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Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Any test that provides sufficient prognostic information to guide treatment decisions in idiopathic pulmonary fibrosis (IPF) is not available. The aim of our study was...
BACKGROUND AND AIM
Any test that provides sufficient prognostic information to guide treatment decisions in idiopathic pulmonary fibrosis (IPF) is not available. The aim of our study was to determine the predictive factors of mortality in patients with IPF treated with antifibrotics.
METHODS
Patients with diagnosis of IPF who were treated with antifibrotics between 2016 - 2021 were included in the study. Demographic, clinical and laboratory characteristics of the patients was derived from hospital records retrospectively. Kaplan Meier and multivariate cox regression analysis were achieved for detection of mortality predictors.
RESULTS
Study population was composed of 119 IPF patients with a male predominance of 80.7% (n=96). Mean age of the patients was 67.9 ± 7.07 years. On univariate analysis, sex was not a significant predictor of mortality (HR 1.79; 95% CI: 0.87 - 3.69, p =0.11). BMI ≤ 26,6 m2/kg, DLCO ≤ 3.11 ml/mmHg/min, age over 62 years, 6DWT ≤ 382 meters, NLR ≤ 2.67 and PDW ≤ 16.7% were found to be significant for predicting mortality. On multivariate cox regression analysis four parameters remained significant for prediction of mortality: RDW > 14%, NLR ≤ 2.67, BMI ≤ 26,6 m2/kg and DLCO ≤ 3.11 ml/mmHg/min (respectively, HR: 2.0. 95% CI: 1.02 - 3.91, p=0.44; HR: 2.68. 95% CI: 1.48 - 4.85, p=0.001, HR: 2.07. 95% CI: 1.14 - 3.76, p=0.02, HR: 3.46. 95% CI: 1.85 - 6.47, p<0.001). A scoring system with these parameters discriminated patients with worse prognosis with a sensitivity of 89.1 % and a specificity of 65.8 % when total point was over 2 (AUC0.83, p<0.001). Conclusions In this study, DLCO, BMI, RDW and NLR levels significantly predicted mortality in IPF patients. Along with GAP index, scoring system with these simple parameters may give information about the prognosis of an IPF patient treated with antifibrotics.
PubMed: 38940720
DOI: 10.36141/svdld.v41i2.13779 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial lung disease (ILD) characterized by subpleural parenchymal fibrosis and elastosis mainly in the...
BACKGROUND
Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial lung disease (ILD) characterized by subpleural parenchymal fibrosis and elastosis mainly in the upper lobes. PPFE occurs in a secondary form that overlaps with underlying medical conditions or complications. This study evaluated the clinical impact of coexisting factors on the survival of patients with PPFE.
METHODS
Fifty-five PPFE patients were retrospectively evaluated. The patients' diagnoses were categorized as "idiopathic PPFE" with no known cause or "secondary PPFE" with underlying medical conditions or complications. The clinical characteristics and survival rates of these groups were compared.
RESULTS
Twenty-eight patients (50.9%) were diagnosed with idiopathic PPFE and 27 (49.1%) with secondary PPFE, including cases of occupational dust exposure, connective tissue disease (CTD), post-hematopoietic stem cell transplantation (HSCT), and a family history of ILD. The idiopathic and secondary PPFE groups had similar clinical features, laboratory tests, and pulmonary function profiles, including a low body mass index, normal Krebs von den Lungen-6, high surfactant protein-D, and high residual volume/total lung capacity. In the secondary PPFE group, post-HSCT was associated with a worse prognosis, and CTD was associated with better prognosis. A multivariate analysis demonstrated that post-HSCT and a reduced forced vital capacity were significantly associated with a worsened survival in patients with PPFE.
CONCLUSIONS
The prognosis of PPFE is highly influenced by underlying medical conditions or complications. Patients with post-HSCT PPFE should be monitored particularly closely, as they are at higher risk of a poor prognosis than others.
PubMed: 38940719
DOI: 10.36141/svdld.v41i2.13845 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Thoracic involvement of Immunoglobulin G4-related disease (IgG4-RD) is relatively rare and may be disregarded at the time of initial diagnosis due to its asymptomatic...
BACKGROUND AND AIM
Thoracic involvement of Immunoglobulin G4-related disease (IgG4-RD) is relatively rare and may be disregarded at the time of initial diagnosis due to its asymptomatic nature. This study aimed to ascertain the prevalence and patterns of thoracic involvement in a retrospective cohort of Turkish patients with IgG4-RD.
METHODS
A total of 90 patients (47 males and 43 females, with a mean age of 57.7±15.5 years) diagnosed with IgG4-RD were reviewed retrospectively. All computed tomography (CT) scans were re-evaluated by two thoracic radiologists and IgG4-related thoracic disease was assessed on four compartments: The mediastinum, pulmonary parenchyma, airways, and pleura. IgG4-related thoracic disease was categorized as: definite, highly probable, probable or possible.
