Post-Breast Cancer Radiotherapy Bronchiolitis Obliterans Organizing Pneumonia.

BACKGROUND: Radiotherapy for breast cancer has been implicated in the development of bronchiolitis obliterans organizing pneumonia (BOOP). Patients may be asymptomatic or may have pulmonary and constitutional symptoms that are moderate or severe. Postradiotherapy BOOP usually develops during the 12 months after completion of radiotherapy and is characterized by ground-glass opacities in the radiation-exposed lung and frequently in the non-irradiated lung. METHODS: An updated literature search and review was performed to update the systematic review we conducted in 2014. Ten new publications were identified: 2 Japanese epidemiological studies, 1 Japanese case series study, 6 case reports, and 1 review article. RESULTS: The incidence of postradiotherapy BOOP was 1.4% in both Japanese epidemiological studies. Risk factors included increasing age, cigarette smoking, and increasing central lung distance. The case reports included 7 women who had breast cancer postradiation BOOP and 1 woman who had an ataxia telangiectasia mutated (ATM) gene mutation, which may increase radiation sensitivity. CONCLUSION: Postradiotherapy BOOP in women with breast cancer occurs at a rate of 1.0-3.0% and may occur in women with immune system dysfunction and genetic mutations.

Familial Mediterranean fever is associated with a wide spectrum of inflammatory disorders: results from a large cohort study.

Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from "FMF in Central Anatolia (FiCA) Cohort", comprising 971 (mean age 35.3 ± 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch-Schönlein purpura, Behçet's disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (p < 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.

A long-term retrospective study of patients with biopsy-proven cryptogenic organizing pneumonia.

Cryptogenic organizing pneumonia (COP) is characterized by good response to corticosteroids, but frequent relapses after reduction or cessation of treatment are noted. The incidence, risk factors of relapse, and long-term outcomes of patients with COP remain undetermined. Patients with COP from September 2010 to December 2017 were enrolled. Hospital and office records were used as data sources. Clinical information, lab examinations, chest radiographs, treatment courses, and follow-up data were collected. Relapse group was defined as worsening of clinical manifestations in combination with progression of radiographic abnormalities in the absence of identified causes. Eighty-seven patients with COP were enrolled. Of them, 73 patients were treated with corticosteroids with relapse rate yielding 31.5% (23 of 73). Eleven patients were treated with macrolides and none of them relapsed. Fever was more common (65.2% vs. 32.0%, p = 0.004), C-reactive protein (CRP) was higher (31.5 ± 39.4 mg/L vs. 17.5 ± 32.2 mg/L, p = 0.038), and diffusion capacity for carbon monoxide (DLCO) % predicted was lower (45.9 ± 14.2% vs. 57.6 ± 18.5%, p = 0.050) in relapse group compared to nonrelapse group. Four patients who presented with organizing pneumonia (OP) as the first manifestation were ultimately diagnosed with OP secondary to autoimmune disease in follow-up. We showed relapse was common in COP patients treated with corticosteroids, but the prognosis was favorable. Fever, elevated CRP, and a reduced DLCO were related to relapse. As OP may not always be cryptogenic, a careful follow-up should be programmed to diagnose the underlying systemic disease.

Characteristics and outcomes of patients hospitalized with interstitial lung diseases in Spain, 2014 to 2015.

