scholarly journals Constrictive bronchiolitis obliterans in patient with Castelman’s disease

2005 ◽  
Vol 63 (4) ◽  
Author(s):  
E. Radzikowska ◽  
J. Pawlowski ◽  
M. Chabowski ◽  
R. Langfort

A 37-year-old woman with hialin- vascular type Castelman’s disease (CD) localised in the retroperitoneal region, incompletely resected, developed progressive dyspnoea. The chest radiograph taken 3 months before the operation was normal. The chest CT scan revealed diffused bronchiectases, hyperinflation and air trapping. Pulmonary function tests disclosed severe obstructive impairment with hyperinflation. The bronchoscopic examination of the bronchial tree was normal. Cultures of sputum, bronchial washing and blood were negative. No pemphigus antibodies were found. Mycoplasmal, chlamydial and viral infections were excluded. Histological examination of specimens obtained by open lung biopsy revealed bronchiolar inflammation, submucosal bronchial fibrosis with obliteration of bronchiolar lumen. Constrictive bronchiolitis obliterans (CBO) was diagnosed. Despite slight clinical and spirometric improvements that were achieved due to corticosteroid therapy, one year later she died as a result of respiratory failure. It is widely known that patients with CD develop CBO during the course of paraneoplastic pemphigus. However we present the case of CBO and CD but without any symptoms of this condition.

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S226-S226
Author(s):  
Michael G Ison ◽  
Nelson Chao ◽  
Francisco M Marty ◽  
Seung Hyun Moon ◽  
Zhiji Zhang ◽  
...  

Abstract Background Respiratory viruses (RV), including respiratory syncytial virus (RSV), influenza, parainfluenza virus (PIV), and human metapneumovirus (HMPV), frequently lead to serious complications such as lower respiratory tract infections and death in allogeneic hematopoietic cell transplantation (HCT) recipients. We used a large US claims database to compare the total reimbursement (TR), health resource utilization (HRU) and clinical outcomes between HCT patients with and without RV infections (RVI). Methods We used the Decision Resources Group Real World Evidence Data Repository to identify HCT recipients with date of service for the procedure from 1/1/2012-12/31/2017. We estimated the reimbursements from submitted charges using a reimbursement to charge ratio of 0.425. We examined the study outcomes in the year following HCT in patients with and without RVI. We also used a generalized linear model to determine adjusted TR stratified by the presence or absence of any acute or chronic graft-versus-host diseases (GVHD) after adjusting for age, health plan, underlying disease, stem cell source, number of comorbidities, baseline costs, and follow-up time. Results The study included 13,363 patients, representing 22% of HCTs reported to CIBMTR for the study period, of which 1,368 (10%) were coded with an RVI in the year following HCT: 578 (4%) RSV, 687 (5%) influenza, 166 (1%) PIV, and 181 (1%) HMPV. Unadjusted median TR were $132,395 higher for any RVI ($139,439 RSV, $101,963 influenza, $185,041 PIV and $248,029 HMPV) compared to those without RVI (Table 1). Adjusted TR were significantly higher for patients with any RVI compared to patients without that infection (p< .01) with or without GVHD (Figure 1). Patients with any RVI had significantly longer length of stay (LOS) for the HCT hospitalization, readmission rate and LOS after HCT hospitalization compared to patients without RVI (p< 0.05) (Table 2). A significantly higher proportion of patients with any RVI had pneumonia as compared to patients without that infection, irrespective of presence of GVHD (p< .0001). Table 1: Total healthcare reimbursement within one year of undergoing allogeneic HCT for patients with and without respiratory viral infections Figure 1: Adjusted total reimbursements within one year of undergoing allogeneic HCT for patients with and without respiratory viral infections Table 2: Health resource utilization within one year of undergoing allogeneic HCT for patients with and without respiratory viral infections Conclusion Allogeneic HCT patients with RVI have a significantly higher burden of TR, health resource utilization and worse clinical outcomes such as pneumonia during one year of undergoing HCT, regardless of the presence of GVHD. Disclosures Michael G. Ison, MD MS, AlloVir (Consultant) Francisco M. Marty, MD, Allovir (Consultant)Amplyx (Consultant)Ansun (Scientific Research Study Investigator)Avir (Consultant)Cidara (Scientific Research Study Investigator)F2G (Consultant, Scientific Research Study Investigator)Kyorin (Consultant)Merck (Consultant, Grant/Research Support, Scientific Research Study Investigator)New England Journal of Medicine (Other Financial or Material Support, Honorarium for Video)Regeneron (Consultant, Scientific Research Study Investigator)ReViral (Consultant)Scynexis (Scientific Research Study Investigator)Symbio (Consultant)Takeda (Scientific Research Study Investigator)United Medical (Consultant)WHISCON (Scientific Research Study Investigator) Seung Hyun Moon, MD, MPA, AlloVir (Employee, Shareholder) Zhiji Zhang, MS, AlloVir (Independent Contractor) Aastha Chandak, PhD, AlloVir (Independent Contractor)


2015 ◽  
Vol 32 (5) ◽  
pp. 304-314 ◽  
Author(s):  
Azza A.G. Tantawy ◽  
Moustafa M.A. Barakat ◽  
Amira A.M. Adly ◽  
Fatma S.E. Ebeid ◽  
Manal F. El Shamaa ◽  
...  