RESULTS
There were 64 patients who had undergone at least one thorax CT examination, and 18 (28%) were diagnosed with IgG4-related thoracic disease. The rate of IgG4-related thoracic disease increased by 20% and reached a ratio of 48.4% (n=31) after a thorough reevaluation of registry data specifically to thoracic findings. The mediastinum was the most frequently involved compartment, affecting 16 (51.6%) patients, followed by pulmonary parenchyma in 14 (45.2%) patients, and airways and pleura in 10 (32.3%) patients each. Other organ involvements were more prevalent and IgG4 levels were higher in patients with thoracic involvement. Eosinophils were significantly elevated in patients with thoracic involvement (p=0.023).
CONCLUSIONS
IgG4-related thoracic disease is heterogeneous and likely to be more prevalent than currently recognized. The mediastinum is the most frequently involved compartment. It is important to assess IgG4-related thoracic disease at the time of initial diagnosis. Elevated levels of serum IgG4 and eosinophils, as well as a greater number of organ involvements may serve as indicators of thoracic involvement.
PubMed: 38940718
DOI: 10.36141/svdld.v41i2.14165 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Endobronchial biopsy (EBBX) has been reported to increase diagnostic yield for pulmonary sarcoidosis. The purpose of this study is to investigate the diagnostic yield...
The role for endobronchial biopsy in the era of endobronchial ultrasound guided transbronchial needle aspiration for the diagnosis of sarcoidosis: a single center experience.
BACKGROUND AND AIM
Endobronchial biopsy (EBBX) has been reported to increase diagnostic yield for pulmonary sarcoidosis. The purpose of this study is to investigate the diagnostic yield for EBBX following endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA).
METHODS
We identified a cohort of patients in the University of Minnesota Sarcoidosis Registry who had EBBx and EBUS-TBNA as part of workup for abnormal chest imaging. Data regarding demographics, biopsy approach and technique were recorded.
RESULTS
Our cohort included 37 patients (53.24±9.5, Male, 22±0.57; 3.8% were African American). In these patients who had EBBX, EBUS-TBNA was performed in 100% of patients and TBBX was performed in 2 patients (5%). EBBX was positive in 9 patients (24%) and EBUS-TBNA was positive in 34 patients (92%). TBBX was diagnostic in one of two patients. EBBX was the only diagnostic tissue in 3 of the 37 patients (8%). Conclusion: The diagnostic yield of EBBX is lower than previously reported, with only 8% of EBBXs demonstrating granulomatous inflammation. However, instrumentation used for obtaining EBBX as well as the presence of visible lesions does influence the diagnostic yield. Studies with adequate power are needed before implementing changes in clinical practice. When performed alongside EBUS-TBNA, EBBX did not significantly add to the diagnostic yield in sarcoidosis unless visible lesions were observed.
PubMed: 38940717
DOI: 10.36141/svdld.v41i2.14192 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Cardiac sarcoidosis is associated with significant morbidity and mortality. Immunosuppressive treatment focuses on suppressing myocardial inflammation, which can lead to...
Cardiac sarcoidosis is associated with significant morbidity and mortality. Immunosuppressive treatment focuses on suppressing myocardial inflammation, which can lead to major adverse events especially when progressing to fibrosis. Conventional management usually includes steroids and steroid sparing agents such as methotrexate and azathioprine. Tumour necrosis factor alpha inhibitors are often reserved for those with a worsening clinical status and/or evidence of persistent inflammatory activity despite conventional therapy. Refractory cardiac sarcoidosis (CS) can be defined as the persistence or progression of active disease, evidenced either by lack of clinical response or persistence or progression of imaging abnormalities, despite being on conventional therapy. In the United Kingdom, tumour necrosis factor alpha inhibitors are currently not licensed for cardiac sarcoidosis as there are no randomised controlled trials to assess the efficacy of infliximab in this patient cohort. In this study, we present the outcomes of six patients treated with infliximab for refractory cardiac sarcoidosis at Royal Brompton Hospital and performed a systematic review of the existing literature on use of infliximab in cardiac sarcoidosis. We searched the Cochrane Library, OVID Medline, OVID Embase, Web of Science and Pubmed to identify 7 full-text studies assessing the role of infliximab in the management of cardiac sarcoidosis. Infliximab was found to play a vital role in stabilising refractory cardiac sarcoidosis by stemming clinical deterioration, arrythmia burden and even reducing steroids requirements. Further prospective trial data is necessary to validate these findings.
PubMed: 38940716
DOI: 10.36141/svdld.v41i2.14484 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Adequate respiratory muscle strength is required to meet the increased ventilatory demand during physical activities. However, it is not well known whether respiratory...