To assess characteristics and outcomes of patients hospitalized with interstitial lung diseases (ILD) and to analyze patient's comorbidities, procedures, and in-hospital outcomes.We identified patients hospitalized with idiopathic pulmonary fibrosis and others ILD such as hypersensitivity pneumonitis, cryptogenic organizing pneumonia, lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, and sarcoidosis in Spain during 2014 and 2015.We identified 14,565 discharges among patients admitted for ILD in Spain during the study period: idiopathic pulmonary fibrosis (IPF) in 42.32% (n = 6164), sarcoidosis in 37.65% (n = 5484), hypersensitivity pneumonitis in 10.55% (n = 1538), cryptogenic organizing pneumonia in 7.06% (n = 1028), pulmonary Langerhans cell histiocytosis in 1.48% (n = 215), and lymphangioleiomyomatosis in 0.94% (n = 136). The most common associated comorbidities according to those included in the Charlson Comorbidity Index (CCI) were COPD, diabetes, and congestive heart disease. The presence of pulmonary hypertension increased the probability of dying in patients with idiopathic pulmonary fibrosis (OR 1.36; 95%CI 1.06-1.73). Patients with cryptogenic organizing pneumonia had the longest length of hospital stay and the highest percentage of hospital readmissions (23.64%). The highest IHM corresponded to the idiopathic pulmonary fibrosis (14.94%). Computed tomography of the chest was the procedure more used during admissions for ILD.IPF was responsible for larger percentage of hospital admission among ILD in our study. In addition, the IHM were higher in IPF patients in comparison with those with other ILD. The most common associated comorbidity in ILD according to those included in the CCI was COPD. Computed tomography of the chest was the procedure more frequently used.

Spectrum of interstitial lung disease from a tertiary care hospital in Karachi.

OBJECTIVE: To determine the clinical features and patterns of interstitial lung disease. METHODS: This retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised record of patients diagnosed with interstitial lung disease from January 2005 to December 2015. All patients aged 16 years and above diagnosed with interstitial lung disease on the basis of clinical features, radiological features on high-resolution computed tomography of the chest, and lung biopsies were included. SPSS 19 was used for data analysis. RESULTS: Of the 537 patients, 324(60.3%) of the participants were females. The overall mean age was 60.5±14.9 years. The most common co-morbid condition was diabetes mellitus in 72(13.4%) patients, followed by hypertension in 48(8.9%) and ischaemic heart disease in 21(3.9%). The most common interstitial lung disease was idiopathic pulmonary fibrosis in 217(40.4%) patients, followed by non-specific interstitial pneumonia in 106(19.7%), sarcoidosis in 82(15.3%) and connective tissue disease-related interstitial lung disease in 56(10.4%) patients. CONCLUSIONS: Idiopathic pulmonary fibrosis was found to be the most common interstitial lung disease subtype followed by non-specific interstitial pneumonia, sarcoidosis and connective tissue disease-related-interstitial lung disease.

Organizing pneumonia and occupational and environmental risk factors: a case-control study.

OBJECTIVES: A single-center case-control study was carried out to investigate the relationship between occupational and environmental exposure and organizing pneumonia (OP). METHODS: Thirty-seven cases of OP, including 25 cases of cryptogenic OP, and 111 controls were included. Occupational exposure was assessed retrospectively by an industrial hygienist and an occupational physician, through semi-quantitative estimates of exposure. An exposure score was calculated for each subject, based on probability, intensity, daily frequency, and duration of exposure for each period of employment. The final cumulative exposure score was obtained by summing exposure scores for all periods of employment. RESULTS: Significant associations with all-cause OP were observed for exposure to tetrachloroethylene (OR 13.33, CI 95% 1.44-123.5) and silica (OR 6.61, CI 95% 1.16-37.71). A significant association with cryptogenic OP was observed only for tetrachloroethylene (OR 31.6, CI 95% 1.64-610.8). No associations were found for environmental exposure. CONCLUSION: Despite its low statistical power, this work suggests that occupational risk factors could be involved in OP.

[Radiation-induced bronchiolitis obliterans with organizing pneumonia]. / Complications pulmonaires de la radiothérapie après cancer du sein : penser à la BOOP.