2021 ◽  
Vol 9 ◽  
Author(s):  
Xiuhua Yu ◽  
Jiaoyang Wei ◽  
Yanchun Li ◽  
Lu Zhang ◽  
Hongming Che ◽  
...  

Backgroud: Postinfectious bronchiolitis obliterans (PIBO) is a rare respiratory disease. In recent years, the disease has been recognized and diagnosed increasingly in children. Pulmonary function is important for diagnosis, identifying the severity of the PIBO and monitoring progression. But there have been only a few studies that followed the evolution of PIBO on the basis of pulmonary function tests (PFTs).Objective: The study targeted the evolution of pulmonary function and bronchodilator response in a case series of Chinese children with PIBO.Methods: Twelve children between the ages of 6–99 months with PIBO were studied retrospectively from 2009 to 2019. Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), the FEV1/FVC ratio, and maximal midexpiratory flow velocity 25–75% (MMEF25−75%) were collected at each PFT, and bronchodilator responses were evaluated. Spirometric parameters were monitored over time, and generalized linear mixed models were used to analyze longitudinal panel data.Results: The median baseline PFT values for FVC, FEV1, the FEV1/FVC ratio, and MMEF25−75% were 41.6, 39.75, 90.7, and 22.2%, respectively. At the initial PFTs, 10 (83.3%) patients demonstrated a significant bronchodilator response. FVC and FEV1 increased by 8.212%/year and 5.007%/year, respectively, and the FEV1/FVC ratio decreased by an average of 3.537%/year. MMEF25−75% showed improvement at an average rate of 1.583% every year. Overall, FEV1 and MMEF25−75% showed different degrees of improvement after the use of inhaled bronchodilators at each PFT session for 10 patients, and FEV1 measures demonstrated significant (>12%) β2-bronchodilation in 56% of PFT sessions.Conclusions: Pediatric patients with PIBO showed an obstructive defect in pulmonary function. The FVC, FEV1, and MMEF25−75% improved as they grew older, while the FEV1/FVC ratio decreased. This may be due to the development of lung parenchyma more than airway growth. Airway obstruction in some patients improved with the use of β2 agonists.


2001 ◽  
Vol 35 (7-8) ◽  
pp. 894-897 ◽  
Author(s):  
Angel Segura ◽  
Ana Yuste ◽  
Ana Cercos ◽  
Pedro López-Tendero ◽  
Regina Gironés ◽  
...  

OBJECTIVE: To report a case of pulmonary fibrosis resulting from use of cyclophosphamide as chemotherapy to treat a patient with breast cancer. CASE SUMMARY: We describe the case of a 52-year-old woman with breast cancer who developed pulmonary fibrosis after four cycles of chemotherapy that included cyclophosphamide. Pulmonary function tests revealed the presence of a severe ventilatory restriction. The open lung biopsy revealed pulmonary fibrosis with vascular sclerosis and signs of pulmonary hypertension. DISCUSSION: Cyclophosphamide is an alkylating agent that has been associated with interstitial pneumonia and pulmonary fibrosis. The frequency of these unwanted effects is '1%. The clinical picture consists of the progressive appearance of dyspnea and a nonproductive cough that progresses to severe pulmonary insufficiency. The risk factors described for these complications have been the use of chemotherapy regimens that include other drugs with known pulmonary toxicities, the cumulative total dose, the addition of radiotherapy, and the use of high doses of cyclophosphamide. CONCLUSIONS: Even though the frequency of pulmonary fibrosis in patients treated with cyclophosphamide-based chemotherapy regimens is low, the presence of dyspnea and an interstitial pattern in a patient makes it necessary to consider that possible drug toxicity. The open lung biopsy is the most accurate diagnostic technique for these cases. The discontinuation of cyclophosphamide and treatment with corticosteroids is usually followed by clinical recovery in approximately 50% of patients and, in some cases, reversal of the lung injury.


2020 ◽  
Vol 9 (10) ◽  
pp. 3070
Author(s):  
Belén Atienza-Mateo ◽  
Sara Remuzgo-Martínez ◽  
Diana Prieto-Peña ◽  
Víctor Manuel Mora Cuesta ◽  
David Iturbe-Fernández ◽  
...  

