Diagnosis Of Interstitial Pneumonia Research Articles

An approach to the diagnosis of interstitial pneumonias in adults and children

An approach to the diagnosis of interstitial pneumonias in adults and children

An approach to the diagnosis of interstitial pneumonias in adults and children

In adults with an idiopathic interstitial pneumonia (IIP), an updated classification system was published in 2013. Although definitions for the seven histological patterns remain essentially unchanged, there has been substantial progress in our understanding. In relation to usual interstitial pneumonia (UIP), updated management recommendations were published in 2011, with the most significant change recommending varying degrees of confidence in diagnosis. A pattern of UIP is also being increasingly recognised in patients with chronic hypersensitivity pneumonitis. Idiopathic non-specific interstitial pneumonia is now better defined as a specific clinico-pathological entity, with the majority cases proving to have secondary associations/causes after multidisciplinary review. Respiratory bronchiolitis–interstitial lung disease is now commonly diagnosed without surgical biopsy, and acute exacerbation of IIPs is now well defined. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histological patterns, is also introduced. There have also been advances in the undertaking of biopsies with most institutions sampling at least two sites, ideally using preoperative targeting. These histological patterns are also recognised in children, although classification is more complex and includes entities specific to a younger age group (e.g., neuroendocrine hyperplasia of infancy, pulmonary interstitial glycogenosis). Surfactant protein gene mutations are also being increasingly identified. In adults with an idiopathic interstitial pneumonia (IIP), an updated classification system was published in 2013. Although definitions for the seven histological patterns remain essentially unchanged, there has been substantial progress in our understanding. In relation to usual interstitial pneumonia (UIP), updated management recommendations were published in 2011, with the most significant change recommending varying degrees of confidence in diagnosis. A pattern of UIP is also being increasingly recognised in patients with chronic hypersensitivity pneumonitis. Idiopathic non-specific interstitial pneumonia is now better defined as a specific clinico-pathological entity, with the majority cases proving to have secondary associations/causes after multidisciplinary review. Respiratory bronchiolitis–interstitial lung disease is now commonly diagnosed without surgical biopsy, and acute exacerbation of IIPs is now well defined. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histological patterns, is also introduced. There have also been advances in the undertaking of biopsies with most institutions sampling at least two sites, ideally using preoperative targeting. These histological patterns are also recognised in children, although classification is more complex and includes entities specific to a younger age group (e.g., neuroendocrine hyperplasia of infancy, pulmonary interstitial glycogenosis). Surfactant protein gene mutations are also being increasingly identified.

A case of fatal interstitial pneumonia during treatment of radiotherapy plus cetuximab for patient with head and neck carcinoma

We report a case of fatal interstitial pneumonia in a patient treated with radiotherapy plus cetuximab for head and neck carcinoma. The patient was an 86-year-old man with laryngeal squamous cell carcinoma (T2N0M0). Cetuximab was given in combination with radiotherapy; however, the patient developed dyspnea on day 30 and computed tomography findings confirmed a diagnosis of interstitial pneumonia. Noninvasive positive pressure ventilation was immediately started in conjunction with steroid pulse and endotoxin adsorption therapy. This led to temporary relief of symptoms. However, a subsequent relapse was resistant to steroid pulse therapy, and the patient died from interstitial pneumonia on day 47.

Reproducibility of the ATS/ERS classification of idiopathic interstitial pneumonias

Histopathological diagnosis of interstitial pneumonia (IP) requires experiences and is often challenging for general surgical pathologists. In its context, accurate recognition of histopathological usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP) patterns along with separation of idiopathic IPs from ones associated with hypersensitivity pneumonia and systemic connective tissue disease are the critical points. Several reports had indicated insufficient interobserver agreement of IP diagnosis, and we also have investigated the interobserver agreement of chronic interstitial pneumonia in various conditions showing that reproducibility of pathological judgment of above points was not greatest. To improve the reproducibility of diagnosis, recent publication of IPF guidelines set criteria of pathological UIP. It indeed helps pathologists to evaluate definite UIP pattern and works well for pulmonary pathologists, however, it still have some confusions and difficulties to the general surgical pathologists. In the course, summary of interobserver agreement along with our own data among pulmonary and general pathologists before and after the new guidelines will be shown. I will also share possible causes of confusion with audience and suggest a few tips for the improved histological assessment.

Beyond Belief

Beyond Belief

Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection?