Bronchiolitis obliterans with organizing pneumonia is an inflammatory reaction that can occur as a consequence of various pulmonary affections. Radiotherapy is not the sole and systematic cause of bronchiolitis obliterans with organizing pneumonia. Radiation-induced should not be confused with post-radiation, dose-dependent, inflammatory pulmonary fibrosis, which is non-immunological and located within the irradiation field. The role of immunity, local inflammation and individual radiosensitivity in bronchiolitis obliterans with organizing pneumonia is not well defined. Bronchiolitis obliterans with organizing pneumonia represents 1% of irradiated patients with breast cancer. It results in fever (flu-like symptoms), a rather dry cough and dyspnea. In the post-radiation context, bronchiolitis obliterans with organizing pneumonia may be diagnosed several months and up to a year after breast irradiation. The treatment consists of prolonged steroids or immunosuppressants, which do not prevent chronicity in 15% of patients and death in up to 5% of cases, the remaining 80% of patients healing without sequelae.

Patterns of interstitial lung disease and mortality in rheumatoid arthritis.

Objective: To characterize a cohort of patients with RA who have interstitial lung disease (ILD) and to assess the utility of previously developed mortality staging systems [gender, age, lung physiology (GAP) and ILD-GAP]. Methods: All patients with RA and ILD seen at the Mayo Clinic from 1998 to 2014 were identified and manually screened for study inclusion. RA disease characteristics and pulmonary findings including high-resolution CT and pulmonary function testing were evaluated. Survival was estimated using Kaplan-Meier methods. GAP and ILD-GAP models were evaluated using c-statistics and standardized incidence ratios. Results: The study included 181 patients with RA-associated ILD (96% Caucasian; 48% females; 37% never-smokers). The mean age at ILD diagnosis was 67.4 years ( s . d . 9.9). The median time from RA diagnosis to ILD was 4.9 years (range -10.9-48.1). The median follow-up was 3.1 years (range 0.01-14.8). Age, RA disease duration and low initial diffusing capacity for carbon monoxide were predictive of premature mortality in multivariate modelling. Sex, smoking status, obstructive lung disease, seropositivity and erosive disease were not associated with mortality. The 5-year survival rate was 59.7% (95% CI 51.5, 69.2). Survival did not differ between usual interstitial pneumonia, non-specific interstitial pneumonia and organizing pneumonia ( P = 0.42). The GAP model performed well in this cohort for both discrimination and calibration (c-statistic 0.71, standardized incidence ratio 0.97). Conclusion: In this large single-centre cohort of patients with RA-ILD, most patients were seropositive and had a history of smoking. ILD most commonly occurred after the RA diagnosis. Mortality was high and did not differ among the types. The GAP model may be useful in assessing mortality risk.

Clinical features of organizing pneumonia associated with rheumatoid arthritis.

OBJECTIVES: To clarify the clinical features of organizing pneumonia (OP) associated with rheumatoid arthritis (RA) and to determine whether development of OP is related to RA activity. METHODS: A cross-sectional study was conducted, in which medical records of 499 consecutive RA patients who visited our hospital during one month were reviewed. OP was diagnosed by pathological findings by trans-bronchial biopsy or by clinical features (typical computed tomography findings, no causative agents, good response to glucocorticoids, and lack of response to antibiotics). RESULTS: Among 499 patients, OP was found in 19 patients and the estimated prevalence was 1.9-4.8%. No differences in clinical features were noted between the OP and non-OP groups. The mean age of OP development was 57.2 years and the period from the onset of RA to OP ranged from -4 to +34 years. Although 14 patients presented OP after the onset of RA, two developed OP before RA and three developed OP simultaneously with RA. Patients receiving tumor necrosis factor inhibitors also developed OP. RA disease activity just before onset of OP was low in 8 of 14 RA cases. At the onset of OP, only two patients showed exacerbations of arthritis, whereas most patients presented with fever and serum C-reactive protein (CRP) elevations. Glucocorticoids were effective for OP in all patients who received them. Relapse occurred in 4 of 19 cases. CONCLUSIONS: OP develops in approximately 4% of RA patients, which occurs independently from arthritis activity and at any time in RA patients.

Bronchiolitis obliterans organizing pneumonia in patients with autoimmune rheumatic diseases.

