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- Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. [Review]
- RReumatologia 2018; 56(4):249-254
- Interstitial lung disease (ILD) is a group of lung diseases characterized by thickening of the interstitium surrounding pulmonary alveolar walls. It is related to specific radiographic features in lu...
Interstitial lung disease (ILD) is a group of lung diseases characterized by thickening of the interstitium surrounding pulmonary alveolar walls. It is related to specific radiographic features in lung imaging and/or the presence of restrictive disorders in pulmonary function tests (PFTs). ILD is one of the leading causes of death in systemic sclerosis patients. Major risk factors of ILD associated with SSc (SSc-ILD) include male sex, diffuse type of cutaneous SSc and presence of anti-Scl-70 antibodies. SSc-ILD is challenging to diagnose at an early stage as the symptoms are non-specific. The greatest risk of its development is during the 4-5 years after the initial diagnosis of systemic sclerosis. Clinical vigilance at the time, including regular pulmonary function tests and/or high-resolution com-puted tomography (HRCT), is needed. The aim of this paper is to summarize the current knowledge on early diagnostic methods and progression risk factors for SSc-ILD.
- Chronic Lung Disease and Mortality after Cardiac Surgery: A Prospective Cohort Study. [Journal Article]
- JCJ Cardiothorac Vasc Anesth 2018; 32(5):2241-2245
- CONCLUSIONS: Combination of confirmed preexisting lung disease and newly diagnosed cases provides a clear link to mid-term mortality.
- Recovery of Pulmonary Function after Allogeneic Hematopoietic Cell Transplantation in Children is Associated with Improved Survival. [Journal Article]
- BBBiol Blood Marrow Transplant 2017; 23(12):2102-2109
- Abnormal pulmonary function is prevalent in survivors of allogeneic hematopoietic cell transplantation (HCT). Post-transplantation recovery of pulmonary function, and its effect on survival, in child...
Abnormal pulmonary function is prevalent in survivors of allogeneic hematopoietic cell transplantation (HCT). Post-transplantation recovery of pulmonary function, and its effect on survival, in children are not known. This retrospective cohort study of 308 children followed for 10 years after HCT at a single institution included 2 groups of patients. Group 1 comprised 188 patients with 3 or more pulmonary function test (PFT) results, of which at least 1 was abnormal, and group 2 comprised 120 patients with 3 or more PFTs, all of which were normal. Pulmonary function normalized post-transplantation in 51 patients (27%) in group 1. Obstructive lung disease, restrictive lung disease, mixed lung disease, and normal pattern were seen in 43%, 25%, 5%, and 27% of patients, respectively, at a median of 5 years (range, 0.5 to 11.9 years) post-transplantation. Lung volumes recovered better than spirometric indices. Pulmonary complications were seen in 80 patients (43%) in group 1. Patients who recovered pulmonary function had better overall survival (P = .006), which did not differ significantly from that in patients in group 2 with normal lung function post-transplantation (P = .80). After adjusting for duration of follow-up, pulmonary complications (P = .01), and lower pretransplantation forced vital capacity z-scores (P = .01) were associated with poor recovery. T cell depletion (P < .001), lower pretransplantation forced expired volume in 1 second z-scores (P = .006), and chronic graft-versus-host disease (P < .001) increased the risk for pulmonary complications. Nonrecovery of lung function with pulmonary complications (P = .03), acute graft-versus-host disease (P = .004), and mechanical ventilation (P < .001) were risk factors for nonrelapse mortality. Normalization of pulmonary function is possible in long-term survivors of allogeneic HCT. Strategies to decrease the risk of pulmonary complications may improve outcomes.
- Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience. [Journal Article]
- LLupus 2018; 27(3):365-371
- Introduction Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE), characterized by decreased lung volumes and extra-pulmonary restriction. The aim of this stud...
Introduction Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE), characterized by decreased lung volumes and extra-pulmonary restriction. The aim of this study was to describe the characteristics of SLS in our lupus cohort with emphasis on prevalence, presentation, treatment and outcomes. Patients and methods Patients attending the Toronto Lupus Clinic since 1980 ( n = 1439) and who had pulmonary function tests (PFTs) performed during follow-up were enrolled ( n = 278). PFT records were reviewed to characterize the pattern of pulmonary disease. SLS definition was based on a restrictive ventilatory defect with normal or slightly reduced corrected diffusing lung capacity for carbon monoxide (DLCO) in the presence of suggestive clinical (dyspnea, chest pain) and radiological (elevated diaphragm) manifestations. Data on clinical symptoms, functional abnormalities, imaging, treatment and outcomes were extracted in a dedicated data retrieval form. Results Twenty-two patients (20 females) were identified with SLS for a prevalence of 1.53%. Their mean age was 29.5 ± 13.3 years at SLE and 35.7 ± 14.6 years at SLS diagnosis. Main clinical manifestations included dyspnea (21/22, 95.5%) and pleuritic chest pain (20/22, 90.9%). PFTs were available in 20 patients; 16 (80%) had decreased maximal inspiratory (MIP) and/or expiratory pressure (MEP). Elevated hemidiaphragm was demonstrated in 12 patients (60%). Treatment with prednisone and/or immunosuppressives led to clinical improvement in 19/20 cases (95%), while spirometrical improvement was observed in 14/16 patients and was mostly partial. Conclusions SLS prevalence in SLE was 1.53%. Treatment with glucocorticosteroids and immunosuppressives was generally effective. However, a chronic restrictive ventilatory defect usually persisted.
