Publications by authors named "Wiatr Elzbieta"
Hypereosinophilic diseases represent a heterogeneous group that poses a significant medical challenge. An increased number of eosinophils in the blood can be a dominant symptom of hematologic diseases, such as myeloid hematopoietic cancers or lymphomas. However, it is more commonly associated with other health conditions, such as allergies, infections, and systemic inflammatory diseases.
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- The study aimed to identify risk factors for relapse and death in patients with eosinophilic granulomatosis with polyangiitis (EGPA), particularly those negative for antineutrophil cytoplasmic antibodies.
- Among 86 patients analyzed, nearly 35% experienced relapses, and about 9% died; certain factors like severe EGPA and cardiac issues increased death risk, while prolonged corticosteroid treatments raised relapse risk.
- Overall, relapses are common but do not affect mortality, and while cardiac involvement is frequent, chronic asthma treatment correlates with lower death risk despite higher relapse rates.
Article Synopsis
- * Accurate diagnosis of underlying diseases, particularly autoimmune conditions, is critical, and serological testing should be routinely conducted.
- * For treatment, nintedanib is favored over pirfenidone for underlying diseases not responding to standard therapies, and simultaneous use of immunomodulatory and antifibrotic drugs is permissible, with a focus on holistic care including non-pharmacological approaches and eligibility for lung transplantation.
Article Synopsis
- Granulomatosis with polyangiitis (GPA) is a rare condition that primarily targets the upper respiratory system, lungs, and kidneys, though other organs may be affected less frequently.
- Cardiac involvement in GPA is uncommon, and this case highlights a unique instance where thoracic pain was the only symptom presented.
- The diagnosis was achieved through a comprehensive approach utilizing various imaging techniques and cardiac histology to identify the underlying issue.
Article Synopsis
- Eosinophilia, a condition characterized by high levels of eosinophils in the blood, is significant for diagnosing lung disorders and has important implications for treatment and prognosis.
- The study included 46 patients with eosinophilia and respiratory issues, using standard diagnostic methods and various statistical analyses to explore the relationship between eosinophil levels and clinical symptoms.
- Results indicated that severe eosinophilia (≥5,000 cells/μl) was linked to more severe health problems, including skin, heart, and kidney diseases, and higher incidence of vasculitis compared to those with milder forms of eosinophilia.
Article Synopsis
- Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare group of diseases that cause inflammation in small blood vessels, often linked to the presence of ANCA.
- Recent research highlights the role of neutrophil extracellular traps (NETs) in AAV, suggesting they may contribute to ANCA formation.
- While ANCA serology is useful for classifying AAV and understanding its various forms, there is debate over the effectiveness of using serial ANCA measurements to track treatment and predict relapses.
Article Synopsis
- Pirfenidone, an antifibrotic medication, has been used in Poland since 2017 to treat idiopathic pulmonary fibrosis (IPF), with the PolExPIR study analyzing its real-world safety and efficacy among Polish patients from January 2017 to September 2019.
- The study identified 307 patients, with an average age of nearly 69 years, and found that many required dose adjustments; approximately 46% discontinued treatment due to various reasons, including adverse drug reactions and disease progression.
- Overall, patients showed stable pulmonary function during the study, but there was a notable decline in lung capacity over two years, with a 10.75% mortality rate among participants.
Article Synopsis
- The Polish Respiratory Society has created guidelines for diagnosing and treating idiopathic pulmonary fibrosis (IPF), based on input from experts and current literature.
- It includes 28 recommendations covering diagnosis, pharmacological treatment, and non-pharmacological/palliative care approaches.
- The guidelines emphasize avoiding surgical lung biopsy in specific cases, recommend antifibrotic medications regardless of functional impairment, and advocate for pulmonary rehabilitation and other support therapies for IPF patients.
Article Synopsis
- - Granulomatosis with polyangiitis (GPA) is a rare inflammatory disease that primarily affects the respiratory system and involves inflammation of small to medium blood vessels.
- - Diagnosis can be challenging due to vague symptoms and varying clinical presentations, often leading to misdiagnosis, especially in older patients.
- - The report highlights two elderly cases where GPA was only identified 4 to 6 years after the initial symptoms, despite multiple diagnostic procedures being performed.
Article Synopsis
- The study investigates the impact of cardiac involvement on lung function in patients with sarcoidosis, where symptoms like dyspnea and exercise intolerance are typically linked to pulmonary issues.
- Researchers analyzed data from 255 sarcoidosis patients, comparing those with cardiac sarcoidosis to controls without heart involvement, focusing on pulmonary function tests (PFTs) results.
- Findings revealed that patients with cardiac sarcoidosis had lower heart function (LVEF) and a higher prevalence of lung dysfunction compared to controls, indicating a significant relationship between heart health and lung function in these patients.
Article Synopsis
- The study explored the relationship between nasal carriage of Staphylococcus aureus and superantigens (SAg) with disease activity in patients with granulomatosis with polyangiitis (GPA), involving 150 hospitalized patients from 2009-2016.
- Results showed SAg present in about 35% of nasal swabs but did not establish a significant correlation with GPA activity, although specific SAg like SED and TSST-1 appeared more frequently in active cases.