Bronchiolitis obliterans organizing pneumonia (BOOP) is defined by buds of granulation tissue within lung distal airspaces. The diagnosis requires the histopathologic evidence of organizing pneumonia along with a suggestive clinical and radiographic pattern. This disorder is characterized by a good response to corticosteroids and an excellent prognosis. It can occur in association with a broad spectrum of clinical conditions or can be isolated, in this last case named cryptogenic organizing pneumonia. We searched for BOOP in patients with autoimmune rheumatic diseases (ARD) in the literature, and we found 32 well-documented cases. We reported here demographic features, manifestations, treatment and outcome of patients with BOOP associated with ARD. Notably, BOOP can be the presenting feature in some patients with ARD; thus, a close follow-up of patients with BOOP is recommended.

Systematic review of postradiotherapy bronchiolitis obliterans organizing pneumonia in women with breast cancer.

BACKGROUND: Radiation therapy for breast cancer has been implicated in the development of bronchiolitis obliterans organizing pneumonia (BOOP). This inflammatory lung disorder was first noted in 1983, and there have been numerous reports of BOOP occurring in women who have had radiation therapy for breast cancer since 1995. This study was undertaken to perform a systematic review of postradiotherapy BOOP to determine the occurrence, presentation, treatment, and outcome. MATERIALS AND METHODS: A systematic literature review was conducted according to the guidelines provided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses report. RESULTS: The literature search yielded 10 Japanese epidemiological reports with 129 women, 4 case series reports with 36 women, and 24 case reports with 34 women. Common symptoms included fever, cough, and shortness of breath. Most patients received corticosteroid therapy, and duration of treatment ranged from 6 months to 1 year, although some patients received steroids for longer than 1 year because of relapse, which occurred in approximately one half of patients. No deaths have been reported. CONCLUSION: BOOP is a rare but significant complication from radiation therapy for breast cancer. Chest radiographic studies for women who report new respiratory symptoms during the postradiation period can be beneficial for early diagnosis and for guiding appropriate management.

Organizing pneumonia after stereotactic ablative radiotherapy of the lung.

BACKGROUND: Organizing pneumonia (OP), so called bronchiolitis obliterans organizing pneumonia after postoperative irradiation for breast cancer has been often reported. There is little information about OP after other radiation modalities. This cohort study investigated the clinical features and risk factors of OP after stereotactic ablative radiotherapy of the lung (SABR). METHODS: Patients undergoing SABR between 2004 and 2010 in two institutions were investigated. Blood test and chest computed tomography were performed at intervals of 1 to 3 months after SABR. The criteria for diagnosing OP were: 1) mixture of patchy and ground-glass opacity, 2) general and/or respiratory symptoms lasting for at least 2 weeks, 3) radiographic lesion in the lung volume receiving < 0.5 Gy, and 4) no evidence of a specific cause. RESULTS: Among 189 patients (164 with stage I lung cancer and 25 with single lung metastasis) analyzed, nine developed OP. The incidence at 2 years was 5.2% (95% confidence interval; 2.6-9.3%). Dyspnea were observed in all patients. Four had fever. These symptoms and pulmonary infiltration rapidly improved after corticosteroid therapy. Eight patients had presented with symptomatic radiation pneumonitis (RP) around the tumor 2 to 7 months before OP. The prior RP history was strongly associated with OP (hazard ratio 61.7; p = 0.0028) in multivariate analysis. CONCLUSIONS: This is the first report on OP after SABR. The incidence appeared to be relatively high. The symptoms were sometimes severe, but corticosteroid therapy was effective. When patients after SABR present with unusual pneumonia, OP should be considered as a differential diagnosis, especially in patients with prior symptomatic RP.

Non-atopic IgE and eosinophil cationic protein after allogeneic hematopoietic stem cell transplantation in children.

