with similar clinical and radiographic findings, which had developed while receiving minocycline. As a result, we made a diagnosis of minocycline-induced acute eosinophilic pneumonia. Her symptoms and radiographic findings improved within a few days after admission. Corticosteroid therapy was effective. A marked increase of peripheral blood neutrophils were noted on admission. The serum levels of IL-8 and G-CSF increased at the early phase of the disease, but thereafter decreased in association with neutrophils, thus suggesting the contribution of these cytokines to the early phase neutrophilia in this case.

[A case of paraneoplastic cerebellar degeneration associated with small cell lung cancer showing marked response to intravenous immunoglobulin].

BACKGROUND: Paraneoplastic cerebellar degeneration (PCD), one of the paraneoplastic neurological syndromes (PNS), develops a subacute cerebellar dysfunction and its neurological prognosis is poor in most cases. Because it is considered to be immune-mediated, immunosuppressive therapy may be effective, but its past outcomes are not constant. CASE: A 59-year-old woman presented with deteriorating cerebellar manifestations such as ataxic gait, nausea, and dysarthria. Chest CT revealed a nodule in the upper lobe of the right lung, and small cell lung cancer was diagnosed by transbronchoscopic lung biopsy and sputum cytology. Although known antineuronal antibodies were not detected, no other causative diseases were recognized, so PCD associated with SCLC was diagnosed. The cerebellar manifestations improved remarkably with immediate intravenous immunoglobulin (IVIG) therapy. SCLC showed complete response (CR) after chemoradiotherapy, and the neurological symptoms have not worsened at present. CONCLUSION: Treatment with IVIG at the early stage may lead to the improvement of PCD and is worth attempting.

[Case of pulmonary coccidioidomycosis presented with multiple infiltrative opacities].

A 54-year-old man was admitted to our hospital to be operated on for inguinal hernia, but complained of dry cough and cervical lymph nodes swelling after traveling to California in the United States. The chest X-ray films taken on admission showed consolidations in both lungs. The laboratory data revealed an increase in white blood cell counts with eosinophilia, and elevated ESR, IgE and beta-D-glucan. The biopsied lung specimen by VATS showed epithelioid granulomas consisting of giant cells and eosinophils. In addition, spherulitic forms filled with endopores were detected in the specimen. A diagnosis of primary pulmonary coccidioidomycosis was made. Eosinophilia and elevated in IgE and beta-D-glucan were closely related to the severity of the disease gravity after the treatment with fluconazole.

[Nationwide epidemiological survey of patients with idiopathic interstitial pneumonias using clinical personal records].

A nationwide epidemiological survey of idiopathic interstitial pneumonias (IIPs) in 2005 was performed using clinical personal records. According to the data from the Japan Intractable Diseases Information Center, 4,396 patients with IIPs were covered by public insurance. The prevalence of IIPs in Japan was estimated to be 3.44 per 100,000. The forms of 1543 patients (new: 658, recurrent: 885) were collected. Of 1543 cases, 1322 cases (85.7%) had idiopathic pulmonary fibrosis (IPF), therefore details were investigated in patients with IPF. The mean age at onset was 65.4 years and men were more frequent than women (878 in men, 444 in women). The severity, diagnostic method, symptoms, imaging findings, and pulmonary function tests were analyzed in the new forms. Severity levels of I, II, III and IV were seen in 32, 28, 177, and 287 cases, respectively. A pathological diagnosis was made in 67 cases (12%). The positive rates of fine crackles, dry cough, exertional dyspnea, and finger clubbing were 98%, 94%, 98%, and 53%, respectively. On HRCT, honeycomb was noted in the subpleural lung lesions in more than 90% of the cases. As for pulmonary function tests, restrictive and diffusing capacity impairment were noted in 86% and 92%, respectively, and SpO2 was below than 90% in 91% on a 6-minute walking test. The serum KL-6 level was elevated in 95%. Most of the IPF analyzed cases were at a severity level of III or IV because the analysis aimed at those coverage by public insurance. Since this was only the initial nationwide epidemiological survey in Japan, the current situation should be investigated.