To the Editor: Idiopathic pleuroparenchymal fibroelastosis is a peculiar pulmonary fibrosis proposed by Frankel et al . [1] in 2003 and is almost the same concept as idiopathic pulmonary upper lobe fibrosis proposed by Amitani et al. [2]. There are no known causes for fibrosis in idiopathic pleuroparenchymal fibroelastosis. Sometimes, pleuroparenchymal fibroelastosis (PPFE) has underlying diseases or conditions, such as collagen vascular diseases, anti-cancer chemotherapy, irradiation, asbestos exposure and bone-marrow transplantation [3]. Herein, we report the case of a female who received living-donor lung transplantation and died of pulmonary fibrosis, which was pathologically compatible with PPFE in addition to constrictive bronchiolitis, which is a manifestation of chronic lung allograft dysfunction (CLAD) [4]. A 30-yr-old female suffering from idiopathic pulmonary arterial hypertension underwent living-donor lung transplantation surgery and received a right lower lobe from her younger sister and a left lower lobe from her mother in December 2003. 20 months after the lung transplantation she had dyspnoea and a chest radiograph disclosed bilateral ground-glass shadows. 1 month later, right open lung biopsy was performed and a diagnosis of interstitial pneumonia was obtained. Pulse therapy with methylprednisolone slightly improved her condition and prednisolone was administered after the pulse therapy. However, bilateral interstitial opacities gradually deteriorated (fig. 1) with increased dyspnoea. 49 months after the lung transplantation, her daily life had worsened to almost whole-day bed rest. 18 days prior to her death she …

Imaging Diagnosis of Interstitial Pneumonia with Emphysema (Combined Pulmonary Fibrosis and Emphysema)

Based on clinical and radiological findings, Cottin defined combined pulmonary fibrosis and emphysema (CPFE) as pulmonary emphysema in the upper lungs and interstitial pneumonia in the lower lungs with various radiological patterns. Pathologic findings of CPFE probably corresponded with diffuse interstitial pneumonia with pulmonary emphysema, emphysema with fibrosis, and the combination of both. We described reported radiological findings of CPFE.

Pneumonia intersticial associada à esclerose sistêmica: avaliação da função pulmonar no período de cinco anos

To evaluate alterations in pulmonary function in patients with systemic sclerosis-associated interstitial pneumonia over a five-year period. This was a longitudinal study involving 35 nonsmoking patients with systemic sclerosis and without a history of lung disease. At the first evaluation, performed at the time of the diagnosis of interstitial pneumonia, the patients were submitted to HRCT, spirometry, and measurement of DLCO. The patients were subdivided into two groups by the presence or absence of honeycombing on the HRCT scans. Approximately five years after the first evaluation, the patients were submitted to spirometry and measurement of DLCO only. Of the 35 patients, 34 were women. The mean age was 47.6 years. The mean time between the two evaluations was 60.9 months. Honeycombing was detected on the HRCT scans in 17 patients. In the sample as a whole, five years after the diagnosis, FVC, FEV₁ and DLCO significantly decreased (81.3 ± 18.2% vs. 72.1 ± 22.2%; 79.9 ± 17.8% vs. 72.5 ± 20.6%; and 74.0 ± 20.5% vs. 60.7 ± 26.8%, respectively; p = 0.0001 for all), and the FEV₁/FVC ratio significantly increased (98.5 ± 7.2% vs. 101.9 ± 7.8%; p = 0.008). In the same period, FVC, FEV₁, and DLCO values were significantly lower in the patients with honeycombing on the HRCT scans than in those without (p = 0.0001). In systemic sclerosis-associated interstitial lung disease, the detection of honeycombing on HRCT is crucial to predicting accelerated worsening of pulmonary function.

Acute Exacerbation of Idiopathic Interstitial Pneumonia Following Lung Surgery in 3 of 68 Consecutive Patients: A Retrospective Study

Acute exacerbation (AE) of idiopathic interstitial pneumonia (IIP) is occasionally observed after lung surgery. However, the risk of lung surgery in patients with IIPs is not yet clearly known. We conducted a retrospective study of consecutive patients who underwent lung surgery for cancer or for the diagnosis of interstitial pneumonia (IP) between 2000 and 2006. Patients who developed AE following the lung operation were assessed. The data of 68 consecutive patients (males: 56, females: 12) with IP who underwent lung surgery were analyzed. The lung surgery included lobectomy for lung cancer in 48 patients [idiopathic pulmonary fibrosis (IPF) 31, non-IPF 17], and lung biopsy in 20 patients [IPF 8, non-specific interstitial pneumonia (NSIP) 8, unclassified 4]. Three patients with IPF (4.4% in total) developed AE after the operation (2 lobectomy, 1 biopsy). The triggers of AE were considered to be prolonged ventilation at a large tidal volume with oxygen supplementation at a high concentration. At the time of the AE, the extent of parenchymal involvement on the HRCT images was greater on the non-operated side. All three patients died of respiratory failure 12 to 82 days after the onset of AE despite corticosteroid therapy. It is essential to be aware of the risk of AE of IPF following lung operation. Intraoperative respiratory management, such as oxygen supplementation at a high concentration and/or prolonged mechanical ventilation are likely possible etiologic factors.

Surgical Lung Biopsies in 418 Patients with Suspected Interstitial Lung Disease in China

The present study was aimed to evaluate the role and safety of surgical lung biopsies (SLB) in patients with interstitial lung disease (ILD) in China. A retrospective analysis of 418 patients with ILD undergoing SLB from Chinese literature and the data of our hospital during the past ten years was performed. A total of 418 cases underwent SLB, including open lung biopsy (OLB) in 229 cases and video-assisted thoracoscopic lung biopsy (VATLB) in 189 cases. SLB yielded a specific diagnosis for 88.0% cases, and unclassifiable idiopathic interstitial pneumonia diagnosis was seen in 9.1% cases. The total postoperative complication rate was 12.0% and mortality rate was 1.9%. The diagnostic yield, post-operative complication rate, and mortality rate between VATLB and OLB had no significant difference. SLB is a very useful and relatively safe procedure for diagnosis of ILD.

Impact On Survival and Treatment of Late-Onset Noninfectious Pulmonary Complications After Allogeneic Hematopoietic Stem Cell Transplantation.

Abstract 3318Poster Board III-206 IntroductionLate-onset noninfectious pulmonary complications (LONIPCs) are life-threatening complications for allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients. However, the impact of LONIPCs on survival has not been properly evaluated and little is known about treatment efficacy for LONIPCs. Patients and methodsWe retrospectively studied 290 consecutive adult patients (age 16 to 67 years old, median 40 years old) with hematological disorders who underwent allo-HSCT at the University of Tokyo Hospital, Japan, between June 1995 to December 2007, and survived at least 60 days after allo-HSCT. The diagnosis of LONIPCs was made when a patient fulfilled all of the following criteria: 1) respiratory symptoms developed after 60 days of allo-HSCT, 2) no evidence of infection was detected despite intensive examinations, 3) at least one of the following abnormalities are observed: a) abnormal interstitial shadows on chest computed tomography (CT), b) pathological diagnosis of interstitial pneumonia or bronchiolitis obliterans (BO), c) pulmonary function tests (PFTs) revealed obstructive or mixed pattern and FEV1 declined to less than 80 % of the predicted value, or FEV1 declined at least 20 % from baseline. According to these criteria, 44 patients were diagnosed as having LONIPCs. Then we classified them into BO and non-BO subgroups based on PFTs and CT. We compared the clinical aspects and outcome between patients who developed and did not develop LONIPCs. Time-dependent analysis was employed to conduct survival analysis. Furthermore, we analyzed the effect of treatment on outcomes. ResultsOnset of LONIPCs was significantly associated with higher rates of chronic graft-versus-host disease (p<.001) and higher rates of non-relapse mortality (p=.013), and lower rates of relapses (P=0.009). As a result of these effects, overall survival (OS) was significantly worse in those with LONIPCs (p=.003) than those without as shown in Figure 1. Next, we assessed short-term treatment responses and final outcomes. These results were defined by radiological findings, subjective symptoms, oxygen requirement, and survival. Use of inhaled and systemic steroids did not affect either short-term response or final outcomes. However, administration of systemic corticosteroids earlier than 21 days (median interval of time from onset of symptoms to systemic corticosteroids administration) was associated with better outcomes (P=0.054 for short-term response, and P=0.016 for final outcomes). This tendency was also observed when we analyzed BO subgroup's final outcome (P=0.041). As for PFTs, FEV1/FEV1-predicted values before HSCT (A; n=40), between HSCT and onset of respiratory symptoms (B; n=22), at the diagnosis of LONIPCs (C; n=33), during the first 2 months of treatment (D; n=18) and at last follow up (E; n=30) were shown in Figure 2. Patients who received systemic corticosteroids before 21 days from onset of symptoms (early steroid) had significantly less deterioration of FEV1 at last follow up than those who received after 22 days from onset (late steroid) and patients who were not administered (steroid-) (*P=0.033, **P=0.039). ConclusionOur study indicates that LONIPCs negatively affects OS, and early intervention with systemic corticosteroids might be effective in terms of OS and reservation of PFT. [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Progressive renal insufficiency associated with amiodarone-induced phospholipidosis

Progressive renal insufficiency associated with amiodarone-induced phospholipidosis

Interstitial lung disease

Idiopathic interstitial pneumonias represent an important group of interstitial lung diseases, encompassing seven entities: (1) usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis; (2) non-specific interstitial pneumonia (NSIP); (3) organizing pneumonia/cryptogenic organizing pneumonia (COP); (4) diffuse alveolar damage (DAD)/acute interstitial pneumonia (AIP); (5) respiratory bronchiolitis (RB)/respiratory bronchiolitis–interstitial lung disease (RB–ILD); (6) desquamative interstitial pneumonia (DIP); and (7) lymphocytic interstitial pneumonia (LIP). The term idiopathic means that the cause is not certain at the time of diagnosis, but each of these patterns of interstitial pneumonia can occur in the setting of known causes as well. This review will highlight the pathological features and differential diagnosis of interstitial pneumonias, along with the high-resolution computed tomography (HRCT) scan and clinical features, emphasizing the importance of clinical, radiological and pathological correlation to establish the correct diagnosis. Familiarity with the histological patterns of lung injury in these disorders will aid pathologists in recognizing the correct diagnosis. It is also important for pathologists and clinicians to understand the settings in which HRCT can overrule pathological findings. In particular, if an HRCT scan shows classical features of UIP, despite surgical biopsy findings of NSIP, the clinical–radiological–pathological diagnosis would be UIP.

Acute exacerbation of interstitial pneumonia following surgical lung biopsy

Surgical lung biopsy (SLB) plays an important role in the diagnosis of interstitial pneumonia, however, the occurrence of acute respiratory failure following SLB remains largely unreported. We evaluated the incidence, clinical features, therapy and prognosis of acute exacerbation of interstitial pneumonia following SLB. Retrospective study of consecutive patients who underwent SLB to establish a diagnosis of diffuse lung disease between May 1989 and April 2000. Patients with an acute exacerbation following lung biopsy were studied, and the HRCT images of the chest before and after surgery were reviewed. Among the 236 consecutive patients with interstitial pneumonia who underwent a surgical lung biopsy, five (2.1%) (IPF, 3; NSIP, 1; COP, 1) developed acute exacerbation of the diffuse lung disease in the course of 1-18 days after SLB. The extent of parenchymal involvement on HRCT before surgery was not significantly different between operated and contralateral nonoperated lung. Significantly increased regions of parenchymal involvement on HRCT were seen postoperatively compared with the preoperative CT in both the operated (20.7+/-12.5% versus 38.2+/-10.8%, P = 0.0431) and nonoperated lung (22.7+/-13.8% versus 70.5+/-24.4%, P = 0.0431), but the extent of the parenchymal involvement was significantly greater on the nonoperated side (P = 0.0251). Two of the 3 IPF patients died from the acute exacerbation. It is important to be aware of the possibility of acute exacerbation of interstitial pneumonia following SLB even after an apparently uneventful immediate postoperative course. The asymmetric image findings suggest that intraoperative respiratory management is a possible etiologic factor.

A case of interstitial pneumonia caused by bucillamine: a study using serological markers

The patient was a 61-year-old man diagnosed with rheumatoid arthritis (RA) in 2001. He initially received treatment at a nearby clinic, but his condition could not be satisfactorily controlled. He subsequently consulted our hospital during the same year. Although his symptoms improved in response to treatment at our hospital, slight fever, cough, and then high fever and dyspnea subsequently developed. A diagnosis of interstitial pneumonia was made on the basis of findings of diagnostic imaging. The time course of changes in serological markers, including surfactant protein A (SP-A), surfactant protein D (SP-D), and KL-6, as well as markers of inflammatory reaction and lactate dehydrogenase was examined to determine the clinical significance of serological markers in the management of interstitial pneumonia.

Diagnosis of interstitial pneumonia

Diagnosis of interstitial pneumonia

A Case of Interstitial Pneumonia Induced by Rituximab Therapy

Rituximab, a chimeric human/murine monoclonal antibody directed against CD20, is an effective therapy for B-cell type non-Hodgkin’s lymphoma. Most of the adverse responses are mild to moderate nonhematological toxicities, which include fever, chill, or skin reactions. Severe respiratory adverse events have been infrequent [1,2]. Recently, Burton et al reported 2 interstitial pneumonia (IP) cases related to rituximab therapy [3]. IP is rarely induced by rituximab, and its etiological roles are still unclear. Here, we report an IP case related to rituximab therapy, for which we investigated its etiology as well as possible. An 80-year-old man with a diagnosis of diffuse large Bcell lymphoma was treated with rituximab and cyclophosphamide, vincristine, and prednisolone chemotherapy (RCOP). No severe adverse effects were observed during the first course, and after 3 weeks, he was given a second course. With the second rituximab infusion, he had a grade 2 fever and a systemic skin rash. The infusion reaction was abated with hydroxycortisone, and COP therapy was scheduled. Ten days after the second rituximab infusion, the patient complained of general fatigue and had a high fever.A chest x-ray revealed bilateral ground-glass opacities, and a high-resolution computed tomography scan of the chest confirmed the diagnosis of interstitial pneumonia. As respiratory insufficiency advanced, he needed noninvasive positive-pressure ventilation. He was treated with methylprednisolone pulse therapy after a bronchoalveolar lavage (BAL) examination. He made a dramatic recovery, corticosteroid was tapered, and he was discharged after 3 weeks. No pathogens were detected in the patient’s blood, sputum, or BAL fluids, and results for Pneumocystis carini and Mycobacterium tuberculosis were also negative. Serum antibodies did not indicate the presence of viruses, chlamydia A Case of Interstitial Pneumonia Induced by Rituximab Therapy

Pulmonary pathology in association with connective tissue disorders

Patients with connective tissue disorders (CTD) often have associated pleuropulmonary lesions, and these are major causes of morbidity and mortality. CTD may involve all compartments of the respiratory system, including respiratory muscles, pleura and lung parenchyma, with diffuse interstitial pneumonia and fibrosis being common within the parenchyma. During the last decade, non-specific interstitial pneumonia (NSIP) has been recognized as a specific histological pattern, and the definition of usual interstitial pneumonia (UIP) has been narrowed and refined, this having significant impact on the diagnosis of interstitial pneumonias in CTD. Recent data suggest that a pattern of NSIP is the most common pattern of interstitial pneumonia in CTD such as scleroderma, dermatomyositis/polymyositis, rheumatoid arthritis, and Sjoegren's syndrome. This is consistent with the more favourable prognosis seen with interstitial pneumonias associated with CTD, in comparison to patients with idiopathic pulmonary fibrosis who, with rare exceptions, have a histological pattern of UIP.

Significance of Q fever serologic diagnosis in clinically suspect patients.

Q fever is caused by C. burnetii, an intracellular obligate bacterium. For clinical confirmation of Q fever, diagnosis of interstitial pneumonia is of significance. The acute disease varies in severity from minor to fatal, with the possibility of serious complications. Chronic endocarditis is a well-known outcome. Symptoms of Q fever can vary; fixing diagnosis is done by serology with the phase I and the phase II antibody. We tested 44 sera of 31 clinically suspect patients. From these, 22 patients were taken to the infection clinic, 8 to the pulmonary clinic, and one to the general hospital. From the 31 patients, 21 patients had one serum, 7 patients, 2 sera, and 3 patients, 3 sera. Blood samples were collected by vein puncture, and serum samples were kept at -20 degrees C until testing. All sera were processed by indirect immunofluorescent assay (IFA) Q fever IgM and IgG. Of 44 processed sera, 21 were seropositive. Specific IgM antibody was found in sera of 6 patients (19.4%), and specific IgG antibody in sera of 16 patients (51.2%). In sera of 15 clinically suspect patients (48.3%), no specific anticoxiella antibody was found. From these results we can confirm the importance of serology in laboratory diagnosis and clinical affirmation of suspect Q fever. Indirect immunofluorescent assay (IFA) is reliable and appropriate for daily, routine diagnosis of human Q fever.

Breast cancer shortly after the diagnosis of interstitial pneumonia associated with polymyositis.

Polymyositis is sometimes associated with pulmonary fibrosis or malignant diseases, however, rarely with both simultaneously. For such patients, no criterion for initial treatment and tapering of steroid has been reported. We describe a patient who had breast cancer and interstitial pneumonia associated with polymyositis. In this case, slow tapering of the steroid was an important aspect of the treatment, and close clinical follow-up was necessary to monitor for disease exacerbation.