Allogeneic hematopoietic stem cell transplantation (HSCT) in childhood is associated with severe pulmonary complications, but the pathophysiologic mechanisms remain unclear. Our aim was to evaluate the association of total and specific IgE, eosinophil cationic protein (ECP) and eosinophilia in HSCT recipients with pulmonary complications. We prospectively measured total and specific serum IgE, eosinophils, and ECP before and 28, 100, and 180 days after HSCT. We included 30 children (age 2-17 years) undergoing HSCT. Nine patients had a history of previous atopy without being associated with pulmonary complications after HSCT until day +360. Specific IgE levels showed a decline after HSCT, associated with the absence of allergy symptoms, suggesting a reduction of atopy. Elevated total serum IgE levels occurred in seven patients on day +28 after HSCT. This elevation did not coincide with allergy symptoms. ECP showed no correlation with total allergy symptoms, eosinophilia, IgE levels, or pulmonary complications. There was a significant correlation (p = 0.0367) between ECP levels on day +28 and concurrent acute graft-versus-host disease (GvHD). Non-atopic serum ECP and IgE levels are elevated on day +28 after HSCT in children, with ECP showing a potential relation to acute GvHD.

A diagnostic dilemma of cryptogenic organising pneumonia.

Cryptogenic Organising Pneumonia is a rare lung condition, which has incidence of 6-9 cases per 1,000,000 people with onset at age group between 50-60. The pathogenesis of this condition remains unknown. It mimics like pneumonia but has a good outcome with steroid treatment. Early recognition is very important and treatment with steroid therapy can save lives. This case highlights the unusual cause of shortness of breath due to COP and co existing incidental severe AS where we faced a diagnostic dilemma till lung biopsy was performed.

Bronchiolitis obliterans in children: A ghostly journey to the origin

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The clinical characteristics and non-steroidal treatment for radiation-induced bronchiolitis obliterans organizing pneumonia syndrome after breast-conserving therapy.

PURPOSE: A rare and unique occurrence of radiation-induced pulmonary injury was observed outside the tangential field for early breast cancer treatment. The findings appeared to be idiopathic and were termed radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome. The goal of this study was to report and determine the incidence, analyze the characteristics of the pulmonary lesions on the images and also investigate the treatment methods. MATERIALS AND METHODS: A retrospective analysis was conducted of 616 consecutive patients that underwent breast-conserving therapy (BCT) from January 1992 to December 2008. The patients were observed at least one year after radiotherapy for BCT. Radiotherapy was administered by 4 MV photons in all patients. The patients underwent chest X-rays periodically. If the BOOP syndrome was found, chest computed tomography (CT) were conducted to identify the characteristics of the pulmonary lesion outside the radiation field. RESULTS: The incidence of the radiation-induced BOOP syndrome was 12 patients (1.9%). Six of them had fever and cough, 6 had no symptoms. The pulmonary lesions were classified into four patterns on chest CT. Progression of the pulmonary lesions observed on chest X-ray were classified into three patterns. BOOP syndrome appeared within 5.6 months after radiotherapy and completely disappeared within 12 months after its onset. Their clinical conditions were not severe and these pulmonary lesions disappeared gradually without use of steroids in our institution. There was no death caused by BOOP syndrome. CONCLUSIONS: Although the incidence of BOOP syndrome and its associated prognosis are not significant, this clinical condition must be carefully followed using diagnositic imaging in order to not over administer steroids.

Bronchiolitis obliterans organizing pneumonia.

Bronchiolitis obliterans organizing pneumonia (BOOP) is a diffuse infiltrative pulmonary disorder that has an insidious onset. The patient often presents with flu-like symptoms and radiological findings showing bilateral patchy infiltrates. Histological features show fibromyxoid connective tissue plugs that are evenly spaced in small distal airways and alveolar spaces. Treatment for BOOP is corticosteroid therapy. Critical care nurses must be cognizant of BOOP as a differential diagnosis for patients for whom antibiotic therapy has not resolved symptoms.