Sarcoidosis, Vasculitis, and Diffuse Lung Diseases最新文献

Background and aim: The aim was to compare the radiological and clinical characteristics of sarcoidosis between elderly and non-elderly patients.

Methods: This retrospective observational study was carried out in patients with sarcoidosis. Elderly-onset sarcoidosis was defined as sarcoidosis diagnosed in patients ≥65 years-old. Patients were stratified by age (≥65 years versus <65 years) and radiological and clinical data were compared between age groups.

Results: Of the 163 patients, 38 (23.3%) were in the elderly group and 125 (76.7%) were in the non-elderly group. Elderly patients more frequently demonstrated arthralgia (50% vs. 12.8%, p<0.001), coronary artery disease  (15.8% vs. 2.4%, p=0.005), congestive heart failure (13.2% vs. 0.8%, p=0.003), pneumonia (7.9% vs. 0.8%, p=0.04), and pleural fluid (18.4% vs. 0.0%, p<0.001). Clinical remission was significantly more likely in younger patients than in the elderly (76.8% vs. 55.3%, p=0.01). The clinical course to chronic-progressive disease was similar in both groups (p=0.635). Radiologically, lymph nodes measuring 10-25 mm in the short axis (89.5% vs. 72.6%, p=0.032), usual interstitial pneumonia pattern (10.5% vs. 0.8%, p=0.011), and main pulmonary artery diameter above 30 mm (34.2% vs. 16.0%, p=0.014) were significantly more frequent in the elderly group. Elderly patients tended to demonstrate Scadding stage I and II sarcoidosis (39.5% and 31.6%).

Conclusions: Presentation of elderly-onset sarcoidosis appears to differ from young-onset sarcoidosis. Radiologically, lymph node enlargement and the pattern of fibrosis may be distinctive.

Background: The aim of this study is to determine the demographic, clinical and laboratory characteristics of the patients who followed up with the diagnosis of sarcoidosis, to investigate the distribution frequency of rheumatological findings and to examine the disease management from the perspective of rheumatology.

Methods: Patients who were followed up with the diagnosis of sarcoidosis in the rheumatology clinic of Ankara City Hospital between November 2019 and November 2022 were evaluated. Demographic, clinical, radiological, serological, laboratory, and histopathological findings, and rheumatological, systemic, and locomotor system examination findings of the patients were obtained from the medical data registered in the hospital.

Results: A total of seventy sarcoidosis patients (48.98 ± 11.78 years, %75 female) were included in the study. Joint involvement was observed in 64.3% of cases, skin involvement in 48.6% of cases, and ocular involvement in 25.7% of cases. The ankle was the most frequently involved joint, followed by the knee and small joints in the foot. Corticosteroids were the most used therapeutic agent, and pulmonary and joint findings were the most common reasons for starting treatment.

Conclusions: Sarcoidosis is a disease that mimics many diseases, misdiagnosis and treatment should be avoided with a good and fast differential diagnosis. Clinicians, especially rheumatologists, should remember sarcoidosis more frequently and keep it in mind in the differential diagnosis.

Idiopathic pulmonary fibrosis (IPF) is the most common progressive interstitial disease of unknown etiology. The course of disease is not possible to predict. Frequent monitoring using multiple assessments is important to evaluate disease progression. Currently, there is no consensus on how progression should be defined. Nintedanib and pirfenidone slow the progression of IPF, but the disease can progress even under anti-fibrotic treatment. The goal of this review is to examine and summarize the current data about IPF progression in patients who were on anti-fibrotic treatment. Also, we outline the limitations of the tests used for disease progression.

 Bleomycin is associated with pulmonary toxicity ranging from pneumonitis, pulmonary fibrosis, to fatal acute respiratory distress syndrome. Oxygen administration can potentiate or precipitate bleomycin pulmonary toxicity, and the most common setting of oxygen exposure is during anesthesia. We report here the successful management and perioperative care of a patient with documented bleomycin pulmonary toxicity who had to undergo an eight hour long retroperitoneal surgery. With proper preoperative assessment, chest physiotherapy, inhaled steroids and bronchodilators, antibiotics, operative restriction of oxygen and fluids and good postoperative care no further pulmonary insult was inflicted.

Background: Bleomycin causes increased production of reactive oxygen species, leads to pulmonary toxicity, fibroblast activation, and fibrosis.

Objectives: This study aimed to evaluate the protective effect of pirfenidone on bleomycin-induced lung toxicity in rats.

Methods: Twenty-eight adult rats were randomly divided into 3 groups; Bleomycin (B group, n=10), Bleomycin and Pirfenidone (B-PND group, n=13), and the control group (n=5). The bleomycin regimen was administered for 9 weeks. Pirfenidone was administered at 100 mg/kg daily. Total antioxidant level (TAS), total oxidant level (TOS), tumor necrosis factor (TNF-α), transforming growth factor (TGF-β1), matrix metalloproteinase-2 (MMP-2), plasminogen activator inhibitor (PAI) levels were studied. Histopathologically, sections were stained with Hematoxylin-eosin and Masson-trichrome for grading-scoring according to the Ashcroft score.

Results: Stage 3 fibrosis was observed in 50% of the B group rats, stage 3 and higher fibrosis was never detected in the B-PND group and the difference was statistically significant (p=0.003). When evaluating tissue inflammation, the inflammation was higher in the B-PND group than in the other groups (p<0.001). Pleuritis was detected in all rats in group B, while was not observed in B-PND and control group (p<0.001). The TAS level was found to be significantly higher in group B than in group B-PND (p=0.034), while no difference was found between TOS, TNF-α, MMP-2, PAI, TGF-β1.

Conclusions: Pirfenidone had a statistically significant protective effect in bleomycin-induced lung fibrosis and pleuritis in rats.  Despite the presence of inflammation in the tissue, no significant changes were observed in inflammation markers in the peripheral blood. Novel serum biomarkers are needed to indicate the presence of inflammation and fibrosis in the lung.

Background and aim: Tuberculosis and sarcoidosis are the two most important granulomatous diseases that physicians have difficulty in differential diagnosis. In our study, we aimed to observe the place of systemic immune-inflammation index (SII) level in the differentiation of patients diagnosed with endoboronchial ultrasonography (EBUS).

Methods: Our study included 494 patients who applied to our hospital's chest diseases outpatient clinic between 2015 and 2020 and underwent endobronchial ultrasonography (EBUS) for mediastinal lymphadenopathy (LAP). Patients' follow-up for at least 2 years after diagnosis and pre-procedural hematologic parameters were retrospectively recorded.

Results: In the comparison of SII between groups, it was observed that SII was statistically significantly higher in patients followed up for tuberculous lymphadenitis compared to patients with sarcoidosis and reactive LAP (p=0.01, <0.001). In sarcoidosis patients, SII levels were statistically significantly higher than in patients with reactive LAP (p=0.002). Platelet, sedimentation and SII levels were statistically significantly higher in stage 2 patients compared to stage 1 patients, while lymphocyte levels were lower (p=0.009, 0.001, 0.001, 0.001, 0.001 respectively). In the ROC curve analysis of the SII level of patients with sarcoidosis and tuberculosis LAP, the AUC was 0.668 and when the cut-off value for the SII level was 890.667, the sensitivity was 70% and the specificity was 66% in the differentiation of tuberculosis and sarcoidosis lymphadenitis.

Conclusion: SII may be an easily applicable parameter in the differentiation of tuberculosis and sarcoidosis LAP with granuloma and in the differentiation of granulomatous diseases from reactive LAP.

Sarcoidosis may progress to pulmonary fibrosis in 5% of patients with significantly increased mortality. Histopathology shows fibrosis in a lymphangitic pattern surrounding the granulomas. Th1 to Th2 shift in environment along with angiogenesis is implicated in exuberant fibrosis. Clinical features include dyspnea, cough, and frequently with pulmonary function tests showing a mixed ventilatory defect with severely decreased diffusion capacity of carbon monoxide.  Serologic markers including soluble interleukin 2 receptor, chitotriosidase and kern von den lunges 6, and chemokine ligand 18 are elevated and implicated in progression of disease. CT imaging shows fibrosis along bronchovascular bundles with reticulations, traction bronchiectasis and honeycombing predominantly in the upper and central distribution. Complications include sarcoidosis-associated pulmonary hypertension (SAPH) and chronic pulmonary aspergillosis. Treatment involves glucocorticoids and steroid-sparing agents in the presence of active granulomas. Anti-fibrotic agents such as pirfenidone and nintedanib have been shown to slow down pulmonary function decline in randomized clinical trials involving sarcoidosis-associated pulmonary fibrosis. Transplant workup is indicated in New York Heart Association class III or IV with similar success rates as in other lung transplant patients.

Background Sarcoidosis is a granulomatous multisystem disease of uncertain aetiology. The disease has major inflammatory and immune components; however, the immunopathogenesis is not well understood. Micro ribonucleic acids (microRNAs) are classes of miniature, single-stranded, non-coding RNAs. Their key recognised role includes mediating the silencing of target genes post-transcriptionally. Recently, the role of miRNAs has gained interest in numerous disorders, suggested as being involved in pathogenesis of those diseases and acting as disease markers. Very little is known about the role of miRNAs in sarcoidosis, with nothing known regarding miRNAs in South African patients. The main objective, therefore, was to investigate the serum expression of approximately 800 miRNAs in patients with sarcoidosis compared with race-, age- and gender-matched healthy controls. Methods A total of six patients and six matched controls participated in this study. Whole blood samples were collected in EDTA tubes, processed and the plasma retained. RNA was extracted from the stored plasma samples using the QIAGEN miRNeasy Mini Kit® and concentrated using a salt-ethanol precipitation. The extracted miRNA was profiled using an nCounter® miRNA human v3 expression assay and data analysed using the nSolver™ Analysis Software. Results After excluding one sample/control pair because of cellular RNA contamination, the remaining five patient and five matched control samples were analysed, and 145 miRNAs were found to be potentially differentially expressed. On applying a Bonferroni correction, the only miRNA that was significantly different was miRNA let-7a-5p, which was significantly overexpressed (141-fold change; p<0.0003) in patients compared with controls. Conclusion This is the first miRNA report of differentially expressed miRNAs in the serum of patients with sarcoidosis and matched healthy controls in South Africa. The results obtained suggest that miRNAs may play a role in sarcoidosis pathogenesis. Whether these molecules have diagnostic or prognostic implications, needs future studies recruiting larger patient cohorts.

Background and aim: Inhalational exposures have been hypothesized to play a role in the pathogenesis of sarcoidosis. Herein, we describe a cohort of US Military personnel diagnosed with sarcoidosis during or after deployment to Southwest Asia and Afghanistan, who experienced complex inhalational exposures to burn-pits and desert dust.

Methods: Consecutive military personnel at four sub-specialty clinics across the United States were screened for deployment to Southwest Asia and Afghanistan and diagnosis of sarcoidosis based on 1999 ATS/ERS/WASOG Statement on Sarcoidosis. Detailed demographic, deployment and exposure data was collected. The data combined was analyzed after de-identification and local IRB approval.

Results: Twenty-one patients met our case definition. Seventeen patients were male and 62% had extrapulmonary involvement, including 38% with musculoskeletal involvement.  Conclusions: Our study suggests that the sarcoidosis in military personnel to Southwest Asia can be diagnosed many years after deployment. To our knowledge, this is the first case series to describe a group of military personnel diagnosed with sarcoidosis and exposures specific to military deployment to Southwest Asia.

Background and aim: Granulomatous hepatitis (GH) is associated with various aetiologies, especially inflammatory and infectious disorders. Sarcoidosis is a granulomatous disease in which the liver is the fourth most affected organ. Since epithelioid cell granulomas are not specific to sarcoidosis and since most patients with hepatic sarcoidosis are asymptomatic, valuable diagnostic biomarkers are needed to support the diagnosis of sarcoidosis. This study proposes to assess the diagnostic value of serum angiotensin converting enzyme (sACE) and lymphopenia in GH for sarcoidosis.

Methods: We retrospectively analyzed the records of 90 patients referred to the internal medicine or hepatogastroenterology departments of the Lyon University Hospital (Lyon, France) between March 2002 and January 2020 in a context of GH.

Results: In our tertiary center, 38 patients with sarcoidosis were identified among 73 patients with GH. Lymphopenia had a high specificity (85.7%), which increased when combined with elevated (97.0%). Interestingly, specificity increased in patients under 50 years old (100%).

Conclusions: Those results suggests that lymphopenia and sACE may be valuable biomarkers for sarcoidosis diagnosis in GH when combined, especially in younger patients.