Search

Background: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients., Methods: Sarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied. Patients with pulmonary artery wedge pressure (PAWP) of 15 mmHg or less and a mean pulmonary artery pressure (mPAP) ≥ 25 Hg were subsequently analyzed. Data collected included hemodynamics, forced vital capacity (FVC), diffusion capacity of carbon monoxide (DL CO ), chest x-ray, and 6-min walk distance (6MWD)., Results: A total of 176 patients were analyzed. This included 84 (48%) cases identified within a year of entry into the registry and 94 (53%) with moderate to severe PH. There was a significant correlation between DL CO percent predicted (% pred) andmPAP (Rho = -0.228, p = 0.0068) and pulmonary vascular resistance (PVR) (Rho = -0.362, p < 0.0001). PVR was significantly higher in stage 4 disease than in stage 0 or 1 disease (p < 0.05 for both comparisons). About two-thirds of the SAPH patients came from the United States (US). There was a significant difference in the rate of treatment between US (67.5%) versus non-US (86%) (Chi Square 11.26, p = 0.0008) sites., Conclusions: The clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers., (Copyright © 2018. Published by Elsevier Ltd.)

Urothelial carcinoma (Transitional cell carcinoma) of the bladder is the pre-dominant histological type of bladder cancer in the United States and Europe. Patients with bladder cancer usually present with painless hematuria. The diagnosis is often delayed, as the symptoms are similar to various other benign conditions such as urinary tract infection, prostatitis or renal calculi. In some patients, the metastatic lesions will cause the initial presenting symptoms. We conducted a MedLine/PubMED search identifying all relevant articles with "pulmonary manifestations", "urothelial bladder cancer", "manifestations of bladder cancer" or a combination of these terms in the title. The pulmonary manifestations of urothelial carcinoma of the bladder include metastatic disease including cavitary lesions, endobronchial, pleural, or lymph node metastasis pleural effusion and chylothorax. Pulmonary embolism and tumor embolism is another manifestation of this cancer. Intravesical Bacillus Calmette-Gurin therapy for bladder cancer has been associated with a range of adverse effects including the systemic spread of Bacilli Calmette-Guérin immunotherapy affecting the lungs. Other drugs used to treat bladder cancer can be associated with drug-related pneumonitis. Other rare manifestations include a sarcoid like reaction and systemic granulomatous disease to Bacilli Calmette-Guérin therapy. In this review we discuss the various pulmonary manifestations of urothelial carcinoma of the bladder. A high index of suspicion with these presentations can lead to an early diagnosis and assist in instituting an appropriate intervention., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Renal cell carcinoma (RCC) accounts for majority of all primary renal neoplasms. Classic manifestations of RCC include the triad of flank pain, hematuria and a palpable renal mass. Patients with RCC can develop various extra renal manifestations including involvements of the lungs, inferior vena cava, liver and the bones. The pulmonary manifestations of renal cell carcinoma include metastatic disease including endobronchial, pleural, parenchymal or lymph node metastasis, pleural effusion or hemothorax. Pulmonary embolism and tumor embolism is another common manifestation of renal cell carcinoma. RCC is a highly vascular tumor and can cause pulmonary arterio-venous fistulas leading to high output failure. Rarely, RCC can also present with paraneoplastic presentations including cough or bilateral diaphragm paralysis. Drugs used to treat RCC have been associated with drug related pneumonitis and form an important differential diagnosis in patients with RCC on therapy presenting with shortness of breath. In this review we discuss the various pulmonary manifestations of RCC. A high index of suspicion with these presentations can lead to an early diagnosis and assist in instituting an appropriate intervention., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Background: Hot tub lung is an emerging lung disorder associated with exposure to Mycobacterium avium complex organisms contaminating hot tub water., Objectives: To define the clinical characteristics and outcome of patients with hot tub lung., Methods: Retrospective review of 21 patients diagnosed with hot tub lung at a tertiary medical center over a 7-year period., Results: The mean (+/-sd) age at presentation was 46 (+/- 15) years; 9 patients were men (43%). All patients described ongoing exposure to hot tubs. The most common referral diagnoses were sarcoidosis, bronchitis, and asthma. Dyspnea and cough were present in all patients, hypoxemia was noted in 10 patients (48%). High-resolution computed tomography of the chest had been performed in 20 patients and demonstrated diffuse centrilobular nodules and/or ground-glass opacities in all patients. M. avium complex was isolated from the hot tub water, respiratory secretions and/or lung tissue in all patients. Bronchoscopic or surgical lung biopsy was obtained in 18 patients and demonstrated bronchiolocentric granulomatous inflammation. With avoidance of exposure, clinical and radiologic improvement was observed in all patients. Additionally, 13 patients (62%) received corticosteroid therapy, 1 (5%) antimycobacterial therapy, 2 (10%) received both, and 5 patients (24%) received no pharmacologic therapy., Conclusions: Hot tub lung likely represents hypersensitivity pneumonitis due to inhalational exposure to M. avium complex. Antimycobacterial therapy does not appear to be required in the management of this disease. Although corticosteroids may be helpful in the treatment of severely affected patients, others can be managed by avoidance of additional exposure alone.

It is assumed that sarcoidosis is caused by inhalation of air borne agents in susceptible persons triggering the inflammatory reaction. The association of metallic dust exposure, such as beryllium and aluminium, and sarcoidlike pulmonary disorders is well known. The ability of man-made mineral fibres (MMMF) to cause granulomatous lung disease has not been appreciated until now. Recently, we observed the association of sarcoidlike granulomatous reaction and occupational history of glass fibre exposure. We hypothesized that there might be a relationship between MMMF exposure and the development of sarcoidlike granulomas. Therefore, the records of 50 sarcoidosis patients-who visited our outpatient clinic between 1996 and 1999 were reviewed. This revealed that 14 cases recalled a history of exposure to either glass fibres or rock wool, both MMMF fibres. The available obtained tissue specimens (n = 12) were reviewed. In six cases electron microscopy qualitative analysis of small fragments of the tissue revealed among others silica, aluminium and sometimes titanium. A distinct relation between fibre deposits fibre deposits and granulomas was found. These findings indicate that in susceptible people MMMF exposure might be related to a chronic granulomatous disease similar to chronic beryllium disease.