BACKGROUND
Adequate respiratory muscle strength is required to meet the increased ventilatory demand during physical activities. However, it is not well known whether respiratory muscle strength is impaired in patients with idiopathic pulmonary fibrosis (IPF).
OBJECTIVES
This study aimed to investigate the relationship between respiratory muscle strength and exercise capacity, quality of life, physical activity level, and fatigue in IPF patients.
METHODS
The study comprised 30 individuals with idiopathic pulmonary fibrosis (IPF) and 30 healthy controls. Maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) were measured to assess respiratory muscle strength. The International Physical Activity Questionnaire-Short Form, 6-minute walk test distance (6MWD), St George Respiratory Questionnaire (SGRQ), and Fatigue Severity Scale (FSS) were employed to evaluate physical activity level, exercise capacity, quality of life, and fatigue severity, respectively.
RESULTS
MIP (81±29 vs.73±20 cmH2O) and MEP (93±31 vs. 93±34 cmH2O) did not differ significantly between IPF patients and controls (p>0.05). In patients with IPF, MIP was significantly correlated with 6MWD (r=0.533), SGRQ (r=-0.428), and FSS (r=-0.433). Multivariate models including MIP, MEP, FEV1, FVC, and PA level explained 74% of the variance in the 6MWD (p<0.001), and MIP, FEV1, and PA level were independent predictors of the 6MWD, with FEV1 being the strongest predictor (β=0.659). Multivariate models predicting SGRQ revealed none of MIP, FEV1 or PA level was directly influencing the SGRQ score.
CONCLUSIONS
This study suggests that patients with IPF do not have respiratory muscle weakness. Inspiratory muscle strength has a direct influence on exercise capacity but an indirect effect on quality of life, probably by influencing exercise capacity.
PubMed: 38940715
DOI: 10.36141/svdld.v41i2.14884 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Increased calcitriol synthesis in sarcoid granulomas with subsequent hypercalcaemia and hypercalciuria can affect bone metabolism in patients with sarcoidosis. Multiple...
BACKGROUND AND AIM
Increased calcitriol synthesis in sarcoid granulomas with subsequent hypercalcaemia and hypercalciuria can affect bone metabolism in patients with sarcoidosis. Multiple factors can increase the fracture risk in patients with sarcoidosis. This study aimed to evaluate a 10-year osteoporotic and a 10-year hip fracture risk and to analyse factors affecting fracture risk for patients with newly diagnosed sarcoidosis compared to an age- and gender-matched control group from a real-world setting.
METHODS
The cross-sectional study included 171 patients with a histologically verified diagnosis of sarcoidosis who were hospitalised due to suspected sarcoidosis within two years and an age- and gender-matched control group of 178 hospitalised individuals. QFracture algorithm questions were asked during interviews.
RESULTS
A cohort of 349 subjects was analysed. The median age in the patient group was 40 years (IQR:20), and 60.2% were female. 21.6% of patients with sarcoidosis had at least one comorbidity that could potentially influence the osteoporotic fracture risk. Both the median 10-year osteoporotic fracture risk (0.9% (IQR:2) vs 1.3% (IQR:2.3), p=0.005; U=12394) and a 10-year hip fracture risk (0.1% (IQR:0.3) vs 0.2% (IQR:0.5), p=0.003; U=12368.5) was lower in patients with sarcoidosis compared to control group subjects. As compared to the control group, individuals with sarcoidosis exhibited a lower frequency of both osteoporotic (2.4% vs 11.2%, OR=0.189 (95% CI:0.063-0.566), p=0.003) and low-energy trauma fractures (2.9% vs 11.8%, OR=0.225 (95% CI:0.083-0.612), p=0.003) in personal medical history.
CONCLUSIONS
This was the first study to investigate osteoporotic fracture risk and related factors in Latvian patients with newly diagnosed sarcoidosis. Our data show a lower risk of a 10-year osteoporotic and a 10-year hip fracture risk in patients with sarcoidosis compared to age- and gender-matched control group subjects from a real-world setting.
PubMed: 38940714
DOI: 10.36141/svdld.v41i2.15156 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonias; its physical characteristics include a slender build with...
BACKGROUND AND AIM
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonias; its physical characteristics include a slender build with platythorax and progressive weight loss. However, the clinical significance of body mass index (BMI) and weight loss remains unclear in patients with IPPFE. Therefore, we aimed to clarify the association between baseline BMI, weight loss after diagnosis, and the prognosis of patients with IPPFE.
METHODS
This retrospective study included 71 patients diagnosed with IPPFE at our institution between 2005-2021. BMI at diagnosis was classified into three: underweight (<18.5 kg/m2), normal weight (≥18.5 to <25.0 kg/m2), or overweight (≥25.0 kg/m2). An annual rate of weight change after the diagnosis was evaluated, and ≥5% per year decrease was defined as a significant weight loss. We investigated clinical features and prognosis based on baseline BMI and weight loss.
