Sarcoidosis, Vasculitis, and Diffuse Lung Diseases最新文献

Background and objective: Data on natural killer (NK)- and natural killer T (NKT)- like cells in the immunopathogenesis of sarcoidosis remain limited. The aim was to assess NK- and NKT-like cells across different stages in bronchoalveolar lavage (BALF) versus peripheral blood (PB) in comparison to controls.

Methods: Forty four patients (32 women and 12 men, mean age 46.6±14.4 years) with biopsy-proven sarcoidosis and 10 healthy individuals (6 women, 4 men mean age 52.6±19.1 years) were submitted to BALF. Total cells and cell differentials were counted, while CD45+, CD3+, CD4+, CD8+, CD19+, CD3-CD16/56 (NK cells) and CD3+CD16/56+ (NKT-like cells) were determined by dual flow cytometry in BALF and PB.

Results: A significantly lower percentage of both NK and NKT-like cells was observed in BALF of controls and sarcoid patients (SP) compared to PB. Both BALF NK and NKT-cell counts were significantly higher in SP than in controls (NK: p=0.046, NKT-like: p=0.012) In addition BALF NK cell percentage differed among sarcoidosis stages (p=0.005). In PB NK-cell count was lower in sarcoidosis patients but the difference did not reach statistical significance. Also, in sarcoid patients' BALF NK-cell percentage negatively correlated with lymphocyte percentage (r=-0.962, p<0.001).

Conclusions: The increased count of BALF NK and NKT-like cells in sarcoidosis compared to controls along with the increase of NK cells with stage progression are in line with a growing number of investigations suggesting the involvement of NK- and NKT-like cells in the pathogenesis of sarcoidosis.

Aim: In this study, it was aimed to compare the levels of serum uric acid and uric acid/creatinine ratios in patients with COPD during an attack or in stable COPD, and to show whether serum uric acid and uric acid/creatinine ratios are associated with early mortality in COPD patients during an acute attack.

Materials and methods: In this study, COPD acute attack (n=155) and stable COPD (n=30) patients were evaluated. The data of these patients were obtained from patient files and computer records. COPD diagnosis and severity assessment were made according to the GOLD 2006 guideline. Participants' age, gender, body mass index, pulmonary function test, arterial blood gas, uric acid, creatinine values and comorbidity information were recorded in the previously prepared Case Data Form. In 2012, when we conducted this study, gold 2006 was taken as the guideline for spirometry measurement, but spirometric measurements determined with reference values determined according to age, height and gender, and FEV1/FVC measurement <70% as diagnostic criteria in acute attack after bronchodilator were the guidelines used later, gold 2017. It is also compatible with gold2020 and gold2021 spirometry criteria.

Results: It was determined that the uric acid (p<0.001) and uric acid/creatinine (p<0.001) levels of the patients in the acute attack group were significantly higher than the levels of the patients in the stable group. The attack group was divided into two subgroups according to certain cut-off points for uric acid (>6 mg/dl for women and >7 mg/dl for men) and uric acid/creatinine ratio (median value 7.10). Since the upper limit of the uric acid value measured in the blood is 6 mg/dl in women and 7-8 mg/dl in men, the cut-off points for uric acid (>6 mg/dl for women and >7 mg/dl for men) were determined in our study. According to this categorization, it was determined that there was no statistically significant relationship between uric acid level (odds ratio 2.985 [95% confidence interval 0.61814,151]) and early mortality risk.

Conclusion: The results of this study showed that the uric acid and uric acid/creatinine levels in the attack group were higher than the levels in the stable group, but these parameters were not associated with early mortality.

Although interstitial pneumonia is an important respiratory manifestation in microscopic polyangiitis (MPA), no studies have examined the detailed pathogenesis of interstitial pneumonia during the clinical course of MPA. In addition, it is considered that MPA develops at a certain incidence rate from myeloperoxidase (MPO)- antineutrophil cytoplasmic antibody (ANCA) positive interstitial pneumonia. However, there is a lack of consensus among pulmonologist and rheumatologist regarding whether MPO-ANCA positive interstitial pneumonia, which does not accompany other organ damage related to ANCA-associated vasculitis (AAV) other than interstitial pneumonia, should be included in AAV. In this review article, the clinical questions regarding MPO-ANCA positive interstitial pneumonia have been set, and evidence to date and problems to be solved in future are outlined.

Introduction: Echocardiographic measurement of the right ventricular systolic pressure (RVSP) is commonly used for estimating systolic pulmonary artery pressure (PASP) measured during right heart catheterization (RHC) in patients suspected for pulmonary hypertension (PH). Generally, there seems to be a strong correlation. However, this has been reported as less robust in sarcoidosis. We aim to investigate the correlation between RVSP and RHC measurements using real world data and analyzed factors influencing the relationship between RVSP and PASP in sarcoidosis.

Methods & results: Data of patients with and without sarcoidosis associated PH who had both a measurable echocardiographic RVSP and invasive PASP were collected from the RESAPH registry, PULSAR study and Cincinnati Sarcoidosis Clinic database (n=173, 60.1% female, mean age 56.0±9.5 years). Among them, 124 had PH confirmed by RHC. There was a strong correlation between RVSP and PASP (r=0.640). This correlation was significant in both male and female, white or non-white, forced vital capacity (FVC) >60%, and presence of fibrosis (p<0.001). However, it was less robust in patients with FVC of 50% or less. RVSP was considered inaccurate if the difference with PASP was > 10mmHg. Inaccurate echocardiographic estimation of the invasive PASP occurred in 50.8%, with overestimation mostly in patients without PH, and underestimation in patients with severe PH. An RVSP>50mmHg was associated with worse survival.

Conclusions: In this real world multicenter cohort of sarcoidosis patients, we found a significant correlation between RVSP as determined by echocardiography and invasive PASP. Over- or underestimation of PASP occurred frequently. Therefore, echocardiographic RVSP measurement alone to screen for PH in sarcoidosis should be used with caution.

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder characterized by the accumulation of surfactant in the alveolar spaces resulting in hypoxemic respiratory failure. Whole lung lavage (WLL), the preferred treatment for PAP, physically removes the lipoproteinaceous material from the alveolar spaces. Since its initial description in 1963, the WLL procedure has undergone various modifications. However, the procedure has not been standardized yet. After securing a double lumen endotracheal tube, we perform WLL under general anesthesia. One lung is ventilated, while the other is lavaged using one-liter aliquots of pre-warmed saline. We use gravity-assisted drainage of the lavaged lung after each cycle till the milky white and opaque fluid becomes clear (usually 15-20 cycles). Herein, we describe the step-by-step procedure, precautions, and monitoring of WLL. We also provide videos demonstrating one-lung ventilation and bronchoscopic confirmation of lung isolation.

Background: In pulmonary sarcoidosis, respiratory tract lesions almost always appear, and residual lung shadows require treatment in about 20% of cases. Pulmonary fibrosis is among the three leading causes of death. Treatment strategies are urgently needed to inhibit the progression of pulmonary fibrosis by combining antifibrotic drugs and immunosuppressive drugs such as corticosteroids. Establishing consensus on the process of pulmonary fibrosis progression is important for determining the most effective treatment.

Our review: Among more than 2500 cases of sarcoidosis treated at our hospital, cases that led to chronic respiratory failure were analyzed for CT findings of pulmonary fibrosis. Early in sarcoidosis, granulomatous lesions appeared along the bronchovascular bundle. As pulmonary fibrosis progressed, a central consolidation developed on the central side in the direction of lymph flow, a peripheral consolidation developed on the pleural side, and a central-peripheral band developed connecting the two. Infiltrative or wedge-shaped shadows sometimes formed in the immediate subpleural area, appearing as a pleuroparenchymal fibroelastosis-like lesion. Traction bronchiectasis may form cysts at the periphery or may congregate to form a honeycomb lung-like structure. Combination of these lesions led to shrinkage of the upper lobe. Patients with multiple peripheral cysts/bullae had a unique disease course characterized by wheezing and concomitant pulmonary hypertension and pulmonary aspergillosis.

Conclusion: Further understanding of the process of pulmonary fibrosis progression is needed. Summarizing imaging findings and understanding their contribution to respiratory impairment will contribute to comprehensively evaluating the stages of pulmonary fibrosis progression and establishing an optimal treatment strategy.

Introduction: Takayasu's arteritis (TA) is well-known yet rare disorder, defined as a chronic large vessel vasculitis mainly involving the aorta and its major branches. We present a complex case of a 51-year-old female patient who first presented with acute myocardial infarction as an initial manifestation of Takayasu arteritis, and later with an acute onset of ischemic stroke.

Case report: We present a case of 51-year-old female patient who was admitted at the Clinic of Nephrology and Clinical Immunology. During hospitalization, a sudden onset of intense chest pain occurred, followed by a development of heart failure to the level of cardiogenic shock. Electrocardiography showed signs of ST-elevated myocardial infarction (STEMI) of the anterior wall, and an increase in cardiospecific enzymes. CT angiography indicated an occlusion of the left common carotid artery (ACC), subclavian and axillary arteries as well as a penetrating aortic ulcer localized infrarenal. In the further course of treatment, left-sided weakness of the body was registered. Head CT scan showed an acute ischemic lesion high parietal on the right, as well as a chronic ischemic lesion on the front right. Doppler ultrasonography of carotid and vertebral arteries registered left occlusion, right ACC/external carotid artery (ACE) stenosis with suspected "macaroni sign". Final diagnosis of Takayasu arteritis was established and corticosteroids were included in the therapy (primarily in pulse doses) with the first pulse of cyclophosphamide of 1000mg.

Conclusion: This disease should be considered in female patients who present with chronic inflammation and acute coronary syndrome.

Background: Sarcoidosis, a multisystemic disease of unknown etiology, is characterized by non-caseating granulomatous inflammation. This study aimed to investigate the efficiency of atherogenic indices and ultrasonographic evaluation of carotid artery on predicting atherosclerosis in patients with sarcoidosis.

Methods: The study included 44 subjects followed with diagnosis of sarcoidosis and 53 age and gender matched healthy subjects as controls. Laboratory findings, pulmonary function tests and carotid artery ultrasonography of all participants were evaluated.

Results: Of the participants with sarcoidosis 70.5% was female and the mean age was 35.36±7.18 years, while 64.2% of the control group were female and the mean age was 33.58±8.13 years (P=0.511 and P=0.191, respectively). High-density-lipoprotein cholesterol level in the sarcoidosis group was significantly lower than that of the control group (P=0.017), while other cholesterol levels were higher than those of the controls (P<0.05). Intima-media thickness (IMT) and peak systolic velocity (PSV) of carotid artery were higher in patients with sarcoidosis (P<0.001 and P=0.009, respectively). Atherogenic indices (Atherogenic Index (AI), Atherogenic Coefficient (AC) and Cardiogenic Risk Ratio (CRR)) were higher in sarcoidosis group compared to the controls (P<0.001, for all parameters). IMT was positively correlated with PSV, AI, AC, and CRR. A positive correlation between PSV and atherogenic indices was also detected.

Conclusions: Sarcoidosis may be a predisposing factor for atherosclerosis. Atherogenic indices, IMT of carotid artery and PSV might be considered predictors for atherosclerosis and cardiovascular diseases in asymptomatic sarcoidosis patients.