In the present study, we aimed to report our experience with rituximab (RTX) in the treatment of patients with ILD associated with AD (AD-ILD) at a single center. For this purpose, clinical characteristics, radiological findings, and pulmonary function tests (PFTs) of RTX-treated AD-ILD-patients seen from May 2016 until March 2020 at a referral center for individuals with ILD were retrospectively reviewed. Additionally, an updated literature review was conducted. A total of 26 patients (mean age 58.3 ± 11.1 years at ILD diagnosis) was included. The most common ADs related to ILD were systemic sclerosis, idiopathic inflammatory myositis (including anti-synthetase syndrome) and rheumatoid arthritis. Non-specific interstitial pneumonia (n = 12) and usual interstitial pneumonia (n = 11) were the predominant radiological patterns. The sustained improvement in PFTs was observed from the start of RTX, with a statistically significant increase in DLCO from basal to one year after RTX (mean + 4.2%, p = 0.024). Overall, there were no differences when comparing PFT outcome according to the radiological pattern or the specific type of AD. In conclusion, RTX constitutes a good therapeutic option to preserve lung function in patients with AD-ILD, regardless of the radiological pattern or the underlying AD.


2017 ◽  
Vol 15 (3) ◽  
pp. 152-159 ◽  
Author(s):  
Li Huang ◽  
Yaping Xie ◽  
Huifeng Fan ◽  
Gen Lu ◽  
Jialu Yu ◽  
...  

Bronchiolitis obliterans (BO) is in general a rare and life-threatening form of non-reversible obstructive lung disease in which the bronchioles are compressed and narrowed by fibrosis and/or inflammation. The purpose of this study was to evaluate the clinical features of BO in pediatric patients and explore its risk factors. The medical records of 35 pediatric patients with BO at Guangzhou Women and Children’s Medical Center were evaluated. The age at onset of symptoms was 2–42 months (mean 13.3 ± 8.9 months), with age at diagnosis of 5 months–4 years (mean 17.8 ± 9.0 months). High-resolution computed tomography findings included mosaic pattern (100%), atelectasis (37.1%), air trapping (31.4%), and bronchiectasis (20.0%). Three patients received lung biopsies and mainly exhibited an inflammatory process surrounding the lumen of bronchioles. BO predominantly resulted from post-infectious causes (91.4%) which were primarily caused by adenovirus (50%), followed by Mycoplasma pneumoniae (46.7%) and influenza (20%). Pulmonary function tests (PFTs) showed severe and fixed airflow obstruction, decreased compliance, and increased resistance. No significant difference was found between before and after steroid treatment ( P > 0.05). Two patients died owing to severe pulmonary complications, one of whom had inherent immunodeficiency. Our study suggests that the occurrence of BO, especially post-infectious BO, in China is relatively high and might result from primary immunodeficiency diseases in severe cases. Recurrent aspiration pneumonia caused by congenital dysplasia of the larynx and vaccination not on schedule might be potential risk factors for persistent and recurrent BO.


2001 ◽  
Vol 8 (2) ◽  
pp. 98-101 ◽  
Author(s):  
Farah J Nasser-Sharif ◽  
Meyer S Balter

A case of symptomatic hypersensitivity pneumonitis with normal high resolution computed tomography (CT) scans is presented. The patient, a 32-year-old man with systemic lupus erythematosus, had a chronic, progressive history of respiratory symptoms, abnormal findings on examination and abnormal pulmonary function tests but normal high resolution CT scans of the chest. Diagnosis was made through open lung biopsy. Clinical improvement was seen on removal of the offending antigen. The literature on the utility of high resolution CT scans in hypersensitivity pneumonitis is reviewed.


2005 ◽  
Vol 12 (6) ◽  
pp. 317-320 ◽  
Author(s):  
EY Chan ◽  
Sharon D Dell

Idiopathic nontransplant-related childhood bronchiolitis obliterans is an uncommon disease. Most patients present with chronic recurrent dyspnea, cough and wheezing, which are also features of asthma, by far a much more common condition. The present case study reports on a six-year-old girl who presented to a tertiary care centre with recurrent episodes of respiratory distress on a background of baseline tachypnea, chronic hypoxemia and exertional dyspnea. Her past medical history revealed significant lung disease in infancy, including respiratory syncytial virus bronchiolitis and repaired gastroesophageal reflux. She was treated for 'asthma exacerbations' throughout her early childhood years. Bronchiolitis obliterans was subsequently diagnosed with an open lung biopsy. She did not have sustained improvement with systemic corticosteroids, hydroxychloroquine or clarithromycin. Cardiac catheterization confirmed the presence of secondary pulmonary hypertension. Treatment options remain a dilemma for this patient because there is no known effective treatment for this condition, and the natural history is not well understood. The present case demonstrates the need for careful workup in 'atypical asthma', and the urgent need for further research into the rare lung diseases of childhood.


2012 ◽  
Vol 17 (3) ◽  
pp. 42-46 ◽  
Author(s):  
T. A. Ruzhentsova ◽  
A. V Gorelov

The data on clinical, ECG, ultrasound and laboratory features of myocarditides that developed in children with acute respiratory viral infections (ARVI) have been presented in this article. The dynamics of changes and the identified possibility of forming the chronic pathology in a one year after the onset of the disease has been shown.


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