RESULTS
Of the 71 patients, 48 (67.6%) and 23 (32.4%) were classified as underweight and normal weight, respectively, and none were overweight. Significant weight loss occurred in 24 (33.8%) patients, and they tended to have more cases of dyspnea and had significantly older age, lower BMI, higher rates of co-existence of lower-lobe interstitial lung disease, lower pulmonary function test results and higher incidence of pneumothorax after the diagnosis than those without weight loss. Patients with BMI <18.5 kg/m2 and those with weight loss had a significantly worse prognosis than those with BMI ≥18.5 kg/m2 or those without weight loss, respectively (p=0.005, p<0.001). Multivariate analysis revealed that low BMI and weight loss were independent poor prognostic factors.
CONCLUSIONS
Low BMI and weight loss are associated with poor prognosis in patients with IPPFE.
PubMed: 38940713
DOI: 10.36141/svdld.v41i2.15291 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Sarcoidosis is a systemic disease of unknown etiology with diverse clinical manifestations. Disease may resolve spontaneously or require immunosuppression to control...
BACKGROUND AND AIM
Sarcoidosis is a systemic disease of unknown etiology with diverse clinical manifestations. Disease may resolve spontaneously or require immunosuppression to control progression. Currently, there is no predictive model to direct treatment, and management is guided by symptoms and functional impairment. This study examines the association between biopsy features and prognosis.
METHODS
This is a retrospective population-based cohort study. New cases of biopsy-proven sarcoidosis were divided into two groups: those with diffuse thoracic lymph nodes (TLN) involvement, versus partial TLN involvement (Defined as Non-necrotizing granuloma (NNG) found in some but not all sampled TLN). We compared outcomes one year after diagnosis. We assessed the need for immunosuppression, the number of hospitalizations, and lung function deterioration.
RESULTS
77 cases were included in the final analysis. 48.1% demonstrated extensive TLN involvement, and 51.9% demonstrated partial or non-involvement of sampled TLN. The partial positive group had a more aggressive disease, reflected by a significantly higher need for steroid therapy in the first year after diagnosis (45.0% vs. 18.9% p=0.015). The number of hospitalizations and lung functions were not significantly different between groups.
CONCLUSIONS
Our findings demonstrate a significantly increased need for steroidal therapy among sarcoidosis patients with a partial positivity of TLN. These findings suggest that the degree of TLN involvement can help predict worse outcome and guide therapeutic decisions.
PubMed: 38940712
DOI: 10.36141/svdld.v41i2.15336 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease of unknown cause with a poor prognosis. The aim of our study is to determine the role of...
BACKGROUND AND AIM
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease of unknown cause with a poor prognosis. The aim of our study is to determine the role of Krebs von den Lungen-6(KL-6),Matrix metalloproteinase (MMP)-7, Surfactant protein A (SP-A), Surfactant protein D(SP-D), vascular endothelial growth factor (VEGF) and periostin in the diagnosis of IPF and in the response monitoring of patients treated.
METHOD
47 IPF patients, 27 non-IPF interstitial lung disease (ILD) patients and 21 healthy individuals were included in the study. Demographic data, pulmonary function test- Diffusing capacity of the lung for carbon monoxide (PFT-DLCO) measurements, High-resolution computed tomography (HRCT) findings of the patients were recorded, and serum samples were taken.
RESULTS
While periostin and SP-A levels were not significantly different between IPF and non-IPF ILD, they were significantly higher in both IPF and non-IPF ILD compared to healthy control group (p=0.002,p=0.006 for periostin and p=0.002,p<0.001 for SP-A, respectively).By receiver operating characteristic (ROC) analysis, the cut-off point for periostin to distinguish IPF is >594.5 pg/ml (sensitivity 72%, specificity 76%), while the cut-off point for SP-A is found >6.62 ng/ml (sensitivity 87.2%,specificity 57.1%). In the combined ROC analysis based on SP-A=6.62 ng/ml and periostin >634.6 pg/ml values, sensitivity was found to be 85% and specificity was 57%.Considering the correlation of forced expiratory volume in the first second (FEV1)(%), forced vital capacity (FVC)(%), restriction and diffusion severities with biomarker levels in the 6th month of IPF patients treated, a correlation was detected between MMP-7 levels and restriction severities (p=0.020), between KL-6 levels and restriction and diffusion severities (p=0.002), and between SP-A levels and FVC(%)(p=0.006).
CONCLUSION
It is thought that biomarkers SP-A and periostin may contribute significantly to the diagnosis of patients with IPF, and SP-A, MMP-7 and KL-6 levels may contribute significantly to treatment follow-up.
PubMed: 38940711
DOI: 10.36141/svdld.v41i2.15454