- The "Complex Restrictive" Pulmonary Function Pattern: Clinical and Radiologic Analysis of a Common but Previously Undescribed Restrictive Pattern. [Multicenter Study]
- ChestChest 2017; 152(6):1258-1265
- CONCLUSIONS: CR is a common PFT pattern with distinct clinical features. The associated clinical entities share impaired lung emptying (eg, neuromuscular disease, occult obstruction, chest wall limitation). Clinicians should be aware of this novel PFT pattern and how it shapes the differential diagnosis.
- Pulmonary Function Tests for the Radiologist. [Review]
- RRadiographics 2017 Jul-Aug; 37(4):1037-1058
- Pulmonary function tests (PFTs) provide important quantitative information about lung function and can be used to elucidate pathologic conditions responsible for respiratory symptoms, assess the seve...
Pulmonary function tests (PFTs) provide important quantitative information about lung function and can be used to elucidate pathologic conditions responsible for respiratory symptoms, assess the severity and course of disease, and evaluate the patient for suitability and timing for lung transplantation. They are typically used in tandem with chest imaging, along with other ancillary data, to arrive at a specific diagnosis. PFTs may provide the radiologist with clues to the diagnosis and grading of a wide variety of pulmonary diseases. In this review, the authors discuss the clinical use of PFTs, their major components, and important measurements and graphical representations that are essential for understanding and interpreting the results. The key components of PFT panels-static lung volumes, dynamic lung function (spirometry), and diffusion capacity-are explained. The authors present a general algorithmic approach for problem solving, with recognition of common patterns of results (obstructive, restrictive, mixed, nonspecific, and normal). Pulmonary diseases from each of the major patterns and chest imaging are illustrated, and correlations between particular PFT results and disease severity and morphology at imaging are examined. Common pitfalls encountered during interpretation are also highlighted. A basic understanding of the mechanics of PFTs, characteristic patterns in important diseases, and correlation between lung function and imaging findings may assist the radiologist in diagnosis and follow-up of key pulmonary diseases and strengthen the radiologist's role as part of a multidisciplinary diagnostic team. Online supplemental material is available for this article. ©RSNA, 2017.
- Pulmonary function tests findings and their diagnostic value in patients with IgG4-related disease. [Journal Article]
- JTJ Thorac Dis 2017; 9(3):547-554
- CONCLUSIONS: DLCO/VA plays an important role for detecting lung involvement in IgG4-RD patients. The patient with high serum IgG may be more prone to respiratory involvement.
- Transbronchial biopsies safely diagnose amyloid lung disease. [Journal Article]
- AAmyloid 2017; 24(1):37-41
- CONCLUSIONS: Our case series of 25 patients supports the use of bronchoscopic transbronchial biopsies for diagnosis of parenchymal lung amyloidosis. Normal PFTs do not rule out the histologic presence of amyloid lung disease.
- Unabated occupational risk in a patient with rheumatoid pulmonary fibrosis. [Case Reports]
- OMOccup Med (Lond) 2017 Jun 01; 67(4):311-313
- CONCLUSIONS: Findings were consistent with a diagnosis of HP with RA-associated ILD. The patient's history of severe RA biased the diagnosis to one of RA-associated ILD and her occupational risk had been less emphatically addressed. Obtaining a thorough occupational history can uncover exposures to workplace respiratory hazards and may create opportunities for intervention to limit morbidity from chronic lung disease.
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- Long-term pulmonary complications in perinatally HIV-infected youth. [Multicenter Study]
- JAJ Allergy Clin Immunol 2017; 140(4):1101-1111.e7
- CONCLUSIONS: Compared with HEU youth, HIV-infected youth demonstrated decreased reversibility of obstructive lung disease, which is atypical of asthma. This might indicate an early stage of chronic obstructive pulmonary disease. Follow-up into adulthood is warranted to further define their pulmonary outcomes.