- The use of trimethoprim/sulfamethoxazole treatment was found to be protective, while limited disease (subglottic stenosis) was linked to increased activity, indicating further mechanisms are needed to understand GPA exacerbation
Article Synopsis
- Clinicians often find it difficult to differentiate between pulmonary tuberculosis and lung cancer, as the symptoms and imaging results can be quite similar.
- A case report describes a patient who had been treated for advanced lung cancer a decade ago and was recently hospitalized due to concerns about cancer recurrence.
- Ultimately, despite the initial suspicion of cancer progression, the patient was diagnosed with tuberculosis.
A case report of steroid resistant cardiac sarcoidosis successfully managed with methotrexate.
Sarcoidosis Vasc Diffuse Lung Dis
April 2018
Article Synopsis
- * Corticosteroids are the primary treatment for cardiac sarcoidosis but can have notable short and long-term side effects.
- * A 42-year-old man with both lung and heart sarcoidosis saw initial improvement from corticosteroids for his lungs, but after adding methotrexate for his heart, he showed significant recovery confirmed by imaging.
Article Synopsis
- Diffuse alveolar haemorrhage (DAH) is a serious condition caused by damage to the small blood vessels in the lungs, leading to the accumulation of red blood cells in the air spaces.
- The prognosis and treatment for DAH depend on the underlying disease, and it can complicate conditions like venous thromboembolism (VTE), making management difficult for healthcare providers.
- A case study is presented of a young patient who experienced severe DAH due to anti-GBM disease, which was further complicated by a pulmonary embolism.
Article Synopsis
- - The study investigates the prevalence of alpha-1 antitrypsin deficiency (A1ATD) alleles PI*Z and PI*S in patients with pulmonary Langerhans cell histiocytosis (PLCH), a condition often linked to smoking and characterized by cystic lung lesions.
- - Blood samples from 34 adult PLCH patients showed that deficiency alleles PI*Z and PI*S were present in 5.88% and 2.94% of them, respectively, indicating a higher incidence compared to the general Polish population.
- - The findings suggest that the occurrence of A1ATD alleles in PLCH patients is more frequent than previously expected, prompting further investigation into the relationship between
Article Synopsis
- Cryptogenic organizing pneumonia (COP) is typically treated with corticosteroids, but this study compares the effectiveness of clarithromycin (CAM) versus prednisone (PRE) in patients without respiratory failure.
- Over 15 years, 40 patients received CAM and 22 received PRE, with similar clinical presentations but notable differences in treatment response and side effects.
- CAM showed a better tolerance and fewer relapses (10% vs. 54.5%) compared to PRE, suggesting it's a viable alternative for patients with normal pulmonary function, although not effective for everyone.
Article Synopsis
- * This study focuses on the impact of pregnancy on lung function in LCH patients, examining eight pregnant women among 45 hospitalized cases between 2000 and 2015, noting their pregnancy history and outcomes like C-sections and miscarriages.
- * Results indicate that pregnancy does not significantly affect lung function measurements or blood oxygen levels in LCH patients, suggesting that pregnancy is safe for these individuals, with no major complications during delivery or postpartum.
Article Synopsis
- - Sarcoidosis is a disease primarily impacting the lungs, often resolving on its own, but treatment is necessary for severe cases involving critical organs or progressive disease.
- - A study analyzed data from 1,810 sarcoidosis patients discharged between 2010 and 2013, revealing that only 15.5% received systemic immune-modulating treatment, with men and patients over 50 being the most likely to be treated.
- - The findings indicate that less than 16% of sarcoidosis patients generally need systemic treatment, with those experiencing involvement of organs other than the lungs being significantly more likely to require intervention.
Article Synopsis
- Inflammatory cytokines play a role in the development of cryptogenic organizing pneumonia (COP), and research shows that macrolides like clarithromycin can inhibit cytokine production in patients.
- A study with 26 COP patients (average age 56.5) assessed the levels of interleukins and transforming growth factor in the blood and lungs before and after treatment with clarithromycin.
- Results indicated that treatment led to significant reductions in serum levels of IL-6, IL-8, and TGF-β, particularly in responders, suggesting that their decrease correlates with improved patient outcomes.
Article Synopsis
- * Out of 250 GPA patients, 34 (13.6%) developed SGS, and this condition often appeared independently of other disease symptoms, particularly during or after immunosuppressive therapy.
- * IDIT was performed on all patients, leading to immediate improvement, with a median response time of 37 months and no need for tracheostomy, suggesting IDIT is a reliable treatment option for patients with SGS related to GPA.
Article Synopsis
- A 53-year-old woman was diagnosed with pulmonary sarcoidosis, which is a condition where clusters of inflammatory cells form in the lungs.
- The disease progressed to involve her heart, leading to serious complications.
- Cardiovascular magnetic resonance imaging confirmed her heart failure, highlighting the connection between her lung condition and heart issues.
Article Synopsis
- - Cardiac sarcoidosis (CS) is a serious but often overlooked condition affecting patients with sarcoidosis, highlighting the need for a simpler diagnostic tool for healthcare providers.
- - In a study of 201 patients with biopsy-proven sarcoidosis, cardiac involvement was identified in 49 patients (24.4%), with several risk factors associated with CS, including male sex, cardiac symptoms, and elevated serum NT-proBNP levels.
- - A CS Risk Index was created to help predict cardiac involvement, showing an accuracy of 82%, with 50% sensitivity and 94% specificity, indicating it could be useful for clinicians in identifying